Texas Children's Hospital Handbook of Pediatrics and Neonatology

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Texas Children’s Hospital

Handbook of Pediatrics and Neonatology

Notice Medicine is an ever-changing science. As new research and clinical experience broaden our knowledge, changes in treatment and drug therapy are required. The authors and the publisher of this work have checked with sources believed to be reliable in their efforts to provide information that is complete and generally in accord with the standards accepted at the time of publication. However, in view of the possibility of human error or changes in medical sciences, neither the authors nor the publisher nor any other party who has been involved in the preparation or publication of this work warrants that the information contained herein is in every respect accurate or complete, and they disclaim all responsibility for any errors or omissions or for the results obtained from use of the information contained in this work. Readers are encouraged to confirm the information contained herein with other sources. For example and in particular, readers are advised to check the product information sheet included in the package of each drug they plan to administer to be certain that the information contained in this work is accurate and that changes have not been made in the recommended dose or in the contraindications for administration. This recommendation is of particular importance in connection with new or infrequently used drugs.

Texas Children’s Hospital

Handbook of Pediatrics and Neonatology Editor Adam W. Lowry, MD Clinical Postdoctoral Fellow, Cardiology Texas Children’s Hospital Department of Pediatrics Baylor College of Medicine Houston, Texas Associate Editors Kushal Y. Bhakta, MD Assistant Professor, Pediatrics Section of Neonatology Texas Children’s Hospital Department of Pediatrics Baylor College of Medicine Houston, Texas Pratip K. Nag, MD, PhD Assistant Professor, Pediatrics Section of Hospital and Emergency Medicine Texas Children’s Hospital Department of Pediatrics Baylor College of Medicine Houston, Texas

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Between 1977 and 2008, more than 5000 students earned Doctor of Medicine degrees from the Baylor College of Medicine, and approximately 1200 new pediatricians graduated from the Baylor/Texas Children’s Hospital pediatric residency training program. Every one of these individuals was shaped in some way by Dr. Ralph D. Feigin. Simply stated, Dr. Feigin’s contributions to the science and practice of pediatrics, his leadership roles in a multitude of national professional organizations, and his mentorship of thousands of young medical students and pediatric trainees made him one of the most influential and important pediatricians of the past 30 years. I trained in pediatrics at Baylor and Texas Children’s with Dr. Feigin. And along the way, I collected a thousand memories and stories. For sure, I was witness to his legendary memory and unparalleled skills as a physician and diagnostician. I remember one trip I made with him to another top U.S. medical center, where the chief of pediatrics presented the case of a critically ill child he described as having “baffled the best minds at the university.” A wink and an elbow from Dr. Feigin less than five minutes into the presentation told me that he already had made the diagnosis. His diagnosis was correct, of course, and the child was treated and survived. I remember another trip we made together to the Mayo Clinic. Dr. Feigin correctly predicted the number of seconds our accelerating jet airliner would require for takeoff, based on the type of aircraft, the number of passengers, the amount of fuel required to fly to Minnesota, and the approximate weight of the luggage he had seen loaded into the belly of the plane. Then, he pointed out, by number or name, nearly every major highway we flew over between Houston and Minnesota, as if he had memorized the national interstate highway system. Exaggeration was never necessary when one was describing Dr. Feigin. I remember fondly all of our runs together at Houston’s Memorial Park, Dr. Feigin on one occasion exhorting me to run faster so that we would pass a group of U.S. Marines on a six-mile training run. We did, or rather, he did pass every one of them.

And I remember a hundred examples of Dr. Feigin’s eternal optimism, his ability to dissect down and solve the most complex challenges, and his knack for seeing the best and the value in everyone. I remember well his admonition to “treat everyone with kindness, especially the people who have no power over you,” and his reminder that “you will meet the same people on the way down that you met on the way up.” Working with Dr. Feigin was one of the greatest honors and privileges of my life. He always led by example, always set priorities straight, and never failed to emphasize that we are here for the children. Dr. Feigin had our admiration, our respect, our affection, and most of all, our love. He was simply the best—not one in one million, not even one in one billion, but rather the finest mentor, role model, and boss that any of us ever had. Texas Children’s Hospital Handbook of Pediatrics and Neonatology is dedicated gratefully and lovingly to his memory. Mark W. Kline, MD J.S. Abercrombie Professor and Chairman Ralph D. Feigin Chair Department of Pediatrics Baylor College of Medicine Physician-in-Chief Texas Children’s Hospital Houston, Texas

Dedications I would like to recognize the mentors, friends, and family in my life, particularly my father Gregory and mother Sharon; I am indebted to each of you for your guidance, encouragement, and unconditional support. —Adam W. Lowry To my beautiful wife Leena, our darling daughter Anya, and our little girl we’re expecting in October: thanks for understanding all the long nights, and even more so, thanks for always being supportive in this enormous endeavor. I love you guys dearly. —Kushal Y. Bhakta Thanks to my wonderful wife Sue, our sons, and my loving and supportive family and friends for all their unconditional support and encouragement to get me where I am today. —Pratip K. Nag

Contents Contributors ........................................................................................ ix Preface ................................................................................................ xvii Abbreviations....................................................................................... xviii

Section 1: General Clinical Information 1. Clinical Pearls for the Wards....................................................... 2. Analgesia and Sedation ............................................................... 3. Procedures..................................................................................

1 11 21

Section 2: Nutrition, Growth and Formula 4. Neonatal and Pediatric Nutrition ................................................ 5. Assessment of Nutrition and Growth...........................................

35 49

Section 3: Pediatrics 6. 7. 8. 9. 10. 11. 12. 13. 14. 15. 16. 17. 18. 19. 20. 21. 22. 23. 24. 25. 26. 27.

Adolescent Medicine ................................................................... Allergy and Immunology ............................................................. Cardiology .................................................................................. Critical Care................................................................................ Dentistry and Oral Health ........................................................... Dermatology ............................................................................... Ear, Nose, and Throat ................................................................. Emergency Medicine ................................................................... Endocrinology............................................................................. Fluids, Electrolytes, and Acid–Base Balance ................................ Gastroenterology ........................................................................ Genetics ...................................................................................... Hematology ................................................................................ High-Yield Ambulatory Care ....................................................... Infectious Disease ....................................................................... Nephrology ................................................................................. Neurology ................................................................................... Oncology .................................................................................... Orthopedics and Sports Medicine ............................................... Pulmonology ............................................................................... Rheumatology ............................................................................. Toxicology ..................................................................................

51 60 66 83 106 109 125 130 143 153 169 184 196 209 218 250 268 289 301 312 327 340

viii

Contents

Section 4: Neonatology 28. Newborn Nursery........................................................................ 29. Fetal Assessment and Prenatal Diagnosis..................................... 30. Stabilization, Delivery Room Care, and Initial Treatment of the VLBW Infant ......................................... 31. Discharge Planning ..................................................................... 32. Cardiology .................................................................................. 33. Dermatology ............................................................................... 34. Endocrinology............................................................................. 35. Gastroenterology ........................................................................ 36. Genetics ...................................................................................... 37. Hematology ................................................................................ 38. Infectious Diseases ..................................................................... 39. Nephrology ................................................................................. 40. Neurology ................................................................................... 41. Pain Management ....................................................................... 42. Pulmonology ............................................................................... 43. Surgical Disorders .......................................................................

348 354 363 376 379 386 392 409 421 425 437 445 448 456 458 471

Index .................................................................................................. 479

Contributors All contributors are affiliated with Texas Children's Hospital and Baylor College of Medicine, Houston, Texas, except where noted.

Authors Fahd A. Ahmad, MD Resident, Pediatrics G. Todd Alonso, MD Instructor, Pediatric Emergency Medicine Jeffrey Alten, MD Assistant Professor, Pediatrics and Critical Care Medicine University of Alabama at Birmingham Birmingham, Alabama Mary Goodwin Altobelli, MD Resident, Pediatrics Amy S. Arrington, MD, PhD Resident, Pediatrics Ioanna Athanassaki, MD Assistant Professor, Pediatric Endocrinology Claire E. Bocchini, MD Clinical Postdoctoral Fellow, Pediatric Infectious Disease John P. Breinholt, III, MD Assistant Professor, Pediatric Cardiology Indiana University School of Medicine Indianapolis, Indiana Callie Byrd, MD Resident, Pediatrics Waldemar F. Carlo, MD Clinical Postdoctoral Fellow, Pediatric Cardiology

Michael C. Chapman, MD Clinical Postdoctoral Fellow, Pediatric Sports Medicine Daniel C. Chelius, MD Resident, Otolaryngology Catherine Cibulskis, MD Clinical Postdoctoral Fellow, Neonatology Craig A. Coleby, MD Clinical Postdoctoral Fellow, Primary Care Sports Medicine Kendra J. Conkright, MD Resident, Internal Medicine and Pediatrics Christopher Cotroneo, MD Resident, Pediatrics Ashley M. Cox, MD Resident, Pediatrics Shani H. Cunningham, DO Resident, Pediatrics Tamara Cunningham, MD Resident, Pediatrics Natalie J.M. Dailey, MD Resident, Internal Medicine and Pediatrics Carrie A. Danner, MD Resident, Pediatrics

x

Contributors

Jamie A. Decker, MD Assistant Professor, Pediatric Cardiology

Brent D. Kaziny, MD Resident, Pediatrics

Kristin H. Dillard, MD Clinical Postdoctoral Fellow, Pediatric Allergy and Immunology

Jarrod D. Knudson, MD, PhD Clinical Postdoctoral Fellow, Pediatric Cardiology

Ankhi Dutta, MD, MPH Clinical Postdoctoral Fellow, Pediatric Infectious Disease

Kristin C. Koush, MD Chief Resident in Pediatrics

Lisa Ecroyd, MD Resident, Pediatrics Erik G. Ellsworth, MD Clinical Postdoctoral Fellow, Pediatric Cardiology Peter Ermis, MD Resident, Pediatrics Angela Flores, MD Clinical Postdoctoral Fellow, Neonatology Kathleen Blue Fung, MD Resident, Pediatrics Morey W. Haymond, MD Professor, Pediatric Endocrinology Kelly Hicks, MD Resident, Pediatrics Lindsey Hoffman, DO Resident, Pediatrics Zsofia Intody, MD, PhD Resident, Pediatrics Nick A. Jernigan, MD Resident, Internal Medicine and Pediatrics Jamil Joyner, MD Resident, Pediatrics Meena Rao Julapalli, MD Resident, Dermatology

W. Buck Kyle, MD Chief Resident in Pediatrics Jennifer C. Lee, MD Resident, Pediatric Neurology Kathryn Leung, MD Assistant Professor, Pediatric Hematology and Oncology Kim Little, MD Instructor, Pediatrics Charles G. Macias, MD, MPH Associate Professor, Pediatric Emergency Medicine Thaddeus D. May, MD Resident, Pediatrics and Internal Medicine J. Chase McNeil, MD Clinical Postdoctoral Fellow, Pediatric Infectious Disease Mona L. McPherson, MD, MPH Associate Professor, Pediatric Critical Care Suzanne Melancon-Kyle, MD Resident, Pediatrics Barbara A. Montagnino, MS, RN, CNS Clinical Nurse Specialist, Progresssive Care Unit

Contributors Elizabeth H. Muth, MD Resident, Internal Medicine and Pediatrics Duyen K. Nguyen, DO Clinical Postdoctoral Fellow, Pediatric Allergy and Immunology Trung C. Nguyen, MD Assistant Professor, Pediatric Critical Care Medicine Alison E. Niebanck, MD Chief Resident in Pediatrics Kelly Ann Olson, MA, MD Resident, Pediatrics Gloria Oramasionwu, MD, MPH Clinical Postdoctoral Fellow, Pediatric Infectious Disease Michelle Parker, MD Resident, Pediatrics Rod Pellenberg, MD Resident, Pediatrics Britt Peña, MD Chief Resident in Pediatrics Nina Poliak, MD, MPH Clinical Postdoctoral Fellow, Pediatric Allergy and Immunology

Danielle Rios, MD Resident, Pediatrics John M. Robertson, MD Pediatric Pulmonology Houston Pediatric Pulmonary and Sleep Associates Houston, Texas Amber J. Robinett, MD Resident, Pediatrics Christian P. Schaaf, MD, PhD Clinical Postdoctoral Fellow, Molecular and Human Genetics Margaret D. Scheffler, MD Resident, Pediatrics Heidi Schwarzwald, MD, MPH Associate Professor, Pediatric Retrovirology and Global Health Geeta Singhal, MD, MEd Assistant Professor, Pediatrics Justin Smith, MD Resident, Pediatrics Mary Nell Suell, MD Assistant Professor, Pediatric Hematology and Oncology, Pathology

Vandana Raman, MD Clinical Postdoctoral Fellow, Pediatric Endocrinology

Sarat Susarla, MD Pediatric Pulmonology Houston Pediatric Pulmonary and Sleep Associates Houston, Texas

Monica Ramos, MD Clinical Postdoctoral Fellow, Neonatology

Daniel T. Swarr, MD Resident, Pediatrics

Damitra D. Ramos-Patel, MD Chief Resident in Internal Medicine and Pediatrics

Bridget Sweeney Gotsch, MD Resident, Pediatrics

Rebecca Rawalt, MD Clinical Postdoctoral Fellow, Neonatology

xi

Jun Teruya, MD, DSc Associate Professor, Pathology, Pediatrics, and Internal Medicine

xii

Contributors

Ashaunta R. Tumblin, MD Resident, Pediatrics

Katherine J. Weiss, MD Chief Resident in Pediatrics

Irene T. Tung, MD Resident, Pediatrics

Megan A. Westbrook, MD Resident, Pediatrics

Katri V. Typpo, MD Assistant Professor, Pediatric Critical Care Medicine

Angus A. Wilfong, MD Associate Professor, Pediatric Neurology

Jamie Varughese, MD Clinical Postdoctoral Fellow, Adolescent and Sports Medicine

Wojciech Wiszniewski, MD, PhD Clinical Postdoctoral Fellow, Molecular and Human Genetics

Shannon Wai, MBChB Clinical Postdoctoral Fellow, Pediatric Emergency Medicine

Amber E. Young, MD Resident, Pediatrics

Michael F. Wangler, MD Clinical Postdoctoral Fellow, Molecular and Human Genetics

Suyearn Hong Yu, MD Resident, Pediatrics

Consulting Editors Kaashif A. Ahmad, MBBS, MSc Clinical Postdoctoral Fellow, Neonatology

Teresa K. Duryea, MD Associate Professor, Pediatrics

Carlos A. Bacino, MD Associate Professor, Molecular and Human Genetics

Eric C. Eichenwald, MD Associate Professor, Neonatology

Patricia A. Baxter, MD Instructor, Pediatric Hematology and Oncology Maria A. Carrillo-Marquez, MD Clinical Postdoctoral Fellow, Pediatric Infectious Disease Daniel C. Chelius, MD Resident, Otolaryngology Cathrine Constantacos, MD Clinical Postdoctoral Fellow, Pediatric Endocrinology University of North Carolina at Chapel Hill Chapel Hill, North Carolina

Elaine K. Fielder, MD Clinical Postdoctoral Fellow, Pediatric Emergency Medicine Ricardo J. Flores, MD Clinical Postdoctoral Fellow, Pediatric Hematology and Oncology Amy Bennett Good, MD Clinical Postdoctoral Fellow, Neonatology W. Eddie Gordon, MD Clinical Postdoctoral Fellow, Pediatric Critical Care Medicine J. Nina Ham, MD Assistant Professor, Pediatric Endocrinology

Contributors

xiii

Leslie L. Harris, MD Assistant Professor, Neonatology

Siby Moonnumakal, MD Assistant Professor, Pediatric Pulmonology

Lana Hattar, MD Clinical Postdoctoral Fellow, Pediatric Gastroenterology, Hepatology, and Nutrition

James Owens, MD, PhD Assistant Professor, Pediatric Neurology

Ryan W. Himes, MD Clinical Postdoctoral Fellow, Gastroenterology, Hepatology, and Nutrition Emma M. Jones, MD Clinical Postdoctoral Fellow, Pediatric Hematology and Oncology Roshni Kandyil, MD, PhD Clinical Postdoctoral Fellow, Allergy and Immunology Erin C. Kish, MD Clinical Postdoctoral Fellow, Adolescent and Sports Medicine Federico R. Laham, MD Instructor, Pediatric Infectious Disease Fong W. Lam, MD Clinical Postdoctoral Fellow, Pediatric Critical Care Medicine Katherine Leaming-Van Zandt, MD Clinical Postdoctoral Fellow, Pediatric Emergency Medicine Joyce Li, MD, MPH Clinical Instructor, Pediatric Emergency Medicine Harvard Medical School Boston, Massachusetts Charles G. Macias, MD, MPH Associate Professor, Pediatrics Seema Mehta, MD Clinical Postdoctoral Fellow, Gastroenterology, Hepatology, and Nutrition

Faria A. Pereira, MD Assistant Professor, Pediatric Emergency Medicine Muralidhar H. Premkumar, MBBS, DNB, MRCPCH Clinical Postdoctoral Fellow, Neonatology Andrea A. Ramirez, MD Research Postdoctoral Fellow, Pediatric Rheumatology Stefanie P. Rogers, MD Clinical Postdoctoral Fellow, Neonatology Brian R.E. Schultz, MD Clinical Postdoctoral Fellow, Pediatric Emergency Medicine Timothy Slesnick, MD Assistant Professor, Pediatric Cardiology Hannah Fouts Smitherman, MD Pediatric Emergency Medicine Cook Children’s Physician Network Fort Worth, Texas Poyyapakkam Srivaths, MD Assistant Professor, Pediatric Nephrology V. Reid Sutton, MD Associate Professor, Molecular and Human Genetics Carl Tapia, MD, MPH Assistant Professor, Pediatrics Sharonda Alston Taylor, MD Assistant Professor, Adolescent and Sports Medicine

xiv

Contributors

Keita Terashima, MD Clinical Postdoctoral Fellow, Pediatric Hematology and Oncology

Katri V. Typpo, MD Assistant Professor, Pediatric Critical Care Medicine

Ann Thomas, MD Associate Professor, Pediatrics George Washington University Washington, DC

K. Lynette Whitfield, MD Clinical Postdoctoral Fellow, Gastroenterology, Hepatology, and Nutrition

Senior Reviewers James M. Adams, Jr., MD Professor, Neonatology Coburn H. Allen, MD Assistant Professor, Pediatric Emergency Medicine, Pediatric Infectious Disease Diane M. Anderson, PhD Associate Professor, Neonatology Julie A. Boom, MD Associate Professor, Pediatrics Eileen D. Brewer, MD Professor, Pediatric Nephrology K. Dawn Bunting, RD, CSP, LD Assistant Director, Clinical Nutrition Services Gerardo Cabrera-Meza, MD Associate Professor, Neonatology Judith R. Campbell, MD Associate Professor, Pediatric Infectious Disease A. Bruce Carter, DDS Attending Periodontist See Wai Chan, MD, MPH Assistant Professor, Neonatology Binoy Chandy, MD Assistant Professor, Otolaryngology

Lindsay H. Chase, MD Assistant Professor, Pediatric Hospital Medicine Joseph N. Chorley, MD Associate Professor, Adolescent and Sports Medicine Annabelle N. Chua, MD Assistant Professor, Pediatric Nephrology Murali Chintagumpala, MBBS Professor, Pediatric Hematology and Oncology William J. Craigen, MD, PhD Associate Professor, Molecular and Human Genetics Michael C. Distefano, MD Assistant Professor, Pediatric Emergency Medicine Jan E. Drutz, MD Professor, Academic General Pediatrics Daniel I. Feig, MD, PhD Associate Professor, Pediatric Nephrology Caraciolo J. Fernandes, MD, MBBS Associate Professor, Neonatology Ellen M. Friedman, MD Professor, Otolaryngology

Contributors Joseph A. Garcia-Prats, MD Professor, Neonatology Mark A. Gilger, MD Professor, Gastroenterology, Hepatology, and Nutrition Nancy Glass, MD, MBA Professor, Pediatric Anesthesiology Stuart L. Goldstein, MD Professor, Pediatric Nephrology Imelda Celine Hanson, MD Professor, Pediatric Allergy and Immunology Morey W. Haymond, MD Professor, Pediatric Endocrinology Albert C. Hergenroeder, MD Professor, Adolescent and Sports Medicine Anthony Johnson, DO Associate Professor, Obstetrics and Gynecology Karen E. Johnson, MD Associate Professor, Neonatology

xv

Julie P. Katkin, MD Associate Professor, Pediatric Pulmonology Moise L. Levy, MD Clinical Professor, Dermatology and Pediatrics Laura L. Loftis, MD Associate Professor, Pediatric Critical Care Medicine Timothy E. Lotze, MD Assistant Professor, Pediatric Neurology Donald H. Mahoney, Jr., MD Professor, Pediatric Hematology and Oncology Tiffany M. McKee-Garrett, MD Assistant Professor, Neonatology Kenneth J. Moise, MD Professor, Obstetrics and Gynecology Kathleen J. Motil, MD, PhD Associate Professor, Gastroenterology, Hepatology, and Nutrition

Yvette R. Johnson, MD, MPH Assistant Professor, Neonatology

Eyal Muscal, MD, MS Assistant Professor, Pediatric Rheumatology

Juan Juarez, Jr., MD Assistant Professor, Pediatric Emergency Medicine

Barry L. Myones, MD Associate Professor, Pediatric Rheumatology

Henri Justino, MD Assistant Professor, Pediatric Cardiology

Steven R. Neish, MD, SM Associate Professor, Pediatric Cardiology

Arundhati S. Kale, MBBS Associate Professor, Pediatric Nephrology

Oluyinka A. Olutoye, MBChB, PhD Associate Professor, Pediatric Surgery, Obstetrics and Gynecology

Sheldon L. Kaplan, MD Professor, Pediatric Infectious Disease

Lu-Ann Papile, MD Professor, Neonatology

xvi

Contributors

Mary E. Paul, MD Associate Professor, Pediatric Allergy and Immunology Sarah M. Phillips, MD Instructor, Pediatric Gastroenterology, Hepatology, and Nutrition Lori A. Sielski, MD Associate Professor, Neonatology Geeta Singhal Das, MD Assistant Professor, Pediatrics Timothy Slesnick, MD Assistant Professor, Pediatric Cardiology Marianna M. Sockrider, MD, DrPH Associate Professor, Pediatric Pulmonology

Susanne Trout, RD, LD, IBCLC, RLC Clinical Dietitian, Clinical Nutrition Services Mohan Pammi Venkatesh, MD Assistant Professor, Neonatology Lakshmi Venkateswaran, MD Assistant Professor, Pediatric Hematology and Oncology David E. Wesson, MD Professor, Pediatric Surgery Eric A. Williams, MD Assistant Professor, Pediatric Critical Care Medicine

Michael E. Speer, MD Professor, Neonatology and Ethics

Mark L. Winter, PhD, DABAT Assistant Professor, Pharmacology and Toxicology University of Texas Medical Branch Galveston, Texas

C. Philip Steuber, MD Professor, Pediatric Hematology and Oncology

Teresa S. Wright, MD Assistant Professor, Pediatrics, Dermatology

V. Reid Sutton, MD Associate Professor, Molecular and Human Genetics

Parvin Yazdani-Kachooei, MD Assistant Professor, Pediatric Endocrinology

M. Hossein Tcharmtchi, MD Associate Professor, Pediatric Critical Care Medicine

Robert S. Zeller, MD Professor, Pediatric Neurology

Laura Torres, MD Associate Professor, Pediatric Anesthesiology

Preface Texas Children’s Hospital Handbook of Pediatrics and Neonatology is unlike any other pediatrics pocket manual. It is compact and concise, yet it thoroughly addresses both neonatology and pediatrics. It is written specifically to serve as an actual point-of-care tool; clinical algorithms, figures, and tables are abundant, promising rapid access to the information needed at the bedside. There is a remarkable online component to this handbook, in which the reader can freely access additional topics, updates, and supplementary information. This text is compact enough to comfortably carry in a white coat pocket; in fact, key medications and dosages are cited in-text in lieu of a separate formulary section. In addition, a critical care rapid reference card is included—an invaluable resource that provides immediate access to critical information in emergencies. Authored and reviewed by over 125 interns, residents, fellows, and faculty at Texas Children’s Hospital in Houston, Texas, Texas Children’s Hospital Handbook of Pediatrics and Neonatology collectively embodies the experiences and clinical pearls that are most helpful for students, trainees, and junior faculty. Distinguished faculty in virtually every pediatric discipline have authored and reviewed the content to ensure that this text reflects the current clinical practice at our institution. The practice of medicine is often as much an art as it is a science, and in areas where robust evidence does not exist, the opinions and recommendations of our experts at Texas Children’s Hospital are included. Many chapters and sections in this text are not found in other handbooks: eg, Clinical Pearls for the Wards; Neonatal and Pediatric Nutrition including “TPN in 10 Steps;” and Stabilization, Delivery Room Care, and Initial Treatment of the VLBW Infant, to name a few. You will find that Texas Children’s Hospital Handbook of Pediatrics and Neonatology offers concise, evidence-based information in an easy-to-access format that is directly applicable to bedside care of the patient in both pediatrics and neonatology. Although digital and electronic media have supplanted, in some cases, physical documents, handbooks, and texts, an effective pocket manual remains a crucial training tool, both as a repository for personal clinical experiences and as a point-of-care reference. We are certain that within this handbook you will find the most important and relevant information for the white-coat of the trainee in pediatrics and neonatology. Wherever possible, helpful resources, including internet addresses and literature references, have been included directly in the text. Texas Children’s Hospital Handbook of Pediatrics and Neonatology will continue to evolve; current updates, as well as a vast amount of additional content, are available at www.AccessPediatrics.com. We are confident that you will find this pocket manual invaluable as you learn the art and science of pediatrics and neonatology. Adam W. Lowry Kushal Y. Bhakta Pratip K. Nag

Abbreviations ↔, nl ↓ → ± ♀ ♂ Δ 1,25(OH)2-D 2/2 2,3-DPG 25(OH) D 5′-NT 6-MP AAA A-aDO2 AAP AAT Ab ABC abd ABER ABG ABNL ABPA ABR abx AC ACEI ACL acL ACOG ACS ACTH AD ADA ADC ADEM ADH ADHD ADL AED AET AFB

normal decreased, decreasing, depressed, down causes, results in, next possibly, consider female male change in 1,25-dihydroxy vitamin D secondary to 2,3-diphosphoglycerol 25-hydroxy vitamin D 5′-nucleotidase 6-mercaptopurine anti-actin antibody alveolar–arterial oxygen tension difference American Academy of Pediatrics α1 antitrypsin antibody airway, breathing, circulation abdominal auditory brainstem-evoked response arterial blood gas abnormal allergic bronchopulmonary aspergillosis auditory brainstem response antibiotics assist control angiotensin-converting enzyme inhibitor anterior cruciate ligament anticardiolipin American College of Obstetricians and Gynecologists acute chest syndrome adrenocorticotropic hormone autosomal dominant adenosine deaminase apparent diffusion coefficient acute disseminated encephalomyelitis antidiuretic hormone attention deficit hyperactivity disorder activity of daily living antiepileptic drug atrial ectopic tachycardia acid-fast bacilli

Abbreviations AFI AFP AG Ag AGE AGEP AHA AHI AI AIDS AIH AIHA AIN AKA AKI Al+++ Ala ALCAPA ALD Alk Phos ALL ALP ALT ALTE AMA AML AMMoL amox amp ampho B AN ANA ANCA ANLL anti-dsDNA Ab anti-GAD Ab anti-LC1 Ab anti-LKM1 Ab anti-LKM-1 anti-SLA/LPAb anti-TPOAb AOM AP APC APTT AR

xix

amniotic fluid index α-fetoprotein anion gap antigen acute gastroenteritis acute generalized erythematous pustulosis American Heart Association apnea-hypopnea index (number of apneas + hypopneas per hour on polysomnography) aortic insufficiency; autoimmune acquired immune deficiency syndrome autoimmune hepatitis autoimmune hemolytic anemia acute interstitial nephritis alcoholic ketoacidosis acute kidney injury aluminum alanine anomalous left coronary artery arising from the pulmonary artery adrenoleukodystrophy alkaline phosphatase acute lymphoblastic leukemia alkaline phosphatase alanine aminotransferase apparent life-threatening episode antimitochondrial antibody acute myelogenous leukemia acute myelomonocytic leukemia amoxicillin ampicillin amphotericin B anorexia nervosa antinuclear antibody antineutrophil cytoplasmic antibody acute nonlymphoblastic leukemia anti-double stranded DNA antibody anti-glutamic acid decarboxylase antibodies anti-liver cytosol-antibody anti-liver/kidney microsome antibody antibodies to liver/kidney microsomes anti-soluble liver/liver pancreas antigen antibody anti-thyroid peroxidase antibodies acute otitis media anterior posterior; aortopulmonary antigen-presenting cell activated partial thromboplastin time aortic regurgitation; autosomal recessive

xx

Abbreviations

ARB ARDS ARF AROM ARR ARVC AS ASA ASD ASMA Asp AST asx ATFL AT-II ATN ATP ATRA AUS AV AVB AVCD AVM AVN AVP AXR BA BAL BAS BCG BCx BDG BE β-HCG BG BID BiPAP B/L BLS BM BMA BMD BMI BMP BMT BN

angiotensin receptor blocker acute respiratory distress syndrome acute renal failure artificial rupture of membranes absolute risk reduction arrhythmogenic right ventricular cardiomyopathy aortic stenosis aminosalicylic acid (aspirin) atrial septal defect anti–smooth muscle antibody aspartate aspartate aminotransferase asymptomatic anterior talofibular ligament angiotensin-II acute tubular necrosis adenosine triphosphate all trans retinoic acid abdominal ultrasound arteriovenous; atrioventricular atrioventricular block atrioventricular canal defect arteriovenous malformation avascular necrosis arginine vasopressin abdominal X-ray bone age British antilewisite; bronchoalveolar lavage balloon atrial septostomy bacillus Calmette-Guérin blood culture bidirectional Glenn barium enema beta human chorionic gonadotropin blood glucose twice daily bilevel positive airway pressure bilateral basic life support bowel movement bone marrow aspirate Becker muscular dystrophy; bone mineral density body mass index basic metabolic panel (see Chem 7) bone marrow transplant bulimia nervosa

Abbreviations BNP BP BPD BPM BPO BSA BTS BUN BV BVH BW Bx C Ca CA CAA CAD CAH CAIS CA-MRSA c-ANCA cap refill CAP CAVC CBC CBD CBG CCAM CCB CCK CD CDC CDH CEA CF CFL CFTR CGA CGD CH50 CHAOS CHB CHD Chem 7

xxi

b-type natriuretic peptide blood pressure bronchopulmonary dysplasia beats per minute benzoyl peroxide body surface area Blalock-Taussig shunt blood urea nitrogen bacterial vaginosis biventricular hypertrophy birthweight biopsy Celsius calcium chronological age coronary artery aneurysm coronary artery disease congenital adrenal hyperplasia complete androgen insensitivity syndrome community-associated methicillin-resistant Staphylococcus aureus cytoplasmic antineutrophil cytoplasmic antibody capillary refill community-acquired pneumonia complete atrioventricular canal complete blood count common bile duct capillary blood gas congenital cystic adenomatoid malformation calcium channel blocker cholecystokinin clusters of differentiation; Crohn’s disease Centers for Disease Control and Prevention congenital diaphragmatic hernia carcinoembryonic antigen complement fixation; cystic fibrosis calcaneofibular ligament cystic fibrosis transmembrane conductance regulator corrected gestational age chronic granulomatous disease total hemolytic complement titer congenital high airway obstruction syndrome complete heart block; congenital heart block congenital heart disease chemistry panel (Na, K, Cl, HCO3, BUN, Cr, glucose)

xxii

Abbreviations

Chem 10 CHF CHO chol CI CK Cl CLD cm CMA CML CMV CN CNS CO CONS COPD CP CPAP CPD CPK CPP CPR CPS Cr CREST CRF CRI CRMO CRP CRPS CRS CRT CS C-section CSF CT CTA CTD CV CVA CVL CVP CVS Cx CXR

chemistry panel including all items of Chem 7 + Ca, Mg, Phos congestive heart failure carbohydrate cholesterol cardiac index; confidence interval creatine kinase chloride chronic lung disease centimeter chromosome microarray analysis chronic myeloid leukemia cytomegalovirus cranial nerve central nervous system carbon monoxide; cardiac output coagulase-negative Staphylococcus chronic obstructive pulmonary disease cerebral palsy continuous positive airway pressure citrate–phosphate–dextrose creatine phosphokinase cerebral perfusion pressure cardiopulmonary resuscitation carbamoylphosphate synthase creatinine calcinosis, Raynaud’s phenomenon, esophageal dysmotility, sclerodactyly, telangiectasias corticotropin-releasing factor chronic renal insufficiency chronic recurrent multifocal osteomyelitis C-reactive protein complex regional pain syndrome congenital rubella syndrome capillary refill time coronary sinus caesarean section cerebrospinal fluid computed tomography computed tomographic angiography connective tissue disease cardiovascular costovertebral angle central venous line central venous pressure cardiovascular system culture chest X-ray

Abbreviations cys d d/c d/o D10 D10W D25 DA DBP DC DCM DDAVP DDH DDx DEXA DFA DFS DH DHEA DHEAS DHN DHS DI DIC diff DILV DIOS DIP div DKA DKS dL DLCO DM DMARD DMD DMSA DMSO DNA DOL DORV DRESS DSD DSM-IV DTaP

xxiii

cysteine day discontinue disorder(s) 10% dextrose 10% dextrose in sterile water 25% dextrose ductus arteriosus diastolic blood pressure direct Coombs test dilated cardiomyopathy deamino-8-d-arginine vasopressin (desmopressin) developmental dysplasia of the hip differential diagnosis dual energy x-ray absorptiometry direct fluorescent antibody disease-free survival direct hyperbilirubinemia dehydroepiandrosterone dehydroepiandrosterone sulphate dehydration drug hypersensitivity syndrome diabetes insipidus disseminated intravascular coagulation differential double-inlet left ventricle distal intestinal obstruction syndrome distal interphalangeal divided diabetic ketoacidosis Damus-Kaye-Stansel deciliter diffusing lung capacity diabetes mellitus; dermatomyositis disease-modifying antirheumatic drug Duchenne muscular dystrophy dimercaptosuccinic acid dimethyl sulfoxide deoxyribonucleic acid day of life double-outlet right ventricle drug reaction with eosinophilia and systemic symptoms disorders of sex differentiation Diagnostic and Statistical Manual of Mental Disorders, 4th ed diphtheria toxoid, tetanus toxoid, and acellular pertussis

xxiv

Abbreviations

d-TGA DTR DUB DVT DWI dx dz EB EBM EBV eCCl ECF ECG ECHO ECMO ED EEC EEG EFS eg EGD EHK EIA EKG ELBW ELISA EM EMA EMB EMG endo ENT EOG EPAP EPS ERCP ERV ESR ESRD ETCO2 ETEC ETOH ETT EUS EWS F f/b

d-transposition of the great arteries deep tendon reflex dysfunctional uterine bleeding deep venous thrombosis diffusion weighted imaging diagnosis disease epidermolysis bullosa evidence-based medicine, expressed breast milk Epstein-Barr virus estimated creatinine clearance extracellular fluid electrocardiogram echocardiogram extracorporeal membrane oxygenation emergency department ectodermal dysplasia, ectrodactyly, cleft electroencephalogram event-free survival for example esophagogastroduodenoscopy epidermolytic hyperkeratosis enzyme immunoassay electrocardiogram extremely low birth weight enzyme-linked immunoassay electron microscopy endomysial antibodies ethambutol electromyogram endocrine Ears, nose, and throat electrooculogram expiratory positive airway pressure extrapyramidal symptoms endoscopic retrograde cholangio– pancreatography expiratory reserve volume erythrocyte sedimentation rate end-stage renal disease end-tidal carbon dioxide enterotoxigenic Escherichia coli alcohol (ethanol) endotracheal tube endoscopic ultrasonography Ewing sarcoma Fahrenheit followed by

Abbreviations FB FDA FDP FE FEF25%–75% FENa FEV1 FFP FHx FiO2 FISH fL FLAIR FOB FOC Fr FRC FS FSBG FSGS FSH FT4 FTA-ABS FTT f/u FUO FVC Fx g G6PD GA GABA GABHS GALT GAS GBM GBS GC GCS GCSF GCT GDM GE GER GERD GFR GGT GH

xxv

foreign body Food and Drug Administration fibrin degradation product fractional excretion forced midexpiratory flow rate fractional excretion of sodium forced expiratory volume in 1 second fresh-frozen plasma family history fractional inspired oxygen fluorescent in-situ hybridization femtoliter fluid attenuated inversion recovery fecal occult blood frontal–occipital circumference French functional residual capacity fingerstick fingerstick blood glucose focal segmental glomerulosclerosis follicle-stimulating hormone free thyroxine fluorescent treponemal antibody absorption failure to thrive follow-up fever of unknown origin forced vital capacity fracture gram glucose-6-phosphate dehydrogenase gestational age γ-aminobutyric acid group A β-hemolytic streptococcus galactose-1-phosphate uridyltransferase group A streptococcus glioblastoma multiforme group B streptococcus; Guillain-Barré syndrome glucocorticoid; gonococcus Glasgow Coma Scale granulocyte colony-stimulating factor germ cell tumor gestational diabetes mellitus gastroenteritis gastroesophageal reflux gastroesophageal reflux disease glomerular filtration rate γ-glutamyl transpeptidase growth hormone

xxvi

Abbreviations

GHB GHBP GI GIR Glc Gln Glu Gly GN GNR GnRH GSD GT GU GVHD h H&P HO h/o H+/K+ ATPase H2RA HA HAV Hb HBcAb HBeAg Hb F HBIG HBsAg HCC hCG HCM HCO3− HCV HD HDAg HDL HDV HEENT HELLP Hep B HEV HFOV HbA1C HH

δ-hydroxybutyrate growth hormone binding protein gastrointestinal glucose infusion rate glucose glutamine glutamic acid glycine glomerulonephritis gram-negative rod gonadotrophin-releasing hormone glycogen storage disease genotype genitourinary graft-versus-host disease hour history and physical examination house officer history of hydrogen/potassium adenosine triphosphatase (proton pump) histamine-2-receptor antagonist headache hepatitis A virus hemoglobin hepatitis B core antibody hepatitis B e antigen hemoglobin F hepatitis B immune globulin hepatitis B surface antigen hepatocellular carcinoma human chorionic gonadotropin hypertrophic cardiomyopathy bicarbonate hepatitis C virus hemodialysis; Hirschsprung’s disease; Hodgkin’s disease hepatitis D antigen high-density lipoprotein hepatitis D virus head, eyes, ears, nose, and throat hemolytic anemia, elevated liver enzymes, low platelets hepatitis B hepatitis E virus high-frequency oscillatory ventilation glycosylated hemoglobin A1c hereditary hemochromatosis

Abbreviations HHRH HHS HHV Hib HIDA HIE HIG HIT HIV HLH HLHS HMG-CoA HOCM HPA HPF HPV HR hr HRCT HSCT HSM HSP HSR HSV ht HTN HUS HVA Hx I&D IA IAA IAP IBD IBS IBW I:C IC iCa ICA ICD ICP ICS ICU ID IDDM IDM

xxvii

hereditary hypophosphatemic rickets with hypercalciuria hyperglycemic hyperosmolar state human herpesvirus Haemophilus influenzae type b hepatobiliary iminodiacetic acid hypoxic-ischemic encephalopathy human immune globulin heparin-induced thrombocytopenia human immunodeficiency virus hemophagocytic lymphohistiocytosis hypoplastic left heart syndrome 3-hydroxy-3-methylglutaryl–CoA hypertrophic obstructive cardiomyopathy human platelet antigen high-power field human papillomavirus heart rate hours high-resolution computed tomography hematopoietic stem cell transplant hepatosplenomegaly Henoch-Schonlein purpura hypersensitivity reaction herpes simplex virus height hypertension hemolytic uremic syndrome homovanillic acid history incision and drainage insulin antibodies insulin autoantibody; interrupted aortic arch intrapartum antibiotic prophylaxis inflammatory bowel disease irritable bowel syndrome ideal body weight insulin to carbohydrate inspiratory capacity ionized calcium islet cell antibody implantable cardioverter/defibrillator intracranial pressure inhaled corticosteroids intensive care unit infectious disease insulin-dependent diabetes mellitus infant of a diabetic mother

xxviii

Abbreviations

ie I:E IE IEM IF IFA IGFBP-3 Ig IgA IgE IGF IGF-BP IgG IgM IGT IHA IHS IHST ILD Ile IM INH iNO INR IO IP IPAP IPV IR IRT IRV IT ITP Itra IU IUD IUGR IV IVC IVDU IVF IVH IVIG IVS JAS JCA JDMS

that is ratio of inspiratory time to expiratory time infective endocarditis inborn errors of metabolism immunofluorescence immunofluorescent antibody insulin-like growth factor binding protein 3 immunoglobulin immunoglobulin A immunoglobulin E insulin-like growth factor insulin-like growth factor binding protein immunoglobulin G immunoglobulin M impaired glucose tolerance indirect hemagglutination assay International Headache Society immediate hypersensitivity skin test interstitial lung disease isoleucine intramuscular isoniazide inhaled nitric oxide International Normalized Ratio intraocular; intraosseous interphalangeal inspiratory positive airway pressure intrapulmonary percussive ventilation internal rotation immunoreactive trypsinogen inspiratory reserve volume iliotibial; intrathecal idiopathic thrombocytopenic purpura itraconazole international units intrauterine device intrauterine growth restriction intravenous inferior vena cava IV drug user intravenous fluids; in-vitro fertilization intraventricular hemorrhage intravenous immunoglobulin intact ventricular septum; interventricular septum juvenile ankylosing spondylitis juvenile chronic arthritis juvenile dermatomyositis

Abbreviations JEB JIA JIIM JMML JRA JVD JVP K KCl KD kg KI KOH KUB L LA LABA LCT LAD LAE lb LBBB LBW LCA LCFA LCH LDH LDL LES Leu LFT

LGA LH Li LIP LLSB LM LMP LMWH LOC LP LPA

xxix

junctional epidermolysis bullosa juvenile idiopathic arthritis juvenile idiopathic inflammatory myopathies juvenile myelomonocytic leukemia juvenile rheumatoid arthritis jugular venous distension jugular venous pulse or pulsation potassium potassium chloride Kawasaki disease kilogram potassium iodide potassium hydroxide kidneys, ureters, and bladder x-ray (abdominal x-ray) liter left atrium; lupus anticoagulant long-acting β-agonist long-chain triglycerides lymphadenopathy; left axis deviation; left anterior descending (coronary artery) left atrial enlargement pounds left bundle branch block low birthweight left coronary artery long-chain fatty acids Langerhans cell histiocytosis lactate dehydrogenase low-density lipoprotein lower esophageal sphincter leucine liver function test (liver panel); generally includes at a minimum albumin, total protein, bilirubin (total and direct), alanine and aspartate aminotransferases, alkaline phosphatase large for gestational age luteinizing hormone lithium lymphoid interstitial pneumonia left lower sternal border light microscopy last menstrual period low-molecular-weight heparin level of consciousness lumbar puncture left pulmonary artery

xxx

Abbreviations

LQTS LR LRT LRTI LTBI LUSB LV LVEDP LVH LVNC LVOT LVOTO Lys Lytes m2 MAC MAG-3 MAI MAP MAS MCD mcg MCH MCL MCP MCT MCTD MCV MD MDI MDS MEE MELAS MEN mEq Met Mg mg MGN MH MHA-TP MHC MIBG micro

long QT syndrome lactated Ringer’s solution; likelihood ratio lower respiratory tract lower respiratory tract infection latent tuberculosis infection left upper sternal border left ventricular left ventricular end-diastolic pressure left ventricular hypertrophy left ventricular noncompaction left ventricular outflow tract left ventricular outflow tract obstruction lysine electrolytes (Na, K, Cl, often includes bicarbonate) meter squared Mycobacterium avium complex mercaptoacetyltriglycine Mycobacterium avium intracellulare mean airway pressure macrophage activation syndrome; meconium aspiration syndrome minimal change disease microgram mean corpuscular hemoglobin medial collateral ligament metacarpophalangeal medium-chain triglycerides mixed connective tissue disease mean corpuscular volume myotonic dystrophy metered-dose inhaler myelodysplastic syndrome middle ear effusion mitochondrial encephalopathy, lactic acidosis, and stroke-like episodes multiple endocrine neoplasia milliequivalent methionine magnesium milligram membranous glomerulonephritis microscopic hematuria microhemagglutination assay for antibodies to Treponema pallidum major histocompatibility class methyliodobenzylguanidine microanalysis; microscopic

Abbreviations min MIVF mL MM MMR mo MODY MPA MPGN MPH MPO MPS MR MRA MRCP MRD MRI MRS MRSA MRV MS MSAFP MSG MSM MSSA MSUD MT MTB MTHFR MTP MTX MV MVA MVP Na N/A NAAT NAC NAI NAIT NAS NASH NASPGHAN NAT NB NBL NBS

xxxi

minutes maintenance intravenous fluids milliliter mucous membrane measles, mumps, rubella months maturity onset diabetes of youth main pulmonary artery; milk protein allergy membranoproliferative glomerulonephritis mid-parental height myeloperoxidase mucopolysaccharidosis mitral regurgitation magnetic resonance angiography magnetic resonance cholangiopancreatography minimal residual disease magnetic resonance imaging magnetic resonance spectroscopy methicillin-resistant Staphylococcus aureus magnetic resonance venogram mental status; mitral stenosis maternal serum alpha-fetoprotein monosodium glutamate men who have sex with men methicillin-sensitive Staphylococcus aureus maple syrup urine disease metatarsal Mycobacterium tuberculosis methyltetrahydrofolate reductase metatarsophalangeal methotrexate minute volume; mitral valve motor vehicle accident mitral valve prolapse sodium not applicable nucleic acid amplification test N-acetylcysteine non-accidental injury neonatal alloimmune thrombocytopenia neonatal abstinence scoring nonalcoholic steatohepatitis North American Society for Pediatric Gastroenterology, Hepatology and Nutrition non-accidental trauma nota bene neuroblastoma newborn screen

xxxii

Abbreviations

NC NCPAP NEC NF ng NG NGT NH3 NHL NICU NIH NIV NK nl NLE NMB NMJ NMT NNT NPO NS NSAID NSR NTB NTD N/V O&P O2 OA OAE OCD OCP OCRG OEIS OG OGES OGTT OHV OI OLT OME ONTD OP ophth ORIF Orn

nasal cannula nasal continuous positive airway pressure necrotizing enterocolitis neurofibromatosis nanograms nasogastric nasogastric tube ammonia non-Hodgkin’s lymphoma neonatal intensive care unit National Institutes of Health noninvasive ventilation natural killer normal neonatal lupus erythematosus neuromuscular blockade neuromuscular junction nebulized mask treatment number needed to treat nil per os (nothing by mouth) normal saline nonsteroidal antiinflammatory drug normal sinus rhythm nontuberculous neural tube defect nausea/vomiting ova and parasite oxygen organic acids otoacoustic emissions obsessive–compulsive disorder; osteochondritis dissecans oral contraceptive oxycardiorespirogram omphalocele, cloacal exstrophy, imperforate anus, spinal defects orogastric oral glucose–electrolyte solution oral glucose tolerance test obesity hypoventilation syndrome obesity imperfecta orthotopic liver transplantation otitis media with effusion open neural tube defect organophosphate ophthalmic solution open reduction with internal fixation ornithine

Abbreviations ORS Osm OSAS Osm OT OTC oz Phos P2 PA PAA PAB PaCO2 PAF PAH PA/IVS PALS PAN p-ANCA PaO2 Pap PAPP-A PAPVC PAPVR PAS PB PBF PC PCA PCL PCN PCOS PCP PCR PCV PCV7 PD PDA PDD PDE PE PEEP PEF PEG

xxxiii

oral rehydration solution obstructive sleep apnea obstructive sleep apnea syndrome osmolality occupational therapy ornithine transcarbamylase; over-the-counter ounces phosphorus pulmonic component of second heart sound postero-anterior; pulmonary atresia; pulmonary artery plasma amino acids pulmonary artery band arterial carbon dioxide tension (partial pressure) platelet-activating factor pulmonary artery hypertension pulmonary atresia with intact ventricular septum pediatric advanced life support polyarteritis nodosa perinuclear antineutrophil cytoplasmic antibody partial pressure of oxygen in arterial blood Papanicolaou pregnancy-associated plasma protein A partial anomalous pulmonary venous connection partial anomalous pulmonary venous return periodic acid-Schiff barometric pressure (~760 mm Hg at sea level) pulmonary blood flow platelet count; pressure control patient-controlled analgesia posterior collateral ligament of knee penicillin polycystic ovarian syndrome phencyclidine; primary care provider polymerase chain reaction packed cell volume 7-valent pneumococcal conjugate vaccine peritoneal dialysis patent ductus arteriosus pervasive developmental disorder phosphodiesterase physical exam; pulmonary embolism, pulmonary edema positive end-expiratory pressure peak expiratory flow polyethylene glycol

xxxiv

Abbreviations

PEG 3350 PEP PET PFC PFO PFT PGE1 PHA Phe Phos PI PICC PICU PID PIE PIGN PIP PIV PK PKD PKU PMA PMD PMH PMI PML PMN PNET PNH PO PO2 POC POPE PPD PPHN PPI PPNET PPROM PPS PPV PR PRBC Pro PROM

polyethylene glycol powder (Miralax) positive expiratory pressure positron emission tomography persistent fetal circulation patent foramen ovale pulmonary function testing prostaglandin E1 pseudohypoaldosteronism phenylalanine phosphorous primary immunodeficiency, protease inhibitor percutaneously inserted central catheter pediatric intensive care unit pelvic inflammatory disease pulmonary interstitial emphysema post-infectious glomerulonephritis peak inflation pressure; peak inspiratory pressure; proximal interphalangeal peripheral intravenous (catheter) pyruvate kinase polycystic kidney disease phenylketonuria postmenstrual age primary medical doctor past medical history point of maximal impulse promyelocytic leukemia polymorphonuclear cells primitive neuroectodermal tumor paroxysmal nocturnal hemoglobinuria per os (by mouth) partial pressure of oxygen point of care postobstructive pulmonary edema purified protein derivative persistent pulmonary hypertension of newborn proton pump inhibitor peripheral primitive neuroectodermal tumor preterm prolonged rupture of membranes peripheral pulmonary stenosis positive predictive value; positive pressure ventilation per rectum; pityriasis rosea; pulmonary regurgitation packed red blood cell proline passive range of motion; prolonged rupture of membranes

Abbreviations PS PSC PSG PSH pSLE pt PT PTFL PTH PTHrP PTSD PTT PTX PUBS PUD pulm PUV PUVA PVL PVM PVR PWS Px PZA Q Qhs QID QIg Qp Qs QTc RA RAA RAD RAE RAST RBBB RBC RCA RCM RCT RDEB RDS RDW RES resp retic RF

xxxv

pressure support; pulmonary stenosis primary sclerosing cholangitis polysomnography past surgical history pediatric systemic lupus erythematosus patient physical therapy; prothrombin time posterior talofibular ligament parathyroid hormone persistent parathyroid hormone–related peptide posttraumatic stress disorder partial thromboplastin time pneumothorax percutaneous umbilical blood sampling peptic ulcer disease pulmonary posterior urethral valve psoralen with ultraviolet A periventricular leukomalacia pulmonary vascular markings pulmonary vascular resistance port-wine stain prognosis pyrazinamide every every night four times a day quantitative immunoglobulins pulmonary blood flow systemic blood flow corrected QT interval rheumatoid arthritis, right atrium right atrial appendage right axis deviation right atrial enlargement radioallergosorbent test right bundle branch block red blood cell right coronary artery restrictive cardiomyopathy randomized, controlled trial recessive dystrophic epidermolysis bullosa respiratory distress syndrome red cell distribution width reticuloendothelial system respiratory reticulocyte rheumatoid factor

xxxvi

Abbreviations

RHD RIA RIF RNA RNC RNP r/o ROM ROP ROS RP RPGN RPR rpt RR RRR RSV RTA RUQ RV RVEDP RVH RVOT RVOTO Rx rxn SAA SABA SaO2 SAPHO SBBO SBE SBFT SBI SBP SBS SCD SCFE SCID SCM SCT SD SE SEM SES SG SGA

rheumatic heart disease radioimmunoassays rifampin ribonucleic acid radionuclide cystogram ribonucleoprotein rule out range of motion retinopathy of prematurity review of systems Raynaud’s phenomenon rapidly progressive glomerulonephritis rapid plasma reagin repeat respiratory rate; risk ratio relative risk reduction respiratory syncytial virus renal tubular acidosis right upper quadrant residual volume; right ventricle right ventricular end-diastolic pressure right ventricular hypertrophy right ventricular outflow tract right ventricular outflow tract obstruction prescription; treatment reaction serum amino acids; serum amyloid A short-acting β-agonist systemic arterial oxygen saturation syndrome of synovitis, acne, pustulosis, hyperostosis, and osteitis small bowel bacterial overgrowth subacute bacterial endocarditis small bowel follow-through serious bacterial infection systolic blood pressure short bowel syndrome sickle cell disease; sudden cardiac death slipped capital femoral epiphysis severe combined immunodeficiency sternocleidomastoid stem cell transplant standard deviation side effects systolic ejection murmur socioeconomic status specific gravity small for gestational age

Abbreviations SGS SHBG SI SIADH SIDS SIMV SIRS SJS SLE SMA SMV SOB SPECT SpO2 s/p spp. SSRI SSTI Staph STI STS sub-Q SVC SVCO2 SVR SVT SWI sx T&A T1DM T3 T4 TA TAPVC TAPVR TAR TB TBG TBI TBSA TBW TCA TcB TCH TCI TCS

xxxvii

short gut syndrome steroid hormone–binding globulin sacroiliac syndrome of inappropriate antidiuretic hormone secretion sudden infant death syndrome synchronized intermittent mandatory ventilation systemic inflammatory response syndrome Stevens-Johnson syndrome systemic lupus erythematosus spinal muscular atrophy; superior mesenteric artery superior mesenteric vein shortness of breath single-photon emission computed tomography oxygen saturation status post species selective serotonin reuptake inhibitors skin and soft tissue infections staphylococcus sexually transmitted infection soft tissue sarcoma subcutaneous superior vena cava superior vena cava oxygen saturation systemic vascular resistance supraventricular tachycardia susceptibility weighted imaging symptoms tonsillectomy & adenoidectomy type 1 diabetes mellitus triiodothyronine thyroxine tricuspid atresia; truncus arteriosus total anomalous pulmonary venous connection total anomalous pulmonary venous return thrombocytopenia with absent radius tuberculosis thyroid-binding globulin traumatic brain injury total body surface area total body water tricyclic antidepressant transcutaneous bilirubin Texas Children’s Hospital topical calcineurin inhibitor topical corticosteroid

xxxviii

Abbreviations

Td TDID TDx-FLM Te TEE TEF TEG TEN TFPI TFT TG TGA TH Ti TIBC TID TIPS TLC TLSO TM TMJ TMP TMP/SMX TNF TOA TOF TOF/PA TORCH tox TP tPA TPN TR TRALI TRH TS TSB TSH TSS TST TT4 TTE TTG TTN TTP TV tx

tetanus toxoid, diphtheria toxoid total daily insulin dose (commercial fetal lung maturity assay) expiratory time transesophageal echocardiography tracheoesophageal fistula thromboelastography platelet mapping toxic epidermal necrolysis tissue factor pathway inhibitor thyroid function test triglycerides transposition of the great arteries thyroid hormone inspiratory time total iron-binding capacity three times daily transjugular intrahepatic portosystemic shunt total lung capacity thoracic lumbosacral orthosis tympanic membrane temporomandibular joint trimethoprim trimethoprim/sulfamethoxazole tumor necrosis factor tubo-ovarian abscess tetralogy of Fallot tetralogy of Fallot with pulmonary atresia toxoplasmosis, other (varicella, parvovirus), rubella, cytomegalovirus, herpes, HIV, syphilis toxicology total protein tissue plasminogen activator total parenteral nutrition tricuspid regurgitation transfusion-related acute lung injury thyroid-releasing hormone tricuspid stenosis total serum bilirubin thyroid-stimulating hormone toxic shock syndrome tuberculin skin test total thyroxine transthoracic echocardiography tissue transglutaminase transient tachypnea of newborn thrombotic thrombocytopenic purpura tricuspid valve; tidal volume therapy

Abbreviations Tyr UA UAC UAG UAW UC UCL UDCA UFH UGI UGT U/L UOA UOP UProt:UCr UPJ UPT URAP URI URTI US UTI UV UVB UVC V/D VACTERL

Val VATER

VATS VBG VC VCO2 VCUG VD VDRL VG VHL VIP VLBW VLDL VMA

xxxix

tyrosine urinalysis umbilical arterial catheter urine anion gap upper airway ulcerative colitis ulnar collateral ligament ursodeoxycholic acid unfractionated heparin upper gastrointestinal series uridine diphosphate glucuronosyltransferase upper to lower body segment ratio urine organic acids urine output urinary protein to urinary creatinine ratio ureteropelvic junction urine pregnancy test unidirectional retrograde accessory pathway upper respiratory infection upper respiratory tract infection ultrasonography urinary tract infection ultraviolet ultraviolet B umbilical venous catheter vomiting/diarrhea vertebral anomalies, anal atresia, cardiovascular anomalies, tracheoesophageal fistula, esophageal atresia, renal or radial anomalies, and limb defects valine vertebral anomalies, anal atresia, tracheoesophageal fistula, esophageal atresia, and renal or radial anomalies video-assisted thoracoscopic surgery venous blood gas vital capacity; volume control CO2 production voiding cystourethrography; voiding cystourethrogram dead space Venereal Disease Research Laboratory volume guarantee von Hippel-Lindau vasoactive intestinal polypeptide very low birth weight very low-density lipoprotein vanillylmandelic acid

xl

Abbreviations

VO2 VOD VPA VPS V/Q VRE VS VSD VT VT VTE VU VUR vWD vWF VZIG VZV w/u WBC WBG wk WNL WPW WT wt XLHR XR y yo yr ZES

oxygen uptake veno-occlusive disease valproic acid ventriculoperitoneal shunt ventilation-perfusion vancomycin-resistant Enterococcus volume support ventricular septal defect tidal volume ventricular tachycardia venous thromboembolism vesicoureteral vesicoureteral reflux von Willebrand disease von Willebrand factor varicella zoster immunoglobulin varicella zoster virus work up white blood cell whole-blood glucose week within normal limits Wolff-Parkinson-White syndrome Wilms tumor weight X-linked hypophosphatemic rickets X-ray year years old years Zollinger-Ellison syndrome

CHAPTER 1

Clinical Pearls for the Wards ORDERS Admission Orders (ADC VANDALISM) • Admit to: Floor, service, MD • Diagnoses (prioritized) • Condition: Good, fair, poor, guarded, critical • Vitals and monitoring: Frequency of monitoring (eg, q4, q shift, per routine), type of monitoring (eg, continuous oximetry, telemetry, arterial line, CVP, end-tidal CO2) • Activity: Ad lib, bed rest with or without bathroom privileges, crib with side rails up, restrictions, ambulate TID, and so on • Nursing/respiratory: strict I/O, daily weights, turn patient q shift, dressing care and changes, drain care, NG care, Foley care, suctioning, pulmonary toilet • Diet: Regular, clear liquid, special requirements (ie, ADA, low fat, low calorie), restrictions (ie, 2-g sodium renal diet), NPO • Allergies: Medication and food • Labs • IVF: Type, volume, rate (specify mL/hr for all; for infants, also specify mL/kg/d) • Studies • Medications: Name, dose (also specify mg/kg), frequency, route, duration, reason

Preoperative Orders • Diagnoses (prioritized) • Procedure • Preoperative labs (including blood bank orders)

• Preoperative studies • Diet: NPO/IVF after midnight, and so on • Consent form signed and on chart • H&P reviewed

Discharge Orders • Discharge: When, to where • Diagnoses (prioritized) • Diet • Condition • Activity: Ad lib, bedrest, physical limitations, and so on

• Special needs: Home health needs, monitoring, and so on • Discharge medications • Discharge instructions: When and why to return, where to return, and so on • Follow-up appointments

NOTES On-Service Note • Admit date • Admit diagnoses (prioritized) • Hospital course summary • Physical examination

• Problem list (prioritized) • Assessment or plan (problem based or system based)

Progress Note (SOAP Note) Subjective: Patient comments or complaints, nursing comments, relevant events Objective: • Vitals: Temperature, HR, RR, blood pressure, oxygen saturation, weight (including change from previous) • I/O: Totals and components of IVF, PO intake, emesis, residuals, urine, stool, drains

1

G E N E R A L C L I N I C A L I N F O R M AT I O N

Section 1: General Clinical Information

2

General Clinical Information

• Physical examination (focused) • Medicines: All current medicines with weight-based dose (scheduled and prn) • Laboratory and test data: New or pending Assessment: Analysis of above, including differential dx or tentative dx Plan (problem based or system based)

Discharge Summary (usually dictated) • Admission and discharge date • Admission and discharge diagnoses (prioritized) • Service: Service name, attending physician, resident(s) • Consulting services • Procedures • Physical examination and vitals (admission) • Hospital course (system based or problem based, pertinent labs and studies) • Physical examination and vitals (discharge) • Discharge condition: Improved, good

• Disposition: To outside hospital, home, hospice, and so on • Discharge medications: Name, formulation, dosage, length of treatment, refills • Discharge activity • Discharge diet • Discharge instructions: Dressing or cast care, symptoms to warrant further treatment, where to return for further treatment, and so on • Follow-up appointments

PRESCRIPTION WRITING: ESSENTIAL COMPONENTS Identifying Information: Name, date, weight, DOB Rx: Drug name, strength, formulation (ie, amoxicillin 250mg/5mL suspension) SIG: Quantity (mL, tablets, capsules, puffs, and so on), route, frequency, duration (specify mg/kg when possible) DISP: Number or volume to be dispensed Other: Number of refills? Substitution acceptable? Flavoring acceptable? Language?

GROWTH PEARLS WEIGHT, HEIGHT, AND HEAD CIRCUMFERENCE: 5TH, 50TH, AND 95TH PERCENTILES Boys Age∗

Height (cm)

Weight (kg)

FOC (cm)

0 mo

46-50-54

2.5-3.5-4.3

32-36-39

0.5 mo

49-53-57

3.0-4.0-4.9

34-37-40

1.5 mo

53-57-61

3.8-4.9-6.0

36-39-42

3.5 mo

58-62-67

5.2-6.4-7.8

39-42-44

6.5 mo

64-68-73

6.7-8.2-9.9

42-44-46

9.5 mo

68-72-77

7.9-9.5-11.4

43-45-48

1 yr

72-76-81

8.8-10.5-12.6

44-46-48

2 yr†

82-88-94

10.7-12.7-15.3

46-49-51

3 yr

89-95-102

12.0-14.3-17.4

47-50-52

4 yr

96-103-110

13.6-16.3-20.3

—

5 yr

101-109-117

15.2-18.5-23.5

—

10 yr

128-139-150

24.9-32.1-46.1

— (continued on next page)

3

Girls Age∗

Height (cm)

Weight (kg)

FOC (cm)

0 mo

46-49-54

2.5-3.4-4.2

32-35-38

0.5 mo

48-52-56

2.9-3.8-4.6

34-36-39

1.5 mo

51-55-59

3.5-4.5-5.5

36-38-41

3.5 mo

56-60-65

4.7-5.9-7.1

38-40-43

6.5 mo

62-66-70

6.1-7.5-9.0

41-43-45

9.5 mo

66-71-75

7.2-8.7-10.4

42-44-46

1 yr

69-74-79

8.1-9.7-11.6

43-45-47

2 yr

80-86-92

10.3-12.1-14.7

45-48-50

3 yr

88-94-101

11.6-13.9-17.2

46-49-51

4 yr

94-101-108

13.1-15.9-20.4

—

5 yr

100-108-116

14.7-18.0-23.8

—

10 yr

127-138-150

24.8-33.1-48.2

—

†

∗Data available in half-month increments; thus, values for 1 year are actually for 12.5 m, 2-year values are for 24.5 mo, and so on. † Data obtained from a 0- to 36-month-old cohort; values are slightly different in the 2- to 20-year cohort.

Weight • Average birth weight: 3.2 kg (girls); 3.6 kg (boys) • Regain birthweight by 7 to 14 days (7–10 days for term infants; 10–14 days for preterm infants) • Doubles in 4 mo, triples in 12 mo, quadruples in 24 mo Weight (Rate of Gain) • 0–3 mo → 20–30 g/d • 3 mo–6 mo → 20 g/d • 6 mo–1 yr → 10 g/d or 1 lb/mo

• 2 yr–puberty → 0.5 lb/mo or 2 kg/yr∗ (∗Abnormal prepubertal velocity = 10 kg) BSA = (kg × 4) + 7/(90 + kg)

Mosteller’s Formula (N Engl J Med. 1987;317(17):1098)

BSA (m2 ) =

height (cm) × weight (kg) 3,600

PRE-PROCEDURAL DIET GUIDELINES Procedure

Diet Recommendations

Barium enema

Normal∗

Bone scan

Normal

Bronchoscopy

NPO: 4–6 h (continued on next page)

Procedure

Diet Recommendations

CT scan with contrast: Abdomen, body

NPO: 6 h, food and formula; 4 h, EBM; 2 h, clears

CT scan with or without contrast: Head

Normal

3-D CT scan: Head

NPO: 6 h, food and formula; 4 h, EBM; 2 h, clears

Echocardiography (sedated)

NPO: 4 h, food and formula; 2 h, EBM and clears

Fluoroscopy

Normal

Gastric emptying study

NPO: 4 h

HIDA scan

6 mo, NPO 4 h

Interventional radiology procedure

NPO: 6 h, food and formula; 4 h, EBM; 2 h clears

Milk scan

NPO: 4 h

MRI (when sedation is necessary)

NPO: 6 h, food and formula; 4 h; EBM; 2 h, clears

NG / NJ tube insertion

Normal

Renal flow with magnesium

Normal

Renal scan DMSA

Normal

Renal scan with Lasix

Normal

Swallow study

NPO: 2–3 h

Ultrasonography, abdominal

NPO: 4 h

Ultrasonography, hip

Normal

Ultrasonography, renal

Normal

Upper GI (UGI) with or without small bowel follow-through

NPO: 4 h

VCUG

Normal

5

∗no specific pre-procedural limitations/restrictions

EAR WAX MANAGEMENT Why Not Remove Cerumen? • Natural cerumen turnover: Produced in the outer third of the external canal; daily chewing and growth of the ear canal lining slowly transition cerumen outward • Contains natural bacteriostatic agents • Protects the eardrum from external debris • Protects the lining of the ear canal Cerumen Impaction Affects ~10% of children, 5% adults, and 35% of adults and children with mental retardation; often secondary to anatomy; increased number of external auditory canal hairs, use of devices such as cotton swabs or ear candles Cerumen Removal (evidence limited) • Indicated: Cerumen impaction, hearing loss 2/2 cerumen, physical examination of the outer ear structures or tympanic membrane. • Methods: Manual, ceruminolytics, irrigation. • In trials, no ceruminolytic is consistently superior to saline; therefore, saline is the firstline ceruminolytic; it can be left in the ear canal for 15 to 30 min after failed irrigation attempt before repeating (Br J Gen Pract. 2004;54:862–867).


Clinical Pearls for the Wards

6


• First irrigation unsuccessful → Ceruminolytic for 15 to 30 min; repeat • Second irrigation unsuccessful → Ceruminolytic BID for 2 to 3 d; repeat • Third irrigation unsuccessful → Consider ENT referral

CERUMINOLYTIC PREPARATIONS∗

Water Based (Increase Miscibility)

Oil Based (Lubricate)

Docusate sodium (Colace™ liquid)† Triethanolamine (Cerumenex™)† 3% Hydrogen peroxide 2.5% Acetic acid 10% Sodium bicarbonate Normal saline‡

Almond oil Olive oil Peanut oil Mineral oil 10% oil of turpentine (Cerumol™)

Non oil, Non–Water Based (Increase Miscibility) Carbamide peroxide (Debrox™)† Glycerol

∗None of these drugs are FDA approved for use in children. † Recommended second-line agent or for prescription home use. ‡ Recommended first-line agent.

Ear Wax Removal Indicated

Manual Removal

Ceruminolytics*

• Metal or plastic loop or spoon (never pointed or sharp) • Difficult if uncooperative patient or if cerumen impacted

• Fill ear canal on affected side, cotton ball in canal • Saline recommended as first line trial • Wait 15–30 min prior to irrigation • Use 2–3 days BID if unsuccessful

Irrigation*

†

• Use body temperature water (35–38°C) with rubber ear syringe, Water-Pik™ on low setting, or improvised device‡ • Irrigate repeatedly until water return is clear

Figure 1-1 Ear wax removal methods. ∗Avoid in all patients with tympanostomy tubes, suspected perforation of the tympanic membrane, or vertigo. †Ceruminolytic use before irrigation dramatically increases the success rate. ‡A 20- to 30-cc syringe with a plastic catheter from a butterfly needle or an 18-gauge plastic IV catheter.

Indications for ENT Referral Irrigation ineffective; manual removal not possible; patient has vertigo, hearing loss, or severe pain

Hygiene and Prophylactic Care • Cotton swabs should only be used for cleaning the outer ear structures. • Normal hygiene: Cleaning the ear with running water while bathing or showering is usually sufficient. • Occasionally, a ceruminolytic may be used once or twice weekly on a regular basis to prevent excessive cerumen buildup in patients with recurrent impaction.

DRAWING LABS: TUBE COLORS, STANDARD PANELS, HEMOLYSIS Hematology: EDTA tubes (purple) Chemistry: Heparin tubes (green) Coagulation: Citrate tubes (blue)

COLLECTION TUBE TYPES FOR SELECTED LAB SAMPLES* Red

Blue

Purple

Green

CBC

Chem 7/10

Chem 7/10

Bartonella titer

ESR

Liver panel

Liver Panel

Hepatitis panel

Fibrinogen

ABO/Rh

Amylase

Amylase

HIV Western blot

PTT Hepzyme

Coombs

Lipase

CRP

RPR

D-dimer

Crossmatch

Gentamicin peak, trough, random

Gentamicin (peak, trough, random)

Rubella IgG/IgM

Unfractionated heparin level

Reticulocyte count

CRP

TSH/free T4

Bilirubin (total, direct, indirect)

Antithrombin

HgbA1C

Blood fungus culture

LH/FSH

Monospot

Rubella IgG

Hemoglobin profile

HIV ELISA antibody

EBV titers/EBNA

Drug screen, serum

EBV, CMV, adenovirus: quantitative PCR

Rubella IgG

CMV titers

Osmolality, serum

Amylase

Lipid panel

Iron panel

CK, CKMB

HIV: DNA PCR, RNA, viral load

Bilirubin (total, direct, indirect)

Quantitative immunoglobulins

Vancomycin (random, peak, trough)

Rubella IgG

Osmolality, serum

ANA/ANA profile

Most serum drug levels

CK, CKMB

C3/C4

Vancomycin (random, peak, trough)

HCG

Ammonia (on ice)

ASO titer

PT/INR/PTT

∗Some labs may be drawn in multiple collection tube types.

POTENTIAL EFFECTS OF HEMOLYSIS ON SELECTED LABORATORY VALUES Increase with hemolysis

Bilirubin,∗ albumin, protein, ALT, AST, LDH, alk phos, CK, phosphate, iron, ammonia, Ca, K

Decrease with hemolysis

Bilirubin,∗ haptoglobin, thyroxine (T4), troponin T, RBC, HCT

∗May increase or decrease with hemolysis.

7



8


SI LAB VALUE CONVERSION TABLE Lab Value

US Unit

SI Unit

Factor* Lab Value

Chemistry

US Unit

SI Unit

Factor*

Blood Gas

ALT, AST

U/L

μkat/L

0.0167

PaCO

mm Hg

kPa

0.133

Alk phos

U/L

μkat/L

0.0167

PaO

mm Hg

kPa

0.133

Amylase

U/L

nkat/L

0.0167

Toxicology & Drug Monitoring

Bilirubin

mg/dL

μmol/L

17.1

Acetaminophen

mcg/mL

μmol/L

6.62

BUN

mg/dL

mmol/L 0.357

Carbamazepine

mcg/mL

μmol/L

4.23

Calcium

mg/dL

mmol/L 0.25

Digoxin

ng/mL

nmol/L

1.28

Cholesterol

mg/dL

mmol/L 0.0259

Gentamicin

mcg/mL

μmol/L

2.09

Cortisol

mcg/dL

nmol/L

27.6

Phenytoin

mcg/mL

μmol/L

3.96

Cr Kinase

U/L

μkat/L

0.0167

Salicylate

mg/L

mmol/L

.00724

Creatinine

mg/dL

μmol/L

88.4

Theophylline

mcg/mL

μmol/L

5.55

Glucose

mg/dL

mmol/L 0.0555

Tobramycin

mcg/mL

μmol/L

2.14

LDH

U/L

μkat/L

0.0167

Valproate

mcg/mL

μmol/L

6.93

Lipase

U/dL

μkat/L

0.167

Vancomycin

mcg/mL

μmol/L

0.690

2+

Mg

mEq/L

mmol/L 0.5

Phos

mg/dL

mmol/L 0.322

Folate

ng/mL

nmol/L

2.27

T4

mcg/dL

nmol/L

12.9

Hemoglobin

g/dL

mmol/L

0.621

Hematology

T3

mcg/dL

nmol/L

0.0154

Iron, TIBC

mcg/dL

μmol/L

0.179

Uric acid

mg/dL

μmol/L

59.5

Vit B12

pg/mL

pmol/L

0.738

∗Factor to convert from standard to SI units.

ACETAMINOPHEN DOSING Elixir (160 mg/ 5 mL)

Age∗

Weight

Drops (80 mg/ 0.8 mL)†

0–3 mo

6–11 lb (2.7–5 kg)

0.4 mL

—

—

4–11 mo

12–17 lb (5.5–7.7 kg)

0.8 mL

1/2 tsp

1 tab

1–2 yr

18–23 lb (8.2–10.5 kg)

1.2 mL

3/4 tsp

1 1/2 tabs

2–3 yr

24–35 lb (10.9–15.9 kg)

1.6 mL

1 tsp

2 tabs

4–5 yr

36–47 lb (16.3–21.4 kg)

2.4 mL

1 1/2 tsp

3 tabs

∗Age is provided as a convenience only; dosing should be based on weight. † 5 mL = 1 tsp.

Chewable Tablets (80-mg tablets)

9

• Dosages may be repeated every 4 h but should not be given more than five times in 24 h. • Alternating acetaminophen and ibuprofen: An alternating treatment regimen of acetaminophen (12.5 mg/kg/dose) and ibuprofen (5 mg/kg/dose) every 4 h for 3 d is more effective than monotherapy in lowering fever in infants and children (Arch Pediatr Adolesc Med. 2006;160:197).

Dosages Children:

10–15 mg/kg/dose every 4–6 h prn; maximum, 15 mg/kg/dose or 75 mg/kg/d or 2.6 g/d

Adults:

325–650 mg every 4–6 h prn or 1000 mg 3–4 times/d prn; maximum, 4 g/d

IBUPROPHEN DOSING Elixir (100 mg/ 5 mL)

Chewable Tablets (50 mg tablets) —

Age∗

Weight

Drops (40 mg/ 1.5 mL)†

6–11 mo

12–17 lb (5.5–7.7 kg)

1 dropper

—

1-2 yr

18–23 lb (8.2–10.5 kg)

1 1/2 droppers

—

—

2-3 yr

24–35 lb (10.9–15.9 kg)

2 droppers

1 tsp

—

4-5 yr

36–47 lb (16.3–21.4 kg)

—

1 1/2 tsp

3 tablets

∗Age is provided as a convenience only; dosing should be based on weight. † 5 mL =1 tsp. Doses may be repeated every 6 to 8 h but should not be given more than four times in 24 h.

• Alternating acetominophen and ibuprofen: An alternating treatment regimen of acetaminophen (12.5 mg/kg/dose) and ibuprofen (5 mg/kg/dose) every 4 h for 3 d is more effective than monotherapy in lowering fever in infants and children (Arch Pediatr Adolesc Med. 2006;160:197).

Dosages Mild to moderate temperature or pain: 5 mg/kg every 6–8 h as needed; maximum, 40 mg/kg/d Severe temperature or pain: 10 mg/kg every 6–8 h as needed; maximum, 40 mg/kg/d

SYSTEMIC CORTICOSTEROID EQUIVALENCIES

Compound

Relative Antiinflammatory Potency

Duration of Action

Relative Sodium Retaining Potency

Approximate Equivalent Dose (mg)∗

Cortisone

0.8

S

0.8

25

Hydrocortisone

1

S

1

20

Prednisone

4

I

0.8

5

Prednisolone

4

I

0.8

5

Methylprednisolone

5

I

0.5

4

(continued on next page)



10


Compound Triamcinolone

Relative Antiinflammatory Potency

Duration of Action

Relative Sodium Retaining Potency

Approximate Equivalent Dose (mg)∗

5

I

0

Fludrocortisone

10

S

125

4 —

Betamethasone

25

L

NA

0.6–0.75

Dexamethasone

25

L

0

0.75

∗These dose relationships apply only to oral or IV administration. These values, which correlate with individual systemic corticosteroids, are used to create a ratio for switching between different corticosteroids (IM or IV only; same route when switching between classes). I = intermediate or 12- to 36-h half-life; L = long or 36- to 72-h half-life; NA = data not available; S = short or 8- to 12-h biological half-life.

Available at www.AccessPediatrics.com • Evaluation and feedback • Breaking bad news • Medication compliance: helping kids take medicines • Infiltrations, extravasation, and vesicant injury • Epidemiology and statistics: the basics

Analgesia and Sedation PAIN ASSESSMENT TOOLS • Physiologic parameters: Tachycardia, vasoconstriction, diaphoresis, pupil dilatation, increased minute ventilation, hypertension

0

2

4

6

8

10

No hurt

Hurts little bit

Hurts little more

Hurts even more

Hurts whole lot

Hurts worst

Figure 2-1 FACES pain scale. (From Hockenberry MJ, Wilson D. Wong’s Essentials of Pediatric Nursing, 8th ed. St. Louis: Mosby; 2009. Used with permission. Copyright Mosby.)

NONPHARMACOLOGIC PAIN CONTROL (Emerg Med Clin North Am 2005;23:393) • Cognitive: Music, guided imagery, distraction, positive reinforcement, decentralization, hypnosis • Behavioral: Relaxation techniques, biofeedback exercises, breathing control, distraction, education • Physical: Hot and cold compresses, massage or touch, position and comfort, temperature regulation, transcutaneous electrical nerve stimulation, acupuncture, chiropractic therapy, immobilization • Gate control hypothesis: Close the “pain gates” via nonpainful receptors or excitatory messages from the brain

PHARMACOLOGIC PREPARATION General Principles • Formulations containing epinephrine should NOT be used in terminal capillary circulations (eg, pinna, digits, nose, penis, and other areas of end-capillary circulation).

Category

Drug

Dosage

Warnings

Topical local anesthetic

LET (lidocaine, epinephrine, tetracaine) suturing solution

≤3 mL on gauze or cotton pad in direct contact with wound

• Do not repeat dosing • Laceration should be >5 mm from mucous membranes

ELA-Max, L.M.X. 4% (lidocaine) or EMLA (eutectic mixture of lidocaine and prilocaine)

Maximum dosing to intact skin (30 min for ELA-Max; 60 min for EMLA): Birth–3 mo or 5 kg: 2 g 1–6 yr and >10 kg: 10 g >7 yr and >20 kg: 20 g

• Methemoglobinemia (especially in neonates and infants) • Irregular heart beats • Seizures • Coma • Respiratory depression (continued on next page)

11


CHAPTER 2

12


Category

Injectable local anesthetic

Drug

Dosage

Warnings

Ethyl chloride topical spray

Spray at target area 3–9 in away for 3–7 s until skin turns just white; immediate onset

• Irritation at site • Pigmentation changes to application site

Lidocaine (with or without epinephrine)

Maximum dose: 4.5 mg/kg Buffer 1:10 with sodium bicarbonate (8.4%) to reduce burning sensation at injection site

Bupivacaine

Maximum dose: 2.5 mg/kg Buffer with 1:30 with sodium bicarbonate (8.4%) to reduce burning sensation at injection site

• Methemoglobinemia (especially in neonates and infants) • Irregular heart beats • Seizures • Coma • Respiratory depression • If it must be used in neonates and infants, in general, reduce dosing by 30%

ORAL AND INTRAVENOUS ANALGESIA • Avoid salicylates because of their association with Reye syndrome. • Oral sucrose: Neonates and infants younger than age 6 months during procedures such as heel sticks, venipunctures, and LPs up to 1 mL for three doses. • Nonopioid analgesics: For mild to moderate pain if the FACES pain scale below 5 or objective pain scale or numeric pain scale below 6. • Nonopioid analgesics are used very infrequently in the NICU and should be used only after discussion with the attending physician.

NONOPIOID ANALGESICS Drug

Dosage

Comments

Acetaminophen (nonsalicylate)

PO or PR 10–15 mg/kg q 4–6 h Maximum dosage: 1000 mg q6h

• Not anti-inflammatory • Caution in patients with liver disease

Ibuprofen (NSAID)

PO 4–10 mg/kg q 6-8 h (not for use in patients younger than 6 mo of age) Maximum dosage: 800 mg q6h

• Caution with renal insufficiency • Increased risk of GI ulcers or bleeds

Ketorolac (NSAID)

IV or IM 0.5–1.0 mg/kg q 6 h followed by 0.5 mg/kg q 6 h Maximum dosage, 30 mg q 6 h; no more than 5 days/mo

• Keep patient well hydrated • Caution with renal insufficiency • Only parenteral NSAID

Naproxen (NSAID)

PO 5–7 mg/kg q 8–12 h (not for use in patients younger than 2 yr of age) Maximum dosage: 500 mg q8h

• Increased risk of cardiovascular events and GI ulcers or bleeds

Indomethacin (NSAID)

PO 1 mg/kg q 6–8 h Maximum dosage: 50 mg q 6 h

• See comments on other NSAIDS

13

• Opioid analgesics: For moderate to severe pain if pain scales above 6; bind μ-receptors in the CNS. • Opiates may cause severe respiratory depression, hypotension, CNS depression, bladder retention, ileus, pruritus, nausea and vomiting. • Opiate antagonist Naloxone: For intoxication, use 0.1 mg/kg up to 2 mg IV/IM; for respiratory depression, use 0.01 mg/kg, which may be repeated every 2 to 3 minutes up to 0.05 mg/kg if needed.

OPIOID ANALGESICS* Drug

Dosage

Comments

Codeine

PO 0.5–1 mg/kg q 4–6 h Maximum dose: 60 mg

Commonly combined with acetaminophen

Fentanyl

IV 1–2 mcg/kg/dose < 12 yr IV 0.5–1 mcg/kg/dose ≥ 12 yr

Muscle rigidity and chest wall spasm (may require a nondepolarizing muscle relaxant) IV onset: 4–5 min Duration: 20–60 min

Hydrocodone

PO 2.5–5.0 mg q 6-8 h Maximum dose: 10 mg

Commonly combined with acetaminophen

Hydromorphone

IV 0.015 mg/kg q 3–4 h PO 0.03–0.08 mg/kg q 3–4 h Maximum dose: 2 mg

High potential for abuse

Methadone

PO 0.1 mg/kg q 4–12 h Maximum dose: 20 mg

Used in narcotic detoxification May cause QT prolongation

Morphine

IV 0.05–0.2 mg/kg q 2–4 h PO 0.1–0.5 mg/kg q 4–6 h MS Contin: Can give q 8–12 h Maximum dosage: IV 15 mg, PO 30 mg

Do not crush or chew controlled-release tablets IV onset: 5–10 min Duration: 2–4 h

Oxycodone

PO IR 0.2 mg/kg q 3–4 h PO SR 10–20 mg q 12 h Maximum dose: 30 mg

Do not use sustained-release tablets as a PRN analgesic

∗Medications that are italicized indicate routine use in the NICU.

PATIENT-CONTROLLED ANALGESIA (PCA) • Use in patients with moderate to severe recurrent pain who may need a continuous infusion of opiates and have the capacity to understand and push the PCA button to self-administer interval boluses of opioid. May also be administered by a nurse or parent. • Patients with sickle cell disease or severe chronic pain may require higher doses. • Patients may need continuous monitoring, especially if younger than age 6 months. • A background infusion should only be considered for patients with severe or chronic pain, including those with scoliosis, sickle crises, or cancer-related pain. These patients may require a higher 4-h maximum.


Analgesia and Sedation

14


Agent

Loading Dose

Maximum Single Dose

Continuous Infusion

Morphine

0.05–0.1 mg/kg

10 mg

0.01–0.03 mg/kg/h

Fentanyl

1–3 mcg/kg

25 mcg

0.15–0.3 mcg/kg/h

Hydromorphone

0.005–0.015 mg/kg

2 mg

0.003–0.005 mg/kg/h

Agent

Interval Dose

PCA Lockout Interval

4 Hours Maximum

Morphine

0.015–0.025 mg/kg

6–12 min

0.25–0.35 mg/kg

Fentanyl

0.14–0.28 mcg/kg

6–10 min

2.8 mcg/kg

Hydromorphone

0.003–0.005 mg/kg

6–12 min

0.05–0.06 mg/kg

Conversion Between Opiates • Calculate the 24-h opioid requirement. • Example: Patient receiving morphine 30 mg PO q8 h and want to change to hydromorphone as continuous infusion. 30 mg × 3 = 90 mg • Calculate the equinalgesic dose by using the table below for a given route of administration. ⎡ 10 mg IV morphine × mg IV m orphine ⎤ ⎢ 60 mg PO morphine = 90 mg PO morphine ⎥ → × = 15 mg ⎣ ⎦ • Convert to the new opioid. ⎡ 10 mg IV morphine 15 mg IV morphine ⎤ ⎢ 1.5 mg IV hydromorphone = × mg IV hydromorphone ⎥ → × = 2 . 25 mg ⎣ ⎦ • Calculate the required dose. • The new dose would be 2.25 mg/24 h = 0.1 mg/h IV hydromorphone. • With PCAs, the total 24-h dose needs to be calculated including continuous infusion and all interval doses. Then convert.

Drug

IV or IM (mg)

PO (mg)

Codeine

120

200

Fentanyl

0.1

NA

Hydrocodone

NA

15–20

Hydromorphone

1.5

7.5

Methadone

10

20

Morphine

10

30–60

Oxycodone

NA

30

Weaning Opiates (for habituated patients, please see Guidelines for Weaning Habituated Patients from Chronic Sedatives and Analgesics” below) • All pain medicines need to be converted to one medicine in the same group via the conversion technique described above.

15

• Choose a reasonable weaning factor (eg, 10%–20% of the initial dose) and wean by weaning factor every day or every other day (opiate-naive patients who have undergone a relatively short therapetic course may be weaned more rapidly). • Example: To wean a morphine dose of 20 mg/d, decrease the morphine by 2 mg every day or every other day. • To change PCA to PO medicines, administer the first PO dose (dose calculated via the method described above), stop the continuous infusion 30 to 60 minutes later, reduce bolus doses by 25% to 50% with each dose, discontinue PCA after no boluses are required for 6 h, or may need to increase new PO dose if unable to wean PCA boluses.

SEDATION Continuum of Sedation (Ped Clin N Am 2006;53:279) • Sedation occurs along a spectrum, and patients can move through all degrees of sedation (anxiolysis → moderate sedation → deep sedation → general anesthesia) easily without affecting their vital signs. • The depth of sedation should be monitored regularly with verbal and tactile stimuli because a patient’s ability to protect the airway becomes increasingly more impaired with increasing levels of sedation. • Attending personnel should be trained to rescue a patient from one level above the intended level of sedation.

Patient Preparation for Sedation • AMPLE history: Allergies, Medications, Past medical history (including risks for abnormal airway and aspiration), Last food/drink, Events (including prior sedation history, and recent or current illnesses including respiratory infections). • Check NPO status (last meal): There is an increased aspiration risk during sedation for patients who have had solids or non-breast milk within 6 h, breast milk within 4 h, and clear fluids within 2 h. • Discuss the risks and benefits with the patient and family and outline the plan, including planned medications, before starting. • Assess for risk factors for complications, including significant underlying disease, abnormal airway, age younger than 6 years, active respiratory infection or fever, dehydration, or use of multiple sedation medicines. • ASA Physical Status Classification: Patients classified at ASA of 3 to 4 are at an increased risk from sedation.

AMERICAN SOCIETY OF ANESTHESIOLOGISTS PHYSICAL STATUS CLASSIFICATION Class

Description

Examples

I

Normal, healthy patient

—

II

Mild systemic disease

Asthma, controlled diabetes

III

Moderate systemic disease

Stable angina, diabetes with hyperglycemia, moderate COPD

IV

Severe systemic disease

Unstable angina, diabetic ketoacidosis

V

Moribund

—

+E

Modifier added to any classification indicating “emergency” status

All ED patients

Adapted from the American Society of Anesthesiologists.

• Physical exam focusing on identifying patients with potentially difficult airways, cardiopulmonary compromise, decreased neurologic baseline, and loose teeth or poor dentition. • Determine Mallampati classification: Visibility of oral cavity used to predict the ease of intubation.



16


CLASS 1

CLASS 2

CLASS 3

CLASS 4

MALLAMPATI CLASSIFICATION Class 1: Soft palate, uvula, tonsillar pillars visible* Class 2: Soft palate, majority of uvula visible. Class 3: Soft palate, base of uvula visible. Class 4: Soft palate not visible. Figure 2-2 Mallampati classification. *The original Mallampati classification (Can Anaesth Soc J 1985;32:429) was based on observation of the oropharynx of the patient while seated; the patient extends the tongue as far forward as possible and observations are made of the structures of the pharynx (tonsillar pillars, soft palate and base of the uvula). (Adapted from Brunicardi FC, Andersen DK, Billiar TR, et al: Schwartz’s Principles of Surgery, 9th Edition. Copyright © The McGraw-Hill Companies, Inc. All rights reserved.)

• Prepare emergency equipment (SOAP): Suction, oxygen, provide IV access, pharmacy and medications, oral airway, bag or mask, laryngeal mask airway, intubation supplies including ETT and laryngoscope, NG tubes, crash cart nearby, and two health care providers trained in resuscitation must be present during the procedure.

Weight/Age

ETT Size

2 yr

[Age (in yrs) / 4] + 4

Weight/Age

Laryngoscope Size (Type)

12 yr

3 (Miller or MacIntosh)

• Place patient on continuous cardiopulmonary monitoring (monitor presedation vitals, q5–10 min during the procedure, and q5–15 minutes after the procedure until the patient is awake and alert). Also monitor the airway and ventilation and level of consciousness during the procedure. • Some institutions require end-tidal CO2 monitoring even for moderate sedation.

17

Sedation Medications • Procedural sedation and analgesia includes the use of sedative–hypnotic, analgesic, or dissociative anesthetics during unpleasant diagnostic or therapeutic procedures. • Medications should be chosen based on the degree of pain caused by the procedure, the duration of the procedure, immobilization needed for the procedure, underlying medical problems, and physician familiarity and experience with the medications. • Benzodiazepines: Sedative–hypnotic agents with potential amnestic effect, no analgesic properties, bind GABA receptors. • Cause CNS depression, dose-dependent respiratory depression, and (less frequently) hypotension. • Patients may experience paradoxical agitation. • Benzodiazepine antagonist: Flumazenil 0.01 mg/kg up to 0.2 mg IV; repeat doses at 0.005 to 0.01 mg/kg up to 1 mg cumulative dose.

Lorazepam

Diazepam

Dose and Drug Route

Duration

Adverse Effects

Comments

IM or IV 0.04–0.2 mg/kg IM 15 min Maximum single IV 2–3 min dose, 10 mg

Intermediate Blood dyscrasias IM 2–4 h IV 30–90 min Thrombophlebitis

• Active metabolite accumulation may prolong sedation

PO 30–60 min 12 yr PO 2–4 mg IM or IV: 0.05 mg/kg Maximum dose, 4 mg

Long acting PO 2–8 h IM 2–6 h IV 2–6 h

Blurred vision Hallucinations Restlessness Drowsiness Confusion

• Use with caution in patients with renal or liver disease • Ideal for prolonging sedation or anxiolysis and seizure treatment

PO 10–20 min IM 15 min IV 1–2 min IN: 0.2 mg/kg

Short acting PO 2 h IN 60 min IM or IV 2–6 h

Bitter taste Amnesia Blurred vision Headache Hiccoughs Nausea or vomiting Coughing

• Cardiac arrest and hypotension have occurred after premedication with a narcotic, but fewer cardiac complications • Excellent for muscle relaxation or prolonged sedation • IV injection painful • Only benzodiazepine FDA approved for use in neonates; rapid administration may cause myoclonic activity

6 mo and 12 yr IV 0.5–2 mg/dose



18


• Opiates: See Analgesia section. • Barbiturates (Pentobarbital): Sedative, hypnotic, and anticonvulsant, no analgesic properties, binds GABA receptor. • Ketamine: Anxiolytic, analgesic, amnestic, dissociative anesthesia; phencyclidine derivative that acts on cortex and limbic system and antagonizes NMDA receptor; watch for “emergence phenomena” in older children. • Chloral hydrate: Sedative, no analgesic properties, trichloroethanol active metabolite with unknown mechanism, long acting. • Propofol: Amnestic, sedative and anticonvulsant, no analgesic properties, alkyl phenol that reaches high concentrations in the brain. • Etomidate: Sedative and hypnotic, no analgesic properties, carboxylated imidazole, inhibits 11-β-hydroxylase. • Dexmedetomidine: Sedative, selective α2-adrenoceptor agonist

Onset

Adverse Duration Effects

Pentobarbital

Comments

PO or PR >50–100 mg/kg Maximum dose, 1 g

30–60 min 4–8 h

Unpleasant taste • Contraindicated in myocardial GI irritation depression, Disorientation airway obstrucDelirium tion, apnea, and renal or hepatic Headache impairment Respiratory • 30%–40% failure depression rate

>3 mo only PO 6–10 mg/kg IM 3–7 mg/kg IV 0.5–1 mg/kg; may repeat ≤2 mg/kg in 1-h time period Maximum dose, 100 mg

PO 20–45 min IM 3–4 min IV 1–2 min

PO 1–2 h IM 12–25 min

Increased systemic, intracranial, and intraocular pressures (contraindications) Nystagmus Tachycardia Diplopia Hypersalivation (may pretreat with atropine or glycopyrrolate) Hallucinations Emergence reaction (tx with midazolam) Laryngospasm

IM 2–6 mg/kg IV 1–3 mg/kg; may repeat Maximum dose, 200 mg or 6 mg/kg

IM 10–15 min IV 1 min

Variable

IV 10–15 min

Ketamine

Chloral hydrate

Drug Dose/Route

• Potent analgesia • Rapid sedation • Bronchial smooth muscle relaxation (use in patients with asthma) • Rare respiratory depression, maintains airway well • Eyes may remain open • Movement may occur • Use with caution in patients with head trauma, seizures, WPW, and HTN

Agitation (some- • 3%–7% of patients have sigtimes “rage nificant oxygen phenomenon”) desaturation Confusion • Contraindicated Nausea or in liver impairvomiting ment Apnea or • Provides bradycardia immobility Hypotension (continued on next page)

Drug Dose/Route

Children > 10 yr IV 0.1–0.4 mg/kg For general anesthesia: IV 0.2–0.6 mg/kg followed by an infusion of 5–20 mcg/kg/min

Adverse Duration Effects

Comments

IV 50 kg: bolus in 20- to 50-mg increments, followed by infusion as above

Dexmedetomidine

Etomidate

Propofol

Only used for deep sedation in some institutions∗

Onset

19

Apnea Myoclonic jerks Adrenal suppression Nausea or vomiting Hiccups

Bradycardia • Contraindicated in patients with Hypertension (in rapid admin- renal failure, severe hepatic istration) impairment, Hypotension heart block, Nausea hypotension, and severe ventricular failure

• For diagnostic imaging, immobilization, and nonpainful procedures: Pentobarbital, chloral hydrate, benzodiazepines, propofol, etomidate, or dexmedetomidine. • For painful procedures such as orthopedic reductions, large lacerations, or abscess incision and drainages: choices include ketamine/benzodiazepine, benzodiazepine/ opioid, propofol/opioid, or etomidate/opioid. Particularly when combining sedatives, close monitoring of patient is required.

Postsedation Monitoring • Monitor heart rate, blood pressure, respiratory rate, oxygen saturation, level of consciousness, and sedation score q 5 to 15 min for 30 min postsedation. • If flumazenil or naloxone administration was necessary, then the patient must be closely monitored in an increased level of care (eg, PICU, NICU) for a duration of time at least 2-3 half-lives of the medication that was reversed. • Full term infants younger than age 1 mo or premature infants younger than 52 to 60 postmenstrual weeks (per local institutional policy) need to be monitored for 12 apneafree hours. • Discharge criteria: CV function and airway patency stable, easily arousable and protective reflexes intact, can talk if verbal, can sit if appropriate, and can drink fluids with adequate hydration status.



20


RECOMMENDED POST-SEDATION DISCHARGE CRITERIA • Patient is alert and oriented; infants and those with underlying abnormal mental status are at their baseline level of functioning. • Vital signs are stable and within age-appropriate limits, including pain level. • Protective reflexes are intact (gag, cough, swallow) • Patient does not have protracted nausea and vomiting. • Patient is able to ambulate at the preprocedural level. • Patient is discharged to the company of a responsible adult. • Written instructions given for postsedation diet, medications, activities, and contact phone number in case of emergency.

• At discharge, provide patient with discharge instructions and ensure that pain and side effects from the pain medicines will be adequately controlled at home.

Guidelines for Weaning Habituated Patients from Chronic Sedatives and Analgesics Midazolam to Ativan Wean • Day 1: If midazolam >0.3 mg/kg/h, decrease by 0.5 mg/kg/h q 12 h. • Once midazolam 5 kg, discontinue after dose is 0.5 mg × 24 h). Fentanyl to Methadone Wean • Day 1: If fentanyl >10 mcg/kg/h, decrease by 1 mcg/kg/h q 12 h. • When fentanyl 5 kg, discontinue after dose is 0.5 mg × 48 h). • If the patient shows signs of withdrawal during the wean, give extra lorazepam at the same dose of the current day and hold the wean for 24 h.

Available at www.AccessPediatrics.com • Principles of pain management

Procedures NEEDLE DECOMPRESSION OF PNEUMOTHORAX • Indications: (1) Tension pneumothorax • Complications: Pneumothorax, bleeding, nerve damage, infection

Setup Equipment Needed for Needle Decompression of Pneumothorax • Personal protection equipment: Gloves, gown, face and eye shield • Pulse oximeter and cardiorespiratory monitor • Oxygen supply via nonrebreather mask • Antiseptic skin prep solution (eg, Povidone-iodine, Chlorhexidine)

• Local anesthetics: 1% lidocaine drawn up • 16- to 20-gauge needle or plastic-over-theneedle catheter (IV catheter) • 50-mL syringe with a stopcock • Occlusive dressing (vaseline gauze, 4 × 4 gauzes and tape)

Procedure • Attach the patient to a cardiorespiratory monitor and apply oxygen per nonrebreather mask. • On the affected side, locate the second intercostal space at the midclavicular line. • Prep the skin using sterile techniques if patient is stable. • If the patient is stable, raise a wheal with 1% lidocaine over the superior edge of the rib below the intercostal space (third rib). • Penetrate the skin over the superior edge of the rib using a 16- to 20-gauge IV catheter. • Advance the IV catheter gradually until a pop is heard or felt on entry of the pleural space. • Remove the needle (a rush of air may be audible as tension is released). • Attach a syringe with a stopcock to the hub of the catheter. • Withdraw desired air, releasing further tension. • Withdraw the needle at the end of the procedure and apply an occlusive dressing to the site (preferably with 3 of 4 sides fixed to skin to create a one-way flap-valve for further decompression). • Obtain a CXR to assess the results of the procedure.

CHEST TUBE PLACEMENT INDICATIONS FOR CHEST TUBE PLACEMENT Emergent

Non-emergent

Pneumothorax • Large pneumothoraces • In all patients on mechanical ventilation • Tension pneumothorax after needle decompression • Recurrent or persistent pneumothorax • Pneumothorax secondary to chest trauma Hemopneumothorax Esophageal rupture with gastric leak into the pleural space

Malignant pleural effusion Recurrent pleural effusion Treatment with sclerosing agents or pleurodesis Parapneumonic effusion or empyema Chylothorax Postoperative care (eg, after coronary bypass, thoracotomy, or lobectomy)

Reproduced with permission from: NEJM 2007;357:e15.

21


CHAPTER 3

22


• Complications: Bleeding; hemothorax; perforation of visceral organs (lungs, heart, liver, spleen); intercostal neuralgia caused by trauma of neuromuscular bundles; subcutaneous empyema; infection, occlusion of chest tube with blood, pus, or debris • Additional information: NEJM 2007;357:e15.

Setup Equipment Needed for Chest Tube Placement • Personal protection equipment: Gloves, gowns, face and eye shield • Medications for sedation and systemic analgesia • Antiseptic skin prep solution (eg, Povidone-iodine, Chlorhexidine) • 1% lidocaine with epinephrine

• Chest tube tray, including correct size chest tube, a #11 scalpel blade and handle, a curved Kelly clamp, needle driver, and scissors • Chest tube suction unit, including tubing and wall suction hookup • Nonabsorbable 10 silk suture • Occlusive dressing (vaseline gauze, 4 × 4 gauze and tape)

CHEST TUBE SIZE

Age

Chest Tube Size (French)

Preterm infants

8

Term infants

10

1–3 years

12

>3–10 years

16–18

>10–15 years

20–28

>15 years

28

Procedure (Figure 3-1) • Place the patient in the supine position with the ipsilateral arm raised above the head. • Locate the fourth or fifth intercostal space in the anterior axillary line at the level of the nipple. • Prep the skin using antiseptic solution; arrange a sterile field if patient is stable. • Anesthetize the skin, subcutaneous tissue, muscles, and pleura: Raise a wheal with 1% lidocaine over the superior edge of the rib that is below the selected intercostal space (the inferior portion of the selected intercostal space to avoid neurovascular bundle); insert needle and anesthetize by injecting lidocaine as the needle is withdrawn. • Make a 1- to 2-cm incision parallel to the rib with #11 scalpel.

Figure 3-1 Chest tube placement. (Reproduced with permission from: Hall JB, Schmidt GA, Wood LDH: Principles of Critical Care, 3rd ed. Copyright © The McGraw-Hill Companies, Inc. All rights reserved.)

23

• Use a Kelly clamp to bluntly dissect the sub-Q tissue and intercostal muscles down to the pleura. • Insert your index finger or Kelly clamp through the incision, palpating within the pleural layer, ensuring that the lung falls away from the pleura. • Clamp the distal end of the chest tube with a Kelly clamp. • Grasp the proximal end of chest tube with Kelly clamp and insert tube through the incision. • When the tube has entered the thoracic cavity, remove the Kelly clamp and advance the chest tube until all of the tube fenestrations are within the thoracic cavity. • Aim the tube apically/anteriorly for pneumothorax and basally/posteriorly for fluid collection. • Use a continuous, purse-string suture (purse-string) to secure chest tube. • Tape the chest tube to the side of the patient. • Wrap petroleum gauze around the tube at the point where it enters the skin, and cover it with sterile gauze. • Secure the site with pressure dressings. • Attach the chest tube to suction unit and then unclamp the distal end. • Obtain a CXR to confirm placement and adjust the tube accordingly in a sterile fashion (once unsterile, tube can be withdrawn but never advanced).

Removal of the Chest Tube • Sutures, sterile gauze, and a suture kit should be available at bedside. • Cut the skin sutures. • Ask the patient to exhale fully and pull the tube out at end-expiration. • Quickly occlude the site with sterile gauze. • Additional sutures may be required to close the opening. • Secure the site with pressure dressing. • Obtain a CXR 4 to 6 h after the chest tube is removed. If abnormalities are seen then repeat another CXR in approximately 24 h after removal.

LUMBAR PUNCTURE • Indications: (1) Obtain CSF sample for diagnostic studies, (2) measurement of opening pressure, (3) administration of anesthesia, (4) administration of medications for chemotherapy, (5) CSF drainage for symptomatic relief of benign intracranial hypertension • Contraindications: Overlying skin infection, suspected intracranial mass, suspected spinal cord mass or defect (eg, spina bifida), bleeding diathesis (relative contraindication) • Complications: Herniation, cardiorespiratory compromise, local or referred pain, headache, bleeding, infection, leakage of CSF, subarachnoid epidermal cyst • Additional information: NEJM 2006;354:e16.

Setup Equipment Needed for Lumbar Puncture • Personal protection equipment: Sterile gloves (gown, face and eye shield as indicated) • Topical anesthetic cream • Oral sucrose solution for neonates • Antiseptic skin prep solution (eg, Povidoneiodine, Chlorhexidine)

• A 20 to 22-gauge spinal needle with a stylet (NEEDLE LENGTH < 2yr: 1.5 in; 2-12yr: 2.5 in; >12yr: 3.5 in) • Manometer (if measurement of opening pressure is indicated). • Sterile tubes for collection of CSF • Adhesive bandage

Procedure (Figure 3-2) • Anesthetize the patient by topical anesthetic cream at the intended site and wait 30 minutes or for infants give ≤2 ml oral sucrose solution 2 minutes before the LP.


Procedures

24


L4

Dura L4

L5

Subarachnoid space Cauda equina

Figure 3-2 Needle insertion during an LP. (Reproduced with permission from Gomella LG, Haist SA: Clinician’s Pocket Reference, 11th ed. Copyright © The McGraw-Hill Companies, Inc. All rights reserved.)

• Position the child. • Use the lateral recumbent position with the neck flexed and the knees to the chest. Use this position when measuring opening pressure (the child has to be as relaxed as possible). • The sitting position is an option in young children or when not measuring opening pressure. • Identify the L4 spinous process (a line between the iliac crests). • Identify the L4–L5 (or L3–L4) space by palpating above or below the L4 spinous process. • Sterilize the anticipated puncture site with antiseptic solution; start at the proposed site and work outward in a circular manner. Repeat 2-3 times. • Drape the area with sterile drapes. • Check the child’s position to ensure correct spine flexion, maximizing the interlaminar space. • Using a 20 or 22-gauge needle and a stylet with the bevel pointed cephalad, puncture the skin midway between the spinous processes of L4–L5 (or L3–L4). Remove the stylet (Ann Emerg Med 2007;49(6):762). • Advance the needle perpendicularly in the midline and sagittal plane, aimed slightly cephalad. • Advance the needle until a “pop” is felt and CSF starts flowing. (If the needle does not advance, withdraw it slightly and redirect the angle.) • If measuring opening pressure: Reinsert the stylet and ask the patient to straighten the legs and neck. Remove the stylet, connect the manometer to the spinal needle, and measure the opening pressure prior to specimen collection. • Allow CSF to drip into the collecting tubes. • When CSF collection is complete, replace the stylet and withdraw the needle. • Cover the site with an adhesive bandage. • Clean the antiseptic solution off the skin with damp warm towel. • Ideally, instruct the child to stay in the supine position for 4 to 6 hours to lessen the risk of a post-LP headache.

INTRAOSSEOUS (IO) ACCESS • Indications: (1) Immediate access to vasculature via the intramedullary veins for delivery of fluids, drugs, or blood products in children 0 to 7 years of age; (2) emergent situations when PIV is unobtainable; (3) during CPR when access is needed without interrupting CPR

25

• Contraindications: Overlying skin infection, fracture of the limb, congenital musculoskeletal abnormalities (eg, osteogenesis imperfecta), previous insertion attempt in the same extremity • Complications: Fat or bone embolism, growth plate injury, tissue necrosis from fluid extravasation, soft tissue abscess, or osteomyelitis (risk ↓ if IO removed within 3 h of placement)

Setup Equipment Needed for Intraosseous Access • Personal protection equipment: Gloves, (gowns, face and eye shield as needed) • Pillow or rolled towels • Povidone-iodine solutions, alcohol swabs • 1% lidocaine in a 3-cc syringe with a 27-gauge needle for local anesthesia (optional)

• 16- or18-gauge IO needle or EZ-IO (for 3–39 kg use: 15 mm, 15 gauge; ≥40 kg use 25 mm, 15 gauge) or 16- or 18-gauge spinal needle with stylet • Extension tubing with three-way stopcock • 5- or 10-cc syringe • NS flush syringes • Long armboard • Tape

Procedure • Determine the appropriate site of insertion: (1) The anteromedial surface of the proximal tibia (2 cm below the tibial tuberosity), (2) the medial surface of the distal tibia (2 cm above the medial malleolus), or (3) the distal femur along the midline (3 cm above the lateral condyle), (4) iliac crest, or (5) sternum in older children/adults. • Position the patient in supine, frog-leg position with a pillow or towels behind the knee. • Cleanse the skin with povidone-iodine solution followed by alcohol swabs. • If time permits, infiltrate the skin and down to the periosteum with 1% lidocaine. • Prepare IV tubing with a three-way stopcock and fluids. • Hold the IO needle with the stylet ball in the palm of your hand and place the tip of your index finger 1.5 to 2.0 cm from the tip of the needle. • Direct the needle perpendicular to the bony surface. • Insert the needle through the skin, aiming away from the growth plate. • When the needle reaches the periosteum, exert downward pressure in a firm, twisting manner until decreased resistance is felt, indicating the needle is in the marrow space. • Unscrew the cap and remove the inner stylet. • Verify position by (1) attaching the 5- or 10-cc syringe and attempting to draw back blood or marrow and/or (2) flushing with NS to ensure there is no resistance or extravasation of fluid. • Secure the needle and the tubing with adequate tape and clear dressing. • Secure the leg to the long armboard. • Attach the IV tubing and allow IV fluids to flow. Secure tubing to the leg with tape.

CENTRAL VENOUS LINE (CVL) ACCESS • Indications: (1) peripheral sites are inaccessible, (2) emergent situation requiring largebore IV catheter to deliver rapid fluid boluses or concentrated/vasoactive medications, (3) central venous pressure measurement, (4) placement of pulmonary wedge catheters, (5) placement of transvenous pacemakers, (6) for hemodialysis or plasmapheresis • Contraindications: Infection over the insertion site, venous thrombosis, fracture or suspected fracture close to site • Relative contraindication: Coagulopathy • Complications: Vessel laceration, vessel thrombosis, bleeding, infection, air or catheter embolism, cardiac arrhythmias, cannulation of artery. • Additional information: NEJM 2007;356:e21.


Procedures

26


Setup Equipment Needed for Central Venous Line Access • Personal protection equipment: Sterile gloves, gown, face and eye shield • Cardiopulmonary monitor • Sterile drapes • Antiseptic skin prep solution (eg, PovidoneIodine, Chlorhexidine) • Central line kit with appropriate catheter size (2 Fr for neonates; 3 or 4 Fr for children 1-5 years old; 5 Fr for older children)

• 1% lidocaine in a 3-mL syringe with a 25-gauge needle • Sedation and analgesia medications • A rolled-up blanket/towel • IV tubing and IV fluid • Heparinized NS flushes (1 unit heparin per mL NS) • Sterile occlusive dressing (Veni-Gard), Tape • Bedside ultrasound (if available)

Identifying a Central Line Site Subclavian Vein (SCV) • The SCV is located inferior and posterior to middle third of the clavicle and runs parallel to it. The subclavian artery is superior and posterior to the vein. • Place the child supine in a 20- to 30-degree Trendelenburg position and hyperextend the spine with a towel rolled underneath the shoulder. Turn the head away from the site of insertion. • Site of insertion: Just inferior and lateral to the middle third of the clavicle (where the clavicle angles posteriorly); aim the needle toward the midline or the sternal notch. • The right SCV approach is generally preferred. Internal Jugular Vein (IJ) • The IJ lies at the apex of the imaginary triangle formed by the sternal and the clavicular heads of the sternocleidomastoid muscle and the clavicle. • Place the patient in a 15- to 20-degree Trendelenburg position and hyperextend the neck with a rolled blanket under the shoulders. Turn the head away from the site of insertion. • Site of insertion: Apex of the triangle; aim caudally toward the ipsilateral nipple. Femoral Vein (Figure 3-3) • The femoral vein lies medial to the femoral artery in the femoral sheath. • Place the child supine with the hip flexed and abducted in a frog-leg position. • If necessary, place a roll underneath the hip or buttock to expose the inguinal area. • Site of insertion: Identify the midpoint of the inguinal ligament, and locate the femoral artery pulse immediately distal; the femoral vein is medial and parallel to the artery.

Procedure • Sedate the patient if necessary (see Chapter 2). • Position the patient according to the chosen CVL site as described above. • Flush the IV tubing with heparinized NS and attach it to the IV fluid. • Wear sterile gown, gloves, a surgical cap, and a mask. • Flush the central venous catheter with heparinized NS. • Cleanse the site with antiseptic solution and drape adequately. • If appropriate, anesthetize the intended path with 1% lidocaine. • Use the 22-gauge introducer needle on a 3 or 5 cc empty “slip tip” syringe to locate the vein (aspirate as the needle is advanced until a flash of blood returns). If CVP is low (eg. hypovolemia), a flash of blood may not readily return with needle penetration of the vein; it may be necessary to apply negative pressure to a 3 or 5 cc syringe of heparinized NS while attempting to locate the vein. • Stabilize the introducer needle, remove the syringe, and immediately occlude the hub of the needle (maintain a “closed system”). • Thread about half of the J wire into the introducer needle. (The wire should pass easily without resistance; when threading into an IJ or subclavian vein, monitor for arrhythmia, which denotes wire position in the atrium or ventricle.)

27

Femoral nerve Femoral artery Femoral vein Empty space

Figure 3-3 Location of the femoral vein. (Reproduced with permission from Stone CK, Humphries RL: Current Diagnosis & Treatment: Emergency Medicine, 6th ed. Copyright © The McGraw-Hill Companies, Inc. All rights reserved.)

• Hold the J wire in place and remove the introducer needle. • Using a scalpel, make a small incision in the skin adjacent to the entry site of the J wire. • Thread the dilator over the wire; then remove dilator while leaving the J wire in place. • Thread flushed CVL over the wire, twisting it gently while advancing into the vein. • Leave 5 to 10 cm of the catheter outside the skin. • Carefully remove J wire and confirm backflow of blood from each port. • Flush each port with heparinized NS and attach the IV tubing. • Secure the catheter to the skin using single interrupted sutures. • Place a sterile occlusive dressing over the catheter. • Obtain a STAT postprocedure CXR after IJ or subclavian line placement, and an abdominal X-ray after a femoral line placement (evaluate for placement and complications).

UMBILICAL ARTERY CATHETERIZATION (UAC) • Indications: (1) hemodynamic instability; (2) continuous blood pressure monitoring; (3) administration of resuscitation fluids, concentrated or vasoactive medications, and exchange transfusions or (4) anticipated frequent arterial blood draws • Contraindications: Omphalitis, peritonitis, NEC, impetiginous skin lesions near umbilicus, omphalocele, gastroschisis, abdominal trauma • Complications: Infection, vasospasm, thrombosis, infarction, hypertension from renal artery stenosis, hemorrhage, vessel perforation, hypertension from renal artery thrombosis or stenosis


Procedures

28


Setup Equipment Needed for Umbilical Artery Catheterization • Personal protection equipment: Sterile gloves, gowns, face and eye shield, surgical cap • Radiant warmer with a light source • Cardiopulmonary monitor and pulse oximeter • Sterile drapes • Scalpel (#11 or #15) • Antiseptic skin prep solution (eg, Povidone-iodine, Chlorhexidine) • Curved, nontoothed iris forceps • Small, smooth, curved hemostats • Straight Crile forceps • Iris scissors

Figure 3-4 Course of the UAC and UVC.

• Needle holder • Nonthrombogenic, molded-tip umbilical artery catheters (3.5 Fr for infants 1.2 kg) • 3-0 or 4-0 silk suture on a curved needle • Linen umbilical tape • Adhesive tape • 10-mL syringe filled with heparinized NS (1 unit herparin per 1 mL NS) • IVF infusion setup • IV tubing and infusion pump • Three-way stopcock

29

Procedure • Determine the length of catheter to be inserted so that the tip lies above the diaphragm at the level of T6–T9: UAC insertion length in centimeters = (Birth weight in kg × 3) + 9 • Evidence does not support the use of “low catheterization” at the L3–L4 level. • Position the patient in a radiant warmer with the extremities in a supine frog-legged position. • Place the patient on cardiopulmonary monitors. • Consider sedation/analgesic medication to minimize patient movement and agitation. • Wear sterile surgical gown, gloves, a cap, and a mask. • Clean the abdomen and umbilical cord with antiseptic solution. • Drape the umbilical area in a sterile fashion. (Leave the infant’s head exposed for observation.) • Tie a surgeon’s knot loosely with the umbilical tape at the base of the umbilical cord. • Using forceps, grasp the cord in full thickness between 0.5 and 2 cm from its base. • Using a scalpel, cut the cord transversely along the top edge of the forceps. • Identify the two umbilical arteries (smaller with a thicker wall, located 4 and 7 o’clock). • Use a curved hemostat to grasp the end of the umbilicus to hold it steady and upright. • Use forceps to open and dilate the UA. • Insert a catheter to the predetermined length using gentle constant pressure to overcome resistance (a slight twisting motion may help). If there is resistance because of vasospasm, wait 1 to 2 minutes. (Significant force should not be used.) • Aspirate the catheter when in position to verify blood return. • Connect the catheter to the fluid and IV tubing. • Remove the umbilical tape. • Obtain abdominal radiograph to confirm the position and make adjustments to the catheter length (catheter may only be advanced under sterile conditions; once sterile field is broken down catheter may only be withdrawn). • Place a purse-string suture at the base of the umbilical cord, wrap the ends of the suture around the catheter, and tie. • Place a piece of adhesive tape perpendicular to the umbilical stump, incorporating both the catheter and the suture. Secure the tape to the abdominal wall.

UMBILICAL VEIN CATHETERIZATION (UVC) • Indications: (1) Rapid administration of resuscitation fluids, (2) administration of blood products or concentrated, caustic, or vasoactive medications, (3) exchange transfusions, or (4) anticipation of frequent blood draws • Contraindications: Omphalitis, peritonitis, NEC, impetiginous skin lesions, omphalocele, gastroschisis • Complications: Infection, thrombotic or embolic events hepatic necrosis if the catheter tip remains in the portal system, cardiac arrhythmia, portal hypertension, NEC

Setup Equipment Needed for Umbilical Vein Catheterization • See Equipment Needed for Umbilical Artery Catheterization • Nonthrombogenic, molded-tip umbilical vein catheters (5.0 Fr for infants 3.5 kg)

Procedure • Determine the length of the catheter to be inserted. The tip of the catheter should lie above the diaphragm at the junction of the IVC and the right atrium: UVC insertion length in centimeters = Half the UAC length calculated above + 1 or UVC insertion length in centimeters = Birth weight in kg × 7


Procedures

30


• Position the patient in a radiant warmer with the extremities in a supine frog-legged position. • Place the patient on cardiopulmonary monitors as well as a pulse oximeter. • Wear sterile surgical gown, gloves, a cap, and a mask. • Clean the abdomen and umbilical cord with antiseptic solution. • Drape the umbilical area in a sterile fashion. (Leave the infant’s head exposed for observation.) • Tie a surgeon’s knot loosely with the umbilical tape at the base of the umbilical cord. • Using forceps, grasp the cord in full thickness between 0.5 and 2 cm from its base. • Using a scalpel, cut the cord transversely along the top edge of the forceps. • Identify the umbilical vein (usually the larger floppy wall vessel close to the periphery of the stump). • Use a curved hemostat to grasp the end of the umbilicus to hold it steady and upright. • Use forceps to open and dilate the umbilical vein. • Insert a catheter to the predetermined length using gentle constant pressure to overcome resistance. There should be minimal resistance. (Significant force should not be used.) • Aspirate when the catheter is in position to verify blood return. • Connect the catheter to the fluid and IV tubing. • Obtain an abdominal radiography to confirm the position and make adjustments. • Place a purse-string suture at the base of the umbilical cord, wrap the ends of the suture around the catheter, and tie. • Place a piece of adhesive tape perpendicular to the umbilical stump, incorporating both the catheter and the suture. Secure the tape to the abdominal wall.

LACERATION REPAIR • Indications: (1) Primary closure of a wound, (2) restore perfusion to injured tissue • Contraindications: Grossly contaminated wounds, wounds required to heal by secondary intention (eg, bites), facial wounds oldermore than 24 h old, all other wounds oldemorer than 12 h old, complex lacerations involving substantial destruction of underlying structures • Complications: Infection, wound dehiscence, hypertrophic scars, retained foreign body

Setup Equipment Needed for Laceration Repair • Personal protection equipment: Sterile gloves (gowns, face and eye shield, surgical cap as needed) • Sterile drapes • Scalpel • Antiseptic skin prep solution such as chlorhexidine or povidone-iodine solution • Laceration repair kit, including a needle holder, tooth forceps, and scissors • Sterile NS

• Sterile gauze • 60-mL syringe with a splash guard or NS spray bottle for irrigation • Towels and bowl • Appropriate bicarbonate buffered anesthesia (see Laceration Management table) in a 3-mL syringe with a 25- or 27-gauge needle • Suture materials appropriate for wound (see Laceration Management table)

LACERATION MANAGEMENT Superficial Suture Special Location Anesthesia* Deep Suture Suture Dressing Removal Note Scalp

1% lidocaine with epinephrine

3–0 or 4–0 Vicryl

4–0 or 5–0 Nylon

Bacitracin, 5–7 days air

Pressure dressing if early hematoma


31

Superficial Suture Special Location Anesthesia* Deep Suture Suture Dressing Removal Note Pinna

1% lidocaine

5–0 Vicryl in 6–0 Nylon perichondrium

Bacitracin

Eyelid or eyebrow

1% lidocaine

5–0 Vicryl



Do not shave hair

Lip


5–0 Vicryl

6–0 Nylon (rapid)

Air

Care with vermillion border

Face or forehead


5–0 Vicryl

6–0 Nylon


Care with facial nerve

Neck


4–0 Vicryl

5–0 Nylon


Through Platysma

Trunk


4–0 Vicryl


Bacitracin

Extremities or buttocks


4–0 or 5–0 Vicryl

4–0/5–0 Nylon 3–0 for over joints

Bacitracin, 7–10 days xeroform 10–14 days if over joints

Hands

1% lidocaine

None


Bacitracin, 7–10 days xeroform 10–14 days if over joints

5–0 rapid if 2-h volume of feeds), hold feeds and recheck residual in 1 h. • Consider continuous transpyloric feeding. • Position patient with the right side down.

Clogged feeding tube

• Ensure that tube is flushed after checking residual check, bolus feed, and Q4–8 h when on continuous feeds. • Ensure appropriate tubing size for patient and formula being used. • Consider transpyloric or continuous feeds.

Reproduced with permission from Pediatric Nutrition Reference Guide, 8th ed:74.

PRETERM INFANT NUTRITION OVERVIEW Enteral Nutrition • For infants >1800 g and >34 wk gestation: Human milk or term formula indicated for all infants >1800 g and 34 wk (at a goal volume of 180 mL/kg/d) • For infants 18 yr

34

∗For children with adequate nutrition; use their weight in kilograms. For malnourished or obese children, use ideal body weight (IBW; BMI at 50th percentile). These DRI values are based on healthy (nonhospitalized) children; note that DRI values to not apply to preterm infants. Activity level, preexisting malnutrition, and concomitant medical or surgical conditions must all be accounted for in determining energy and protein goals. † Guidelines for premature infants reproduced with permission from AAPCON Nutritional Needs of the preterm infant. In Klienman RE (ed). Pediatric Nutrition Handbook, 6th ed; 2009. Data from Pediatric Nutrition Reference Guide, 8th ed:79.

Neonatal And Pediatric Nutrition

41

Stress Factors Stress factors should be used in conjunction with an estimation of resting energy expenditure such as the Schofield method or WHO; they should not be used with the DRIs above. 12% for each degree >37°C 1.15–1.25

Major surgery

1.20–1.30

Sepsis

1.40–1.50

Catch-up growth

1.50–3.00

Burns

1.50–2.00


• Calculation of basal metabolic rate or resting energy expenditure by the Schofield method:

Basal Metabolic Rate (kcal/d)∗ Male 0–3 yr

Female

(0.167 × wt) + (15.174 × ht) – 617.6

(16.252 × wt) + ( 10.232 × ht) – 413.5

3–10 yr

(19.59 × wt) + (1.303 × ht) + 414.9

(16.969 × wt) + (1.618 × ht) + 371.2

10–18 yr

(16.25 × wt) + (1.372 × ht) + 515.5

(8.365 × wt) + ( 4.65 × ht) + 200

>18 yr

(15.057 × wt) − (1.004 × ht) + 705.8

(13.623 × wt) + (2.83 × ht) + 98.2

∗Weight is expressed in kilograms; height is expressed in centimeters. Reproduced with permission from Pediatric Nutrition Reference Guide, 8th ed:19.

3. Calculate the required protein.

Age

Protein DRI (g/kg/d)

Preterm infants∗

Start at 1.5–2 and advance as tolerated to goal (3.5 parenterally or 3.5–4 enterally)

0–3 mo

1.52

4–6 mo

1.52

7–12 mo

1.20

13–35 mo

1.05

3 yr

1.05

4 yr

0.95

5–6 yr

0.95

7–8 yr

0.95

Boys 9–13 yr

0.95

Boys 14–18 yr

0.85 (continued on next page)

NUTRITION, GROWTH AND FORMULA

Fever Cardiac failure

42

Nutrition, Growth and Formula

Age

Protein DRI (g/kg/d)

Boys >18 yr

0.80

Girls 9–13 yr

0.95

Girls 14–18 yr

0.85

Girls >18 yr

0.80

∗Guidelines for premature infants reproduced with permission from AAPCON Nutritional needs of the preterm infant. In Klienman RE (ed). Pediatric Nutrition Handbook. 6th ed; 2009. Reproduced with permission from Pediatric Nutrition Reference Guide, 8th ed:79.

4. Calculate lipids and the rate for lipids. • The usual infusion is 20% lipid emulsion. • Fat should be approximately 30% of calories. • Minimum of 5% of calories as fat to avoid essential fatty acid deficiency • Maximum of 60% of calories as fat to avoid ketosis Energy from fat = Total energy (kcal/d) × 0.3 • The rate of the lipid is calculated by: [Energy from fat (kcal/d)]/[2 kcal/mL] = mL/d of lipid (Divide by 24 to get the hourly rate.) Example: Patient who requires 2000 kcal/d: 2000 × 0.3 = 600 kcal is energy from fat [600 kcal/d]/[2 kcal/mL] = 300 mL/d of lipid (300 mL/d of lipid)/(24 h) = 12.5 mL/h of lipid • Prematurity or concern for lipid intolerance: Begin with 1 g/kg/d (5 mL/kg/d) and advance by 1 g/kg/d (5 mL/kg/d) daily until energy needs from lipids are met and lipid tolerance is achieved. Check serial measurement of serum triglyceride level as indicated. Goal: 15 mL/kg/d (=3 g/kg/d) for neonates and up to 20 mL/kg/d (4 g/kg/d) for older infants. 5. Calculate the solution strength of carbohydrates and amino acids and their rate. • 70% of calories should come from dextrose and protein. Energy from dextrose and amino acid (kcal/d) = Total energy (kcal/d) × 0.7 • Restrict dextrose to maximum 12.5% and amino acids to maximum of 3% if infusing through a peripheral IV line (This is our practice at Texas Children’s Hospital. Most institutions will use a osmolality limit for use in a peripheral line; the AAP recommends no more than 900 mOsm.) • If concerns of glucose tolerance (ie, extreme low birth weight), initiate at a GIR of 4.5 to 6.0 mg/kg/min. • Do not exceed 4 g protein/kg/d. • The amino acids, dextrose, electrolytes, minerals, and vitamins are all in one solution. • Prematurity: 5% dextrose if 1000 g. • This is for the starter TPN only (for DOL 1 and 2); the standard solution is 12.5% dextrose, but it can be adjusted depending on the infant’s glucose tolerance. • After the final concentration of carbohydrates and amino acids has been determined (in %), the table below can be used for verifying that the solution will provide adequate caloric supply over 24-h period.


43

ENERGY DENSITY OF TOTAL PARENTERAL NUTRITION BASED ON DEXTROSE AND AMINO ACID CONTENT IN kcal/cc OF TOTAL PARENTERAL NUTRITION SOLUTION % Amino Acids 1

2.2

2.4

2.8

3

3.5

4

5

6

7.5

0.30

0.34

0.36

0.37

0.38

0.40

0.42

0.46

0.50

10

0.38

0.43

0.44

0.45

0.46

0.48

0.50

0.54

0.58

12.5

0.47

0.51

0.51

0.54

0.55

0.57

0.59

0.63

0.67

15

0.55

0.60

0.61

0.62

0.63

0.65

0.67

0.71

0.75

17.5

0.64

0.68

0.69

0.71

0.72

0.74

0.76

0.80

0.84

20

0.72

0.77

0.78

0.79

0.80

0.82

0.84

0.88

0.92

25

0.89

0.94

0.95

0.96

0.97

0.99

1.01

1.05

1.09

30

1.06

1.11

1.12

1.13

1.14

1.16

1.18

1.22

1.26

35

1.23

1.28

1.29

1.30

1.31

1.33

1.35

1.39

1.43

40

1.40

1.45

1.46

1.47

1.48

1.50

1.52

1.56

1.60


6. Calculate electrolytes. Daily electrolyte requirements Sodium (NaCl)

2–3 mEq/kg/d

Potassium (KCl)

1– mEq/kg/d

Chloride (Cl)

2–3 mEq/kg/d

Phosphorus (K2PO4)

0.65–2 mmol/kg/d

Magnesium (MgSO4)

0.13–0.5 mEq/kg/d

Calcium (Ca gluconate)

0.3–2 mEq/kg/d


Electrolyte conversion Ca

1 mmol = 2 mEq = 40 mg

P


Na


K


Mg


Cl




% Dextrose

44


If the patient has acidosis, consider adding a base to TPN such as Na acetate or K acetate. Bicarbonate will change the pH and result in calcium and phosphorus precipitation in the TPN. 7. Vitamins, minerals, trace elements.

A B1 B2 B6 B12 C D E K Additional Product (IU) (mg) (mg) (mg) (mg) (mg) (IU) (IU) (μg) information Infuvite® (adult)

3300 6

Infuvite® 2300 1.2 (pediatric)

3.6

6

5

200

200

10

150

Biotin 60 mcg Folic acid 600 mcg Niacinamide 40 mg Dexpanthenol 15 mg

1.4

1

1

80

400

7

200

Biotin 20 mcg Folic acid 140 mcg Niacinamide 17 mg Dexpanthenol 5 mg

IU = International Units; values are expressed as per 10 mL. Adapted from Texas Children’s Hospital Formulary.

Trace minerals added to TPN are zinc, copper, selenium, chromium, and manganese. (Iodine and molybdenum are not added in TPN solutions.) Most hospitals automatically add the trace minerals to TPN, but check with your institution. Most hospitals also automatically add vitamins based on the patient’s weight; check with your institution. 8. Special circumstances. • TPN cholestasis is an incompletely understood complication of TPN characterized by cholestasis, liver injury, cirrhosis, and progressive dysfunction. An elevated serum GGT level may herald an increasing conjugated bilirubin. Ursodeoxycholic acid has been used to mitigate liver injury (the exact mechanism of action in humans has not been fully described, although several theories exist) when cessation of TPN is not feasible. Preventive measures include cycling TPN and feeding enterally when possible (although the efficacy of TPN cycling has not been determined in a randomized, controlled trial). • Hypertriglyceridemia may occur from rapid infusions of IV fats or intolerance to IV fat infusion (eg, because of altered metabolism as in premature infants). Triglycerides are elevated in patients on steroids, those with sepsis or pancreatic disease, and in patients with diabetes. Always check baseline TG before starting TPN in patient with any of these problems. Then check the level in 4 hours. Consider decreasing the rate of lipids if TG level is >200 to 250 mg/dL. • Hyperglycemia may occur with high infusion rates and may be exacerbated by corticosteroids, sepsis, or trauma. It is important to remember that excess carbohydrate administration may cause increased carbon dioxide production. • Carnitine may assist with fat metabolism. Add L-carnitine (10–20 mg/kg/d) if an infant will be on exclusive TPN for more than 2 weeks. 9. Final check. • Calculate the total caloric content of TPN (kcal/kg/d) based on the amount of dextrose and protein and lipids (a standard 20% lipid emulsion provides 2 kcal/cc) • Does 70% of the calories come from dextrose and protein?


45

• Is the GIR in an acceptable range? A GIR of 6 to 15 mg/kg/min is generally well tolerated; however, the initial GIR should be 4.5 to 6.0 mg/kg/min. (For premature infants, start at a GIR of 4.5 to 6.0 mg/kg/min and advance as tolerated to a goal of 8 to 12 mg/kg/min.) GIR = [% Dextrose (g/100 mL)] × [Volume (mL/kg/d)]/1.44

Initially (Until TPN Composition is Stable)

Follow-up

Intake and Output

Daily

Daily

Electrolytes, BUN

2–3 times/wk

Weekly

Ca, P, Mg

Weekly

Weekly to every other week

Prealbumin∗

Weekly

Weekly

Albumin∗

Baseline

Every 3 weeks

Triglycerides

4 h after initiating infusion

4 h after infusion rate change

ALT (SGPT)

Baseline

Every other week

Bilirubin (direct and indirect)

Baseline

Weekly to monthly

∗As clinically indicated. Reproduced with permission from Pediatric Nutrition Reference Guide, 8th ed:83.

FORMULA SELECTION OVERVIEW • Human milk • Ideal form of nutrition. • AAP recommends all babies be exclusively breastfed at least until age 6 months and continued with addition of complimentary foods until 12 months of age. • Contains protein, energy, and cellular and humoral immunologic factors. • First 5 days: Colostrum, which is rich in immunoglobulins • After 5 days: Transitional with increased fat and lactose • Contains approximately 20 kcal/oz. • May be used in LBW infants but does not have enough calcium, protein, and phosphorus to meet the needs of VLBW infants. • VLBW infants on human milk require the addition of a human milk fortifier. • Protein as 20% casein, 80% whey. • Carbohydrate is lactose. • Must supplement vitamin D if breastfeeding. • Contraindications • Galactosemia in an infant. • Mothers receiving radioactive isotopes. • Maternal HIV. • Mothers using drugs of abuse.


• Does 30% of calories come from fat? Remember that fat should be at least 5% of calories to prevent essential fatty acid deficiency. • Have I exceeded 4 g protein/kg/d? If so, the patient may develop azotemia. • Are there any electrolyte abnormalities that I need to correct? • Do I need to check triglycerides 4 hours after initiating TPN? • Have I written for monitoring labs based on my patient’s need? 10. Lab monitoring (neonates and pediatric and adolescent populations) and advancement.

46


• Active untreated TB. • Active herpes simplex lesions on the breast. • Maternal human T-cell lymphotrophic virus type I or II infection. • For safety profile of medications while breastfeeding. see www.toxnet.nlm.nih.gov (click LactMed). • Cow’s milk–based term formulas • Most useful for term infants with no special nutritional or GI requirements, if human milk is not available. • Protein: Various whey:casein ratios (60:40, 48:52, 100:0, 18:82) • Carbohydrate is lactose. • Soy-Based Formula • Lactose free. • Indications: Galactosemia, parental vegetarian diet preference or congenital lactase deficiency (extremely rare). • Frequently misused for infants with cow’s milk protein (CMP) allergy. An unacceptably high proportion of CMP allergic infants (~30%) will cross-react to soy protein. • Cow’s milk–based premature formulas. • Protein: Whey, 60%; casein, 40% • Carbohydrate is a combination of glucose polymers and lactose. This is easier to absorb than lactose alone. • Fat is a mixture of long chain triglycerides and medium chain triglycerides. • Increased vitamins A and D, calcium, phosphorus, and electrolytes. • Premature infant follow-up formulas. • Recommended for former premature infants from 2 kg to 1 year of age. • Protein is whey, 50% to 60%; casein, 50% to 40%. • CHO is 50:50 mix of glucose polymers and lactose. • Fat is MCT:LCT 25:75. • Protein hydrolysate formulas. • Indicated in infants with known cow’s milk protein allergy or galactosemia. • Protein is hydrolyzed to shorter peptides and thought to be less allergenic than intact proteins. • Free amino acid formulas. • Used when protein hydrolysate fails to remedy symptoms of cow’s milk protein allergy. • Lactose-free formulas. • For infants with primary or secondary lactose intolerance. • Low-carbohydrate formulas. • Indicated for children with severe disaccharidase deficiency or carbohydrate intolerance. • Carbohydrate in the form of glucose polymers or fructose must be added to make these formulas nutritionally complete. • Metabolic formulas • For patients with inborn errors of metabolism to bypass the enzymatic defects.

Formula Mixing Guide Caloric Density (kcal/oz)

Liquid Mead Johnson Concentrate∗ Powder† All brands

Enfamil Lipil, AR,‡ LactoFree Lipil, ProSobee, Next Step

Nestle Powder†

Abbott Powder†

Nestle Good Start

Similac Advance, Similac Sensitive, Lactose Free, Isomil and Isomil 2, Similac 2, Alimentum or PM 60/40 (continued on next page)

Neonatal And Pediatric Nutrition Caloric Density (kcal/oz)

47

Nestle Powder†

Abbott Powder†

20

1 oz conc. + 1 oz sterile water

1 unpacked scoop + 2 oz sterile water = 2.2 oz (65 mL) formula

1 unpacked scoop + 2 oz sterile water = 2.25 oz (67.5 mL) formula

1 unpacked scoop + 2 oz sterile water = 2 oz (60 mL) formula

24


3 unpacked scoops + 4.8 oz (144 mL) sterile water = 5.6 oz (168 mL) formula


3 unpacked scoops + 5 oz (150 mL) sterile water = 6 oz (180 mL) formula

27


2 unpacked scoops + 3 oz (90 mL) sterile water = 3.5 oz (105 mL) formula


3 unpacked scoops + 4.25 oz (127.5 mL) sterile water = 5 oz (150 mL) formula

∗Preparation from concentrate does not vary from brand to brand. Use sterile water to reconstitute powdered formula for all infants younger than age 3 mo. † Must use the scoop provided with the container. ‡ Do not concentrate Enfamil AR to more than 24 kcal/oz because of increased viscosity. Reproduced with permission from Pediatric Nutrition Reference Guide, 8th ed:44.

Human Milk Concentration with Infant Formula Powder Caloric Density (kcal/oz)∗ Human Milk + Powder to Yield ã

22

24

27

30

g powder/tsp

Similac NeoSure Advance with Iron

1.3

2.6

4.5

6.5

2.1

Enfamil EnfaCare Lipil with Iron

1.4

2.7

4.8

6.8

2.4

Nestle Good Start Supreme with DHA and ARA

1.3

2.6

4.6

6.5

2.2

Similac Advance with Iron

1.3

2.5

4.4

6.3

2.1

Enfamil Lipil with Iron

1.3

2.6

4.5

6.4

2.3

∗All values represent the number of grams of powder preparation to add per 100 mL of human milk to make the desired caloric concentration. Reproduced with permission from Pediatric Nutrition Reference Guide, 8th ed:42.


Liquid Mead Johnson Concentrate∗ Powder†

48


Human Milk Fortifier (HMF) g 4 packets

mg (mEq)/4 packets

Product

CHO

Fat

Prot

Na

K

Ca

P

Fe

VitD (IU / dL)

Enfamil HMF (Mead Johnson)

6yo), 1% permethrin rinse or overnight application of 5% cream, or Ivermectin (200 mcg/kg PO once; may repeat in 7–10 days). Also wash bedding in hot water.

Scabies • Clinical features: Pruritis (worse at night and with hot bath); small erythematous papules with excoriations or crusting in the GU area, on interdigital webs, wrist, ankles, feet, axilla, buttocks, areolae. May see vesicles, burrows. May present on scalp and face in infants. • Treatment: Apply 5% Permethrin cream applied overnight (neck down), rinse in AM (for infants 10 mm Hg), diminished heart sounds, tachycardia, JVD. Cardiac tamponade may demonstrate Beck’s triad, which includes hypotension, increased CVP, and diminished heart sounds. • ECG: Progression of changes = diffuse ST elevation or PR depression (>80% of patients) → normalization of ST segments → widespread T-wave inversion → normalization of T waves (see below).

72

Pediatrics

Treatment of Pericarditis • NSAIDs, corticosteroids, rarely colchicines (Pediatr Cardiol 2000;21(4):395). Treatment of Effusion • Large effusions or evidence of tamponade physiology: Drainage via pericardiocentesis • Bacterial pericarditis or chronic, recurrent effusions: Surgical drainage with pericardial window

CARDIOMYOPATHIES Dilated Cardiomyopathy • Definition: Cardiomyopathy with increased ventricular size and reduced contractility in the absence of coronary, valvar, or pericardial disease. • Common associations: Mitral insufficiency, ventricular ectopy and tachyarrhythmias. • Newborns and infants have highest rate of disease; boys are more often affected than girls. • Without transplantation, 1-year mortality is approximately 50% (sudden cardiac death or ventricular failure) ( JACC 2010;55(13):1377). Genetics • Approximately one-third of patients with DCM have a familial form. AD (most common is cytoskeletal protein involvement), X-linked (typically occurs in boys with rapid progression; elevated CK-MM), Barth syndrome (LV noncompaction common). • Nearly all genes identified for inherited DCM also cause skeletal myopathy. • Presentation: Shortness of breath, fatigue, orthopnea, diaphoresis, chest pain, palpitations, exercise intolerance, syncope. • Physical exam: Tachypnea, tachycardia, diaphoresis, S3 or S4 gallop, holosystolic murmur (MR), hepatomegaly, ± ascites or peripheral edema.

EVALUATION OF DILATED CARDIOMYOPATHY Diagnostic Study

Key Findings

CXR

Cardiomegaly, pulmonary edema, atelectasis from bronchial compression (LA dilation), pleural effusions

ECG

Sinus tachycardia, LAE, ventricular ectopy and arrhythmias, bundle branch block

Echocardiogram

Reduced contractility, ventricular dilation, MR, pericardial effusion

24-hr Holter

Ventricular arrhythmia, AV block, or bradycardia

Exercise testing

May provide data regarding ventricular arrhythmias and physiologic reserve

Cardiac catheterization

Elevated LVEDP, and pulmonary vascular resistance, obtain biopsy specimen for viral PCR and mitochondrial studies

Skeletal muscle biopsy

When primary muscle disease is suspected, send microscopy, electron microscopy, electron transport chain biochemistry

Metabolic Studies

Specific Studies

Serum

CBC, acylcarnitine profile (fatty acid oxidation defects), lactate, pyruvate dehydrogenase complex, electrolyte profile, creatine kinase (+ isoform analysis if elevated), amino acids, genetic testing

Urine

Amino and organic acid profiles

Cardiology

73

Treatment Outpatient Management • ACE inhibitors and β-blockers (metoprolol/carvedilol): Mainstays of therapy • Diuretics: May be useful for symptomatic edema; no proven long-term benefit • Digoxin: Now used less often, but may improve symptoms in some patients • Vitamins (coenzyme Q10, carnitine, thiamine, riboflavin): Usually reserved for mitochondrial or metabolic disease in any type of cardiomyopathy Inpatient Management • Inotropic medications avoided whenever possible; reserved for compromised hemodynamic states (milrinone ± low-dose dopamine) • Diuretics: Decrease pulmonary edema acutely; may improve preload in volume overload states • Nesiritide: Recombinant B-type natriuretic peptide infusion; improves urine output • Mechanical circulatory support: Ventricular assist devices and ECMO for those who fail medical management as a bridge to recovery or transplantation • Cardiac transplantation

ELECTROPHYSIOLOGY EKG Interpretation NORMAL PEDIATRIC ELECTROCARDIOGRAM VALUES Heart Rate (bpm)∗

Frontal Plane QRS PR Vector Interval (degrees)∗ (sec)∗

3 yr: 0.05–0.1 s; normal ampliII III tude 98% for age) • Short: Accessory pathway, enhanced AV node conduction, increased AV node cell size (mannosidosis, Fabry disease, Pompe disease), low RA rhythm • QRS: normally < 0.1 sec for > 1 year and < 0.08 sec for < year • QT interval: Interval from the start of the QRS complex to the end of the T wave measured in leads II or V5 normally (measured from shortest R-R interval) • Must be corrected for heart rate (QTc = QT interval/√preceding R-R interval) • U waves not counted in QT interval unless > 50% of T wave amplitude • Normal 5 yr: R in V6 >30 mm • Child 98% for age • Possible LVH: • Child >5 yr: R in V6 above 98% for age but below 30 mm. • S in V1 more than 5 mm above 98% for age • Q in V6 or III >98% for age + prominent LV forces exist

Cardiology

75

Arrhythmias (Ped Clin N Am 2006;53(1):85) DIFFERENTIATION OF SINUS TACHYCARDIA AND SVT Sinus Tachycardia

SVT

Rate

Usually < (220 - age)

Usually >(220 - age)

P waves

Present, normal axis

Absent, buried in QRS, or abnormal axis

RR interval (rate variability)

Varies

Fixed

Onset

Gradual

Abrupt

Termination

Gradual

Abrupt

Response to vagal maneuvers

Gradual slowing and gradual return

± abrupt termination

Synchronized cardioversion

No response

Usually abrupt termination

P E D I AT R I C S

• RVH: • R in V1 >98% for age • Upright T wave in V1 between ages 1 wk and 6 yr • QR in V1 (Q wave >1 box wide) • QR in V3r and V4r: Presence of Q and R height >mean for age (R V1 normals) (7 mm (5+ yr) • rSR’ with R’ >15 mm (10 mm (>1 yr) • Possible RVH: • S in V6 >98% for age • Pure R in V1 in child >6 mo • RAD + prominent RV forces • RV pressure estimate = R in V1 + 47 mm Hg or 5 × R in V1 • RV strain: tall R with asymmetrically inverted T wave in V1 ± ST depression • BVH: • R + S in any lead >60 (55 (>1 yr) • Possible BVH: • Meets criteria for LVH + exceeds mean values for RVH or meets criteria for RVH + exceeds mean values for LVH precordial voltage normals. • Ischemia and Infarction • Q waves • Abnormal width: >1 box (any lead) • Abnormal height: aVL >2 mm, I >3 mm, II and aVF >4 mm; otherwise variable • PR segment depression ± ST segment elevation: Pericarditis • ST segment • Abnormal: Limb leads: >1 mm above or below baseline (TP segment); precordial leads: >2 mm above or below baseline (TP segment is baseline). • T waves • Should be symmetric, 9 mEq/L: Arrhythmias, AV block, VT, VF.

Cardiology

77

• Calcium • Hypocalcemia: Prolongs T wave (and therefore QT interval); may see AV block; other arrhythmias uncommon. • Hypercalcemia: Shortens QT interval; may slow sinus node. • Magnesium • Hypomagnesemia: ECG effects are similar to hypokalemia. • Hypermagnesemia: May prolong the PR interval. Drugs • Tachyarrhythmias • Most antiarrhythmic drugs have proarrhythmic effects. • Drugs that are catecholaminergic may be proarrhythmic. • Drugs that prolong the QT interval (refer to www.torsades.org) may result in torsades de pointes and sudden death in patients with long QT syndrome.

OVERVIEW OF ANTIARRHYTHMICS VaughanWilliams Classification

Mechanism of Action

Example

Target

Arrhythmia to Treat

Sodium channel blockade

Procainamide, quinidine, disopyramide

Atrium and ventricle

AET, AFlutter SVT, VT

Class 1B


Lidocaine, phenytoin, mexiletine

Ventricle

VT

Class 1C


Flecainide, propafenone

Atrium

AET, AFlutter, SVT

Class II

β-Blockade

Propranolol, atenolol, nadolol

SA and AV node

SVT, AET, VT

Class III

Potassium channel blocker

Sotalol, amiodarone

Atrium, ventricle

AET, AFlutter, Afib, SVT, VT

Class IV

Blocks L-type calcium channels

Verapamil, diltiazem

SA and AV node

SVT, VT

Class V

Digitalis agents

Digoxin

SA and AV node

Rate control in Afib

Afib, atrial fibrillation; Afl, atrial flutter.

Guide to Pacemakers Who Needs a Pacemaker? • For guidelines for device-based therapy in adults and children see Circulation, 2008;117(21):e350 and for those with CHD, see Pacing Clin Electrophysiol 2008;31 (suppl 1):S17. Types of Pacemakers • Transvenous: Leads inserted through systemic vein onto the endocardial surface; may be single (atrial only or ventricular only) or dual chamber (atrial and ventricular). • Epicardial: Leads placed surgically on the epicardial surface; may be single or dual chamber. Usually placed in smaller children or patients with complex congenital heart disease. • Biventricular: May be epicardial or transvenous; paces both the right and left ventricles; used predominantly in patients with symptomatic heart failure (“resynchronization therapy”).

P E D I AT R I C S

Class I A

78

Pediatrics

CHEST PAIN (Pediatr Clin North Am 2004;51(6):1553) The vast majority of pediatric chest pain is benign; often recurrent.

“Red Flags”: (suggestive of cardiac etiology) • History: Pain has crushing quality; associated with syncope, nausea, palpitations; may be exercise induced • Medical history: Kawasaki disease (chest pain in a patient with known CA aneurysms is ischemia until proven otherwise); homozygous familial hypercholesterolemia; aortic stenosis; prior congenital heart surgery involving manipulation of the coronary arteries (eg, arterial switch operation for d-TGA, reimplantation for anomalous origin of a coronary artery, Ross procedure), Marfan syndrome or other connective tissue disease (risk for dissection) • Physical exam: Tachycardia, diaphoresis, pallor, abnormal heart murmur

“Green Flags”: (suggestive of non-cardiac etiology) • History: Pain is brief, infrequent, transient, worse with inspiration, worse after meals, not exercise induced; prolonged history of similar pains (weeks to months). • Medical history: Reactive airways disease (RAD), recent psychosocial stressor, GERD. • Physical exam: Reproducible pain on exam (ie, chest wall tenderness to palpation).

Differential Diagnosis 1. Idiopathic (most common) a. Sharp, recurrent, normal exam 2. Musculoskeletal (second most common) a. Ask about trauma, especially weight lifting, sports, heavy school bag b. Costochondritis: Reproducible pain at costochondral/costosternal junctions; sharp, worse with activity, recurrent c. Chest wall strain d. Precordial catch syndrome: Localized anterior chest pain, sudden onset, very transient (typically lasts only seconds) 3. Psychogenic a. Inquire about recent psychosocial stressors b. Vague, changing, long-standing pain; patient or parent often anxious 4. GI a. GERD: Pain after eating; worse in recumbent position 5. Respiratory a. Exercise-induced RAD: Chest “tightness” with SOB, coughing after exercise, resolves with rest, improves with bronchodilators b. Pneumonia, empyema: Fever, cough, rales and dullness to percussion c. Pneumothorax: Most common in tall, thin boys d. Pulmonary embolus: Rare in children 6. Cardiac a. Myocardial ischemia i. Rare in children, even in children with heart disease ii. Crushing, diffuse, unrelenting, not sharp iii. Seen with anomalous coronary artery, history of coronary aneurysms (Kawasaki disease), cocaine use b. Arrhythmia i. Sustained arrhythmias, especially tachyarrhythmias, may be uncomfortable, often described as chest tightness; younger children often describe this as chest pain; in some studies, the most common cardiac cause of chest pain is arrhythmia c. Inflammatory i. History of preceding viral illness ii. Pericarditis: Fever, friction rub, worse lying flat iii. Myocarditis: Tachycardia, gallop rhythm

Cardiology

79

d. Structural (very rarely associated with chest pain in children and adolescents) i. Aortic stenosis ii. Hypertrophic obstructive cardiomyopathy iii. Aortic dissection: Particularly in Marfan syndrome Workup • The history and physical exam are sufficient in most cases. Typically, the ECG is the most useful diagnostic test.

SYNCOPE (Cardiol Clin 1997;15(2):277) • Definition: A sudden and transient loss of consciousness and muscle tone that is spontaneously self-terminating • Secondary to insufficiency of nutrients or oxygen to the brain (related to blood flow), most typically from decrease in systemic arterial pressure beyond the limits tolerated by cerebral autoregulation (typically when cerebral blood flow 0.07 • PaO2 0.40 (PaO2/FiO2 ratio 5%

(Ped Crit Care Med 2009;10(1):1.)

STATUS ASTHMATICUS IN THE INTENSIVE CARE UNIT • Definition: An acute exacerbation of asthma that does not respond to treatment with bronchodilators and corticosteroids. • Symptoms: Chest tightness or pain, dyspnea, dry cough, or wheezing. Ppatients may have nausea, vomiting, difficulty speaking or speaking in single words, or altered mental status as part of the presentation, which indicates greater severity of illness. Risk Factors for ICU Admission and for Sudden Death with Status Asthmaticus • Previous ICU admissions • Previous need for mechanical ventilation with an asthma exacerbation • Syncope during an asthma exacerbation • Seizures during an asthma exacerbation • History of cardiac arrest with asthma exacerbation • Poor adherence to controller therapy • Poor perception of severity of asthma • Comorbid psychiatric disorder • Rapid deterioration with current episode • Use of more than 1 canister of home β-agonist medication per month

CLINICAL RESPIRATORY SCORE AS A GUIDE FOR EVALUATING SEVERITY OF EXACERBATION AND FOLLOWING RESPONSE TO THERAPY Clinical Respiratory Score (CRS) Patient Score

Assess

Score 0

Score 1

Score 2

Respiratory Rate

< 2 mos < 50 2-12 mos < 40 1-5 yrs < 30 > 5 yrs < 20

< 2 mos 50-60 2-12 mos 40-50 > 1-5 yrs 30-40 > 5 yrs 20-30

< 2 mos > 60 2-12 mos > 50 > 1-5 yrs > 40 > 5 yrs > 30 (continued on next page)

Critical Care

91

Clinical Respiratory Score (CRS) Patient Score

Assess

Score 0

Score 1

Score 2

Auscultation

Good air movement, scattered expiratory wheezing, loose rales/crackles.

Decreased air movement, inspiratory and expiratory wheezes or rales/ crackles

Diminished or absent breath sounds, severe wheezing or rales/ crackles, or markedly prolonged expiration.

Use of Accessory Muscles

Mild to no use of accessory muscles. Mild to no retractions or nasal flaring on inspiration.

Moderate intercostal retractions, mild to moderate use of accessory muscles, nasal flaring.

Severe intercostal and substernal retractions, nasal flaring.

Mental Status

Normal to mildly irritable

Irritable, agitated, restless.

Lethargic

Room Air SpO2

> 95%

90 – 95 %

< 90%

Color

Normal

Pale to normal

Cyanotic, dusty

Total Score (Sum of the component scores)

THERAPIES FOR THE MANAGEMENT OF STATUS ASTHMATICUS First-Line Treatment Route

Dose

Albuterol

SABA (airway smooth muscle relaxation)

Inhaled (nebulized or MDI)

Nebulization: 0.15–0.5 mg/kg/h or 10–20 mg/h of continuous therapy; can give sequential nebulization × 3 (≤5 mg each) Max, 20 mg/h MDI: 4–8 puffs per dose Q20 min × 3; then Q1h

Levalbuterol

SABA (levorotary enantiomer, airway smooth muscle relaxation)


MDI: 4–8 puffs Q20 min × 3 then Q1h

Methylprednisolone

Steroid∗ (antiinflammatory)

IV

2 mg/kg IV once; then 1 mg/kg IV Q6h Max, 60 mg/dose

Prednisone

Steroid∗ (antiinflammatory)

PO

2 mg/kg PO once; then 1–2 mg/kg/ day divided Q12h Max, 60 mg/dose

Prednisolone

Steroid∗ (anti inflammatory)

PO (tastes better)

2 mg/kg PO once; then 1 mg/kg PO Q6h Max, 60 mg/dose (continued on next page)

P E D I AT R I C S

Type and Mechanism

Medication

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Pediatrics

Second-Line Treatment Ipratropium bromide

Anticholinergic (airway smooth muscle relaxation)


12 yr: Nebulization 500 mcg Q30 min × 3 then Q2–4h prn; MDI 4–8 puffs per dose Can “spike” the albuterol nebulization solution with ipratropium bromide dose and give as one treatment

Magnesium sulfate

Electrolyte (smooth muscle relaxation)

IV

40 mg/kg IV over 20 min; max 2g Although hypotension is exceedingly rare, watch for hypotension and be ready to give a 10–20 cc/kg NS bolus if it develops

Terbutaline†

β-agonist (smooth muscle relaxation)

Sub-Q, IV

IV: 10 mcg/kg IV once; then IV infusion starting at 0.5 mcg/kc/min, titrate as HR tolerates to goal 3–6 mcg/kg/min Max, 10 mcg/kg/min

Aminophylline‡

Bronchodilator

IV

6 mg/kg load over 20–30 min; then continuous infusion

Ketamine

Dissociative anesthetic, bronchodilator

IV

0.5–2.0 mg/kg IV; may be used as a continuous infusion May have bizarre dreams, hallucinations; watch for oversedation and need for immediate intubation Often reserved as a last medication trial or sedative before intubation

Heliox

Gas

Inhaled

Available as 70/30 or 80/20 concentrations

∗In general, IV or PO steroids are continued as a “burst” over 5 to 7 days and then discontinued. Remember to wean the patient off the steroids if they are continued after 7 days because they may cause adrenal suppression. † The usual doses for IV terbutaline infusion range from 1 to 6 mcg/kg/min. Major side effects may include coronary ischemia, chest pain, and arrhythmias. Toxicity will limit the maximum dose for the patient. Closely monitor for side effects. ‡ Aminophylline levels must be monitored closely to avoid toxicity. Obtain the first level 30 minutes after the end of the 30-minute loading dose. Continuous infusion dosing is based on the age and weight of the child. Repeat levels after the loading dose should be obtained 12 to 24 hours after the continuous infusion begins. There are many medication interactions; check with the pharmacy to avoid medication errors.

Advanced Therapies and Treatment Modalities These therapies are rarely needed in the management of patients with status asthmaticus but may be necessary for a small subset of patients who fail to respond to conventional management. • BiPAP • Intubation and mechanical ventilation • Gas anesthesia with Isoflurane • ECMO Indications for Intubation and Mechanical Ventilation for Patients in Status Asthmaticus • Hypoxemia, severe work of breathing, altered mental status, impending or present respiratory or cardiac arrest

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Intubating Patients with Asthma • Use ketamine for sedation (secondary to its bronchodilatory properties). • Be prepared to give an IVF bolus of NS (10–20 cc/kg) because the patie4nt may become hypotensive or experience cardiac arrest after intubation. • Initial mechanical ventilator settings: Titrate settings for adequate chest rise with the goal of 6 to 8 cc/kg delivered VT; minimize peak inspiratory pressure; goal plateau pressure 7.25) and higher pCO2 (ie, permissive hypercapnia).

Pearls for Monitoring and Managing ICU Patients with Status Asthmaticus Airway and Breathing • The decision to intubate is clinical and should not be determined solely by ABG values. • Most complications for patients intubated for asthma occur during intubation or immediately afterward (pneumothorax, cardiac arrest, hypotension, arrhythmia, among others). • Intubated patients with status asthmaticus may require a very slow respiratory rate to complete exhalation. Patients with a high pCO2 may require a decrease in ventilator rate to improve the pCO2. Circulation and Cardiac • Monitor for atrial and ventricular arrhythmias, especially SVT in patients on high-dose β-agonist therapy. • Monitor for cardiac ischemia and chest pain. Obtain a 12-lead ECG. Consider obtaining cardiac enzymes in patients who have chest pain or ECG changes. • Limit β-agonist therapies when excessive tachycardia is present or there is evidence of cardiac ischemia. Fluid, Electrolytes, and Nutrition • Keep the patient NPO. (The patient may deteriorate, have altered mental status, and require mechanical ventilation.) • Check potassium Q6h because high-dose β-agonist therapy may cause hypokalemia. • Patients in status asthmaticus are often dehydrated and may require fluid resuscitation. Do not be afraid to give fluid to patients with asthma. • Status asthmaticus has been associated with metabolic and lactic acidosis.

• Shock Definition: Inadequate delivery of oxygen and nutrients to meet the metabolic needs of the tissues. Shock is not defined by low BP. A patient can have a normal BP and still be in shock.

Stages of Shock • Compensated (warm): Normal to low SBP and low DBP with signs and symptoms of inadequate tissue and organ perfusion. Compensatory mechanisms: ↑HR, ↑ cardiac contractility (↑SV), and ↑ SVR. (Younger children and infants are less able to compensate and rely on tachycardia.) • Clinical signs: Warm extremities with bounding pulses, tachycardia, tachypnea, altered mental status, ↓ UOP. • Physiologic signs: Widened pulse pressure, ↑ CO, ↓ mixed venous saturation, ↓SVR. • Biochemical abnormalities: Hypocarbia, ↑ lactate, ↓ bicarbonate, hyperglycemia. • Decompensated (cold): Systolic hypotension with signs and symptoms of inadequate tissue and organ perfusion. Compensatory mechanisms are overwhelmed. • Clinical signs: Cyanosis, cold and clammy skin, rapid and thready pulses, shallow respirations, obtundation, worsening oliguria or anuria. • Physiologic signs: ↓ Mixed venous sats, ↓ CO, ↓ CVP, ↑ SVR, ↓ UOP, myocardial dysfunction, capillary leak. • Biochemical abnormalities: Worsening metabolic acidosis, hypoxia, coagulopathy, hypoglycemia, thrombocytopenia, DIC.

P E D I AT R I C S

SHOCK STATES, SIRS, AND SEPSIS

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Pediatrics

Defining Shock in Sepsis Fluid-Refractory, Dopamine-Resistant Shock • Shock persists despite ≥60 mL/kg fluid resuscitation in the first hour and dopamine infusion to 10 microg/kg/min. Catecholamine-Resistant Shock • Shock persists despite use of catecholamines (epinephrine or norepinephrine). Refractory Shock • Shock persists despite goal-directed therapy use of inotropic agents, vasopressors, and vasodilators and maintenance of metabolic (glucose and calcium) and hormonal (thyroid and hydrocortisone) homeostasis.

Defining Hypotension (SBP 70%

Cold shock with normal blood pressure:

Cold shock with low blood pressure:

Warm shock with low blood pressure:

1° goals: Titrate epinephrine, MVO2 > 70%, Hgb > 10g/dL

1° goals: Titrate epinephrine, MVO2 > 70%, Hgb > 10g/dL

1° goals: Titrate norepinephrine, MVO2 > 70%,

2° goals: Add vasodilator* (nitrosovasodilators, milrininone, and others) with volume loading, consider levosimendan

2° goals: Add norepinephrine Add dobutamine if MVO2 < 70%, Consider milrinone, enoximone or levosimendan

2° goals: Consider vasopressin, terlipressin or angiotensin Add dobutamine or low dose epinephrine if MVO2 < 70%

P e d i a t r i c

shock not reversed? Persistent catecholamine resistant shock: Rule out and correct pericardial effusion, pneumothorax, and intra-abdominal pressure > 12 mm/Hg. Use pulmonary artery catheter, PICCO monitor, FATD and/or doppler ultrasound to guide fluid, inotrope, vasopressor, and hormonal therapies. Goal C.I. > 3.3 and < 6.0 L/min/m2 shock not reversed? Refractory shock: ECMO (110 mL/kg/min) and/or CRRT (> 35 mL/kg/hr)

Figure 9-3 Stepwise management of hemodynamic support in infants and children. (Adapted from Crit Care Med 2009;37(2):666–688.)

P E D I AT R I C S

I n t e n s i v e

c a r e

E m e r g

97

Recognize decreased mental status and perfusion. Begin high flow O2. Establish IV/IO access.

e n c y

D e p a r

t m e n t

Critical Care

98

Pediatrics

• Remember after appropriate fluid resuscitation and initial dopamine: • Cold shock: β-adrenergic such as epinephrine (treat the poor cardiac output). • Warm shock” α1 adrenergic such as norepinephrine or vasopressin (vasocontrict the peripheral vessels). • Calcium repletion: Either as bolus dosing or continuous infusion may significantly improve contractility. • Begin antimicrobial coverage ASAP! Use IV cephalosporin and vancomycin initially. Consider covering for Pseudomonas spp. if the airway was previously instrumented. Cardiovascular • Differentiate between cardiogenic shock and other types early; cardiogenic shock treatment does not include ongoing fluid resuscitation with crystalloids or colloids to improve or maintain BP and perfusion. • Look for signs of fluid overload and myocardial dysfunction on exam as you resuscitate: Crackles on lung exam, S3, hepatomegaly; if any are present, slow down on the fluid and titrate up the vasopressors or inotropes. • Hemodynamic goals: BP resulting in end-organ perfusion, normal HR for age, cerebral perfusion pressure normal for age (MAP–CVP, 55–60 cm H2O in infants; 65 cm H2O for ≥2 yr), MVO2 sat of 70%, CVP 8 to 12, capillary refill time 1 cc/kg/h. Respiratory • Intubate patients in shock when AMS is present or GCS is decreasing or 300 mg/dL and the presence of acidosis with a serum bicarbonate 320 with pH >7.3 and absence of ketosis

Prognosis • Mortality rate for DKA is 0.15% to 0.3%; for HHS, it is 14%. • Prognosis is linked to the severity of illness at presentation, degree of dehydration, and electrolyte derangements. • Cerebral edema occurs in 0.5% to 1% of all episodes of DKA and is the most common cause of mortality and morbidity. • Coma is more frequent in HHS, as is long-term morbidity.

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99

DIAGNOSTIC CRITERIA FOR DIABETIC KETOACIDOSIS AND HYPERGLYCEMIC HYPEROSMOLAR STATE Diabetic Ketoacidosis

Hyperglycemic Hyperosmolar State

Mild

Moderate

Severe

Plasma glucose (mg/dL)

>250

>250

>250

>600

Arterial pH

7.25–7.30

7.00–7.24

7.30

Serum bicarbonate (mEg/L)

15–18

10 to 320

Anion gap

>10

>12

>12

Variable

Alteration in sensorial or mental obtundation

Alert

Alert or drowsy

Stupor or coma

Stupor or coma

Reproduced with permission from Kitabchi AE, Umpierrez GE, Murphy MB, et al: Hyperglycemic crises in diabetes Diabetes Care 2004;27(suppl 1):S94–S102.

Important History • Polyuria, polydipsia, increased thirst. • Weight loss. • Abdominal pain (DKA only) or vomiting. • Headache, altered mental status.

• May have a illness or infection triggering DKA. • In a patient with known diabetes mellitus, there may be a history of missed insulin doses. • Family history of DM or other autoimmune disorders.

• Correct the dehydration over 36 to 48 hours in addition to maintenance IVF. Initial IVF should be at 2.5 L/m2/d (including initial boluses) for slow correction of dehydration and avoidance of cerebral edema. Some patients may need additional fluids because of extreme dehydration or hemodynamic compromise. Do not exceed 4 L/m2/d. Determine the degree of dehydration based on physical exam or weight loss (if previous weight is known). • Do not give NaHCO3 to correct acidosis (associated with worsened outcome in the pediatric population). • Patients with HHS have greater fluid and electrolyte deficits than patients with DKA. • Correct hyperglycemia with insulin at a rate of decrease no more than 100 mg/dL/h; faster correction may increase the risk of cerebral edema. • K and Phos may appear normal on initial laboratory examinations, but patients in DKA or HHS are total-body K and Phos depleted; this may become apparent with correction of acidosis. • Red flags for cerebral edema: Altered mental status, increased vomiting, incontinence, cranial nerve palsies, abnormal pupillary responses, bradycardia, and increasing or decreasing BP.

P E D I AT R I C S

Correction of Acidosis, Dehydration, and Electrolytes

100

Pediatrics

Patient off algorithm, treat as appropriate

Yes

Interventions

Diagnostic Testing

Patient in shock?

For all patients: Β-hydroxybutyrate, glucose, CHEM 7, VBG, Hb A1C. Calculate and follow anion gap, osmolarity, corrected Na.

No

Consider subcutaneous insulin.

Bolus Lactated Ringer's 10ml/kg IV over 1 hour. May repeat once if clinically indicated

New onset diabetes patients Islet cell, insulin, and anti-GAD antibodies, C-peptide, insulin, thyroid peroxidase and thyroglobulin antibodies. Celiac panel

Age > Haemophilus influenzae (Post PCV7 52%, Pre PCV7 15-30%) > Pneumococcus spp. (Post PCV7 34%, Pre PCV7 25-50%) > Moraxella spp. (Pre PCV7 3%–20%) > GABHS > other (Pediatrics 2004;113:1451) • Diagnosis • History: Fever (especially increasing fever curve), URI, ear pain or fullness, hearing loss, vomiting, ear drainage or diarrhea. • Physical exam: Bulging tympanic membrane (TM), purulent material, air-fluid level, ↓ or no movement of TM, otorrhea, ± redness, ± bullae. • Best predictors are position (ie, bulging), mobility of TM (↓ or no movement of TM), and color (PIDJ 1998;17(6):540). Consider tympanocentesis and bacterial culture for children with recurrent or chronic disease. • Treatment • Pain and fever control are the most important interventions. • Within 24 hours, 61% of patients have resolution of symptoms without antibiotics. • Antibiotics (see table below) often do not change the duration of illness of AOM.

CRITERIA FOR INITIATION OF ANTIBIOTICS IN CHILDREN WITH AOM Diagnosis of AOM is Certain

Diagnosis of AOM is Uncertain

39°C (102.2°F)

Initial antibiotic choice

Treatment failure at 48–72 h after initial management

No

Amoxicillin, 80–90 mg/kg/day (If penicillin allergic: Non– type I: cefdinir, cefuroxime, or cefpodoxime; type I: azithromycin, clarithromycin)

Amoxicillin–clavulanate, 90 mg/kg/ day of amoxicillin with 6.4 mg/kg/ day of clavulanate (If penicillin allergic: Non–type I: ceftriaxone for 3 days; type I: clindamycin)

Yes

Amoxicillin–clavulanate, 90 mg/kg/ day of amoxicillin with 6.4 mg/kg/ day of clavulanate (If penicillin allergic, ceftriaxone for 3 days)

Ceftriaxone for 3 days (If penicillin allergic, tympanocentesis, clindamycin)

• Single high-dose azithromycin (30 mg/kg/dose) is equal to amoxicillin in efficacy. (PIDJ 2005;24:153) • Add topical agents as well for AOM with perforation or if the patient has tympanostomy tubes (eg, Ciprofloxacin; Ofloxacin). • Surgical treatment: Consider ENT referral if the patient has >3 episodes in 6 mo or >4 episodes in 1 yr. • Complications: Labyrinthitis, mastoiditis, intracranial extension, conductive hearing loss.

OTITIS MEDIA WITH EFFUSION (OME) • Epidemiology: Highest incidence Moraxella spp., bottle feeding, feeding supine, daycare attendance, allergies, smoke exposure • Diagnosis • Often OME is subjectively asymptomatic → no intervention required if no hearing loss; when symptomatic: • History: ↓ hearing, ear fullness, pressure, pain (rare), recent travel, diving, allergies (environmental) • Physical exam: Air fluid level, TM retraction or bulging, serous middle ear fluid, ↓ TM mobility, ± large tonsils, signs of atopy. • Tests: Hearing test if speech or language delay present • Treatment • Most spontaneously resolve → avoid smoke exposure; ↓ bottle feeding, daycare, and allergens. • More aggressive therapy if the child has a speech or language delay, PDD, craniofacial abnormality (eg, Down syndrome, cleft palate), visual impairment, or developmental delay • Antimicrobial agents: Consider amoxicillin, amoxicillin + clavulanate, trimethoprim– sulfamethoxazole, or erythromycin for 2 to 5 wk. • Tympanostomy tube: OME and >40-dB hearing loss → definite indication; OME and 21- to 40-dB hearing loss; or persistent effusion >3 mo → relative indications. • Steroids, antihistamines, decongestants, and mucolytics are not effective.

MASTOIDITIS • Definition: Suppurative infection of the mastoid air cells. • Pathophysiology: Because the middle ear cavity is contiguous with the mastoid, all middle ear infections cause some degree of mastoid inflammation. However, when inflammation

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127

leads to occlusion of mastoid aditus → ↑ pressure → relative hypoxia and acidosis → bony erosion into surrounding structures → potential for intra- or extracranial spread of infection.

DIAGNOSIS, ETIOLOGIC AGENTS, AND TREATMENT OF MASTOIDITIS Etiologic Agents

Diagnosis

Diagnosis

Acute mastoiditis

Fever (often GABHS > Staphylococcus aureus > others

ENT consultation;† Start, vancomycin/ clindamycin (oxacillin/ nafcillin depending on local prevalence of MRSA) and cefotaxime

Treatment*

Chronic mastoiditis

Usually with persistent ear drainage ± fever ± postauricular swelling Test: CT temporal bone with contrast

S. aureus, Pseudomonas spp., other GNRs, mycobacteria, fungal; 80% of chronic cases demonstrate coinfection with anaerobes

Would also include ticarcillin–clavulanate + gentamicin and topical ear drops (Ciprofloxacin or Cortisporin)

• Complications: Meningitis, intracranial abscess, hydrocephalus, dural sinus thrombosis, Bezold abscess, hearing loss, osteomyelitis of the temporal bone, and facial nerve palsy.

RHINITIS • Inflammation of the nasal cavity commonly manifesting as a “runny nose.” • Most common causes: Viral URI and allergic rhinitis (see table below); also consider sinusitis, and if there is unilateral nasal discharge evaluate for foreign body.

ALLERGIC VERSUS INFECTIOUS RHINITIS Allergic

Infectious

Onset

Subacute to chronic

Acute

Symptoms

Runny, itchy nose; allergic salute or shiners; other atopic disease (asthma, eczema); red, itchy eyes

Low grade fever; rhinorrhea or congestion; cough; sore throat; possible GI symptoms (diarrhea, vomiting)

Etiologic agents

Dust mites, ragweed, grass, dust, animal dander

Any respiratory virus

Treatment

Antihistamines, nasal steroids, nasal saline, leukotriene antagonists

Frequent nasal saline, encourage fluid intake, supportive care

P E D I AT R I C S

* If the patient has an acute onset of symptoms with minimal fluctuance and no signs of intracranial complications, the patient will likely respond to medical therapy with antimicrobials alone. When there has been favorable response change to PO medications for 3 weeks of total treatment (empiric therapy or guided by cultures/ sensitivities). † Consultation with ENT is always recommended for tympanocentesis early for culture and relief of pressure. Surgery is indicated especially if the patient has significant fluctuance, vertigo, neurologic symptoms, vomiting, hearing loss, or any other signs/symptoms of intracranial involvement.

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Pediatrics

SINUSITIS • Pathophysiology: Usually result of bacterial superinfection of viral URI or inflamed nasal mucosa secondary to allergic disease (sinus development: at birth, the maxillary and ethmoid sinuses are present; at age 3 yr, the sphenoid sinus starts developing; the frontal sinus starts developing ~5 yr) • Etiology • Respiratory viruses are the most common cause overall. • Acute bacterial causes: Streptococcus pneumoniae (>30%), H. influenzae (~20%), Moraxella catarrhalis (~20%) and GABHS. After PCV 7 vaccine ↓ incidence of pneumococci and ↑ rates of H. influenzae (≤40%). • Chronic bacterial sinusitis: Same organisms as acute (lower frequency) as well as α-streptococci, S. aureus, Enterobacteriaceae, and anaerobes (Fusobacterium and Peptostreptococci spp.). Fungi should also be considered, including Alternaria and Aspergillus spp. • Diagnosis • Presence of persistent or severe symptoms: • Persistent symptoms: URI symptoms ≥10 d (includes congestion, postnasal drainage, persistent daytime cough that is worse at night, headache, or sinus pain). • Severe symptoms: Fever ≥102°F + purulent nasal discharge for 3–4 days. • Physical exam is not very helpful in children. • Imaging is not necessary in the diagnosis of acute bacterial disease. • Treatment • AAP recommends antibiotic therapy for children with persistent (>10 d) or severe symptoms. • Start with amoxicillin (if the child is antibiotic naïve over the past 90 days) for 21 days; for chronic sinusitis treat with 3-6 weeks of antibiotics. • If treatment failure or persistent disease, start amoxicillin–clavulanate, cefdinir, cefpodoxime. • In patients allergic to β-lactams, may use azithromycin or clindamycin. • Complications • Chronic disease, orbital cellulitis, intracranial extension (especially in adolescent males), Pott’s puffy tumor.

OBSTRUCTIVE SLEEP APNEA • Affects ~5% children; common age is 5 to 7 yr; second peak in adolescence because of obesity.

CLEFT LIP AND PALATE • Background • Incidence of cleft lip is 1:1000. • Left > right > bilateral > midline; majority (80%) are unilateral. • Girls are affected more often than boys. • There are 3 types: Complete (into floor of nose), incomplete (partially through lip), and microform (notch in vermillion border). • About 15% of affected children also have another anomaly. • Incidence of cleft palate is 1:2,500; it always involves the uvula • About 56% of affected children also have another anomaly (most often midline), especially if associated with cleft lip. • Management • Immediate consult of cleft team (ENT, craniofacial surgery, genetics, OT and speech, dental, social work, nutritionist). • Feeding: ↑ Difficulty with cleft palate ± cleft lip than with cleft lip alone; use soft nipple, large opening, squeezable bottle (Haberman or similar); feed in an upright or sitting position with the head slightly tilted back; slow feeding; frequent burping because of ↑ air swallowing.

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129

Symptoms or Signs of OSA? YES

NO

Cardiac or respiratory complications?

Tonsils grade IV?

YES

NO

Expedited sleep study & referral to specialist

YES

NO

Sleep study

Continue screening at subsequent visits

High risk patient? (underlying craniofacial, neuromuscular or metabolic abnormalities, chronic lung disease or infant) YES

NO

Sleep study and referral to specialist

Sleep study

• Staged repair: Lip at age 2 to 3 mo; palate at age 6 to 24 months ± tympanostomy tubes. Usually, the repair is considered when the child is at least 10 wk of age, weigh at least 10l bs with a Hb of 10 or greater. • Complications • OM, speech difficulty, dental anomalies, hearing loss, poor weight gain

Available at www.AccessPediatrics.com • Pharyngitis • Adenotonsillar hypertrophy • Obstructive sleep apnea • Branchial anomalies

P E D I AT R I C S

Figure 12-1 Management of positive snoring screen in children. (Reproduced with permission from Pediatrics 2002;109:704.)

CHAPTER 13

Emergency Medicine FEVER WITHOUT LOCALIZING SIGNS (FWLS) Temp ê38°C (100.4°F) in patient 0–60 days, no source on exam No antibiotics to be given to this group w/o full workup including blood, urine, and CSF studies (NOTE: Some institutions follow this algorithm for children who are 0–3mo) 0–28 days

29–60 days Laboratory workup CBC, blood cx, UA with micro & cx1, CSF (cell count, Gram stain, glucose, protein) & CSF cx (hold CSF for viral studies) Consider: • Rapid viral testing (RSV/Influenza/adenovirus) • HSV workup2

YES Treat as clinically appropriate

Laboratory workup CBC, blood cx, UA1 with micro & cx

Febrile ê 48h

NO

NO

Toxic? (Lethargy, poor perfusion, hypo/hyperventilation, cyanosis, irritable)

NO

Admit to hospital on antibiotics 0–28d Ampicillin & gentamicin ê29d Ampicillin & cefotaxime (meningitis dosing if CSF pleocytosis/gram stain abnormal)

Any culture (+)

NO

YES

YES

CSF cx/studies, consider rapid viral testing → ceftriaxone IM → Admit to Observation unit or inpatient admit

YES

High risk?3 (see Box 1)

NO

Clinically well & Inpatient: Cx (–) ≥ 48h Observation: Cx (–) @23h

Send home without antibiotics if: • Adequate caregiver education • Reliable follow up in 12–24h • PCP & caregiver agree with plan

YES

Send home if: • Reliable f/u in 12–24h • Adequate caregiver education • PCP & caregiver agree with plan

OR Admit ± empiric antibiotics (Do LP prior to antibiotics)

Box 1 1 Urine testing should be done by sterile bladder catheterization or suprapubic aspiration 3High risk: History: prematurity, perinatal antibiotics, tx for unexplained jaundice, previous hospitalization, chronic illness, not sent home with mother after birth, maternal h/o intrapartum fever, antibiotics, GBS positivity. Physical exam: AOM, soft tissue/bone infection. Lab criteria: WBC 15,000, Band count >1,500/microL, Urine nitrite or LE positive, WBC 5–10/hpf, abnormal CSF (see chapter 20 for normal CSF values). Social: No phone, access to nearby hospital &/or physician for follow-up 2HSV and fever workup & management: History: seizure, ill appearing during 2nd week of life, maternal fever 48h prior to delivery, maternal primary HSV infection during gestation. Physical exam: vesicular rash. Lab criteria: ↑CSF mononuclear pleocytosis, ↑LFT Management: Send HSV oral/skin cultures, HSV PCR from CSF, CSF viral culture and start Acyclovir IV. Base duration of treatment on clinical and culture/PCR results.

Figure 13-1 Algorithm for the management of fever without localizing source in patients 0 to 2 months of age. (Adapted from Texas Children’s Hospital: Evidenced based clinical guideline on fever without localizing signs 0–60 days old).

130

Emergency Medicine

131

Temp ê39°C (102.2°F) in patient 2–36 mo, no source on history/physical

Toxic? (Ill-appearing, lethargy, poor perfusion, hypo/hyperventilation, cyanosis, irritable)

YES

Laboratory workup CBC blood cx, UA with micro & cx, CSF (cell count, Gram stain, glucose, protein) & CSF cx WBC >20,000 or if clinically indicated → CXR

YES

NO

Identified bacterial source for fever: AOM, sinusitis, pneumonia, cellulitis, abscess, osteomyelitis, septic arthritis

Treat source for fever as clinically appropriate

Admit to hospital on antibiotics Vancomycin and cefotaxime ± other indicated antibiotics YES

NO

Viral syndrome? Bronchiolitis, varicella, croup, enterovirus, herpangina, HSV, influenza, stomatitis, viral rashes (URI or AGE don't "count" as a source)

Consider rapid viral testing & in patients with bronchiolitis/influenza do UA with micro and cx if: 1. Female ≤ 2 year, 2. Males circumcised ≤6 mo OR uncircumcised ≤12 mo

NO

YES

Received 3 doses of PCV7

U/A with micro and cx if: 1. Female ≤ 2 year, 2. Males circumcised + ≤6mo or uncircumcised + ≤12mo, 3. History of UTI, GU defect Send home without antibiotics if: tolerating PO, reliable follow up arranged, caregiver education complete (OR monitor patient in Observation unit/inpatient admit)

NO

NO

CBC d/p, draw and hold blood cx, UA with micro and cx

CBC or UA abnormal?

YES

3

• If WBC > 15,000/mm or ANC >10,000 → Send blood cx, ceftriaxone IM, & f/u in ≤24h • If WBC > 20,000/mm3 → CXR → If + treat for pneumonia; if – blood cx, ceftriaxone IM, & f/u in 24h • If + UA → PO antibiotics for UTI ± ceftriaxone x 1 and f/u in ≤ 24h

Figure 13-2 Algorithm for the management of fever without localizing signs in patients 2 to 36 months of age. (Adapted from Texas Children’s Hospital: Evidenced based clinical guideline on fever without localizing signs in 2–36 mo.)

SCROTAL PAIN AND SWELLING COMPARISON OF TESTICULAR TORSION, EPIDIDYMITIS, AND TORSION OF TESTICULAR APPENDAGE Torsion of Testicular Appendage

Testicular Torsion

Epididymitis

Age group

Neonatal, 12–28 yo (66%)

Sexually active adolescents

Onset

Sudden

Gradual

Gradual/sudden

Testicular lie

High

Low

Low

7–14 yo


P E D I AT R I C S

Send home if: tolerating PO, reliable follow up arranged, caregiver education complete (or monitor patient in observation unit/inpatient admit)

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Pediatrics

Testicular Torsion

Epididymitis

Torsion of Testicular Appendage

Cremasteric reflex

Usually not present

Present

Present

Prehn’s sign∗

Negative

Positive

Variable

Urinalysis

Normal

+WBCs on UA

Normal

Presentation

Acute onset of pain, nausea/vomiting, purple, swollen, painful testes

Fever, chills, urethral discharge, nausea, neonatal, 12–28 yo (66%), edematous and tender epididymis

Pain located in the superior pole of testicle; mild erythema or edema; blue dot sign (21%)

Doppler ultrasonography

↓ or absent flow

Normal or ↑ flow in epididymis

Torsed appendage with ↓ flow but normal flow to testes

Treatment

Emergency surgery: Bilateral fixation of testes (irreversible damage possible in 5–6 h) Manual detorsion (open book technique) if timely surgical intervention not available

Scrotal support (briefs), NSAIDs Antibiotics† • Prepubertal: Most commonly no antibiotics required; may consider Trimethoprim– sulfamethoxazole • Sexually active: Ceftriaxone + azithromycin/ doxycycline

Scrotal support (briefs), NSAID Resolves within 2–12 days

∗Relief of pain with elevation of testicle. † Etiologic agents include coliform bacteria, viruses in prepubertal boys and Neisseria gonorrhea, Chlamydia in adolescents.

LIMPING AND JOINT PAIN • Definition: Uneven alteration in natural gait (secondary to pain, weakness, deformity). • Etiology: Trauma is the most common cause of limp. See the table below for causes of limp when there is no history of trauma.

CAUSES OF NON-TRAUMATIC LIMP/JOINT PAIN IN CHILDREN % of nontraumatic causes Fracture

2%

Overuse (soft tissue injury, muscular strain, Osgood-Schlatter’s disese, hematoma of the thigh)

18%

Osteomyelitis Other (appendicitis, PID, torsion constipation, etc.)

2%

Transient synovitis

40%

Legg-Calve-Perthes syndrome (avascular necrosis of the femoral head)

2% (continued on next page)

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133

% of nontraumatic causes Inflammation (rheumatologic: JRA, SLE, reactive arthritis or HSP) Infection (skin, soft tissue, joint, septic arthritis)

3% 4%

Foreign body Tumor “Toddler’s fracture” (nondisplaced spiral fracture of the tibial shaft) Trauma

1% 1% —

SCFE Sickle cell pain crisis

101.3°F) ; refusal to bear weight*; warm red, swollen joint; ↓ ROM (may be rigid); if septic hip (hip flexed and abducted); most sxs ~3 days duration; recent URI (53%) trauma (31%)

Recent URI, low grade to normal temperature, allows passive ROM, can bear some weight

Laboratory studies

CBC∗ (↑ WBC, usually >12,000/mm3), ↑ ESR∗ (usually >40), ↑ CRP (>2)

None required; CBC usually normal, ESR or CRP mild elevation

Radiologic studies

US of joint (85% show effusion), MRI ⊕ in 88%, radiography (20% reveal joint space widening)

U/S or Ultrasound, MRI not needed unless need to help distinguish from septic arthritis. Consider X-rays to rule out femoral neck fractures.

Etiology

Staphylococcus aureus (MRSA) > Kingella > Streptococcus pneumoniae > Salmonella; consider GBS in neonates

Postinfectious reactive fluid in joint (joint fluid cx results negative)

Treatment

Orthopedic emergency (needs immediate drainage), joint aspiration (send for WBC, protein, cx), blood cx Antibiotics: MRSA coverage (eg, vancomycin) + GNR coverage (eg, cefotaxime/ gentamicin) ± MSSA, strep coverage (eg, nafcillin) X-rays → modify based on cx results; usual duration, 3–4 wk total

NSAIDS scheduled until symptomatically improved

∗Positive predictive value of septic arthritis using findings in table above denoted by an asterisk: one sign, 3%; two signs, 40%; three signs, 93%; and four signs, 99.6% (J Bone Joint Surg Am 1999;81:1662). In addition, CRP >2 ↑ likelihood of septic arthritis.

BITES • Treatment: • Irrigation is very important for infection prevention (100 mL NS, sterile water, or tap water per cm laceration) • Debride devitalized tissue • If wound appears infected, culture but do not close

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135

• First-degree closure should be avoided in puncture wounds, wounds on hands and feet, and wounds through cartilage • Antibiotic prophylaxis is not routinely recommended except in special cases (see table below) • Tetanus vaccine status should be verified for all patients and prophylaxis provided as indicated. • Rabies prophylaxis: • Consider when exposure to saliva is sufficient for infection (bite not required); saliva exposure to bats [most common cause in United States], raccoons, foxes, skunks (uncommon in small rodents (squirrels, rats) and rabbits). • Treatment • No suspicion → no treatment. • Any suspicion → attempt to quarantine and observe animal and discuss with local health department. • High suspicion or animal not in custody → one-time dose of human rabies immunoglobulin (20 IU/kg, with half given IM and half infiltrated locally at the wound site if feasible) followed by 4 IM injections of human diploid cell vaccine (1 mL) to be given on days 0, 3, 7, and 14 (CDC: ACIP Provisional Recommendations for the Prevention of Human Rabies: http://www.cdc.gov/mmwr/pdf/rr/rr5902.pdf or MMWR 2010;59(02):1

CHARACTERISTICS AND TREATMENT RECOMMENDATIONS FOR CAT, DOG, AND HUMAN BITES Cats

Dogs

Humans 2–3%

Percentage

5–10%

80–90%

Appearance

Puncture wounds

Tears and punctures

Moon shaped

Suture?

Do not close punctures

Can close if 7 reflects inappropriate renal loss of K; 3 mmol/L rarely cause significant problems in otherwise healthy individuals; levels 6 mEq/100 mL must be given via CVL

HYPERNATREMIA (NEJM 2000;342:1493) • Serum Na is a reflection of TBW more than total body sodium → volume status determination is critical to the evaluation and treatment of sodium disturbances.

Signs and Symptoms • Tachypnea, confusion, muscle weakness or cramping, lethargy, coma, seizures, depressed deep tendon reflexes

Fluids, Electrolytes, and Acid–Base Balance

161

Hypernatremia Hypovolemic

Hypovolemic hypernatremia ↓UOSM UNa>20

Hypervolemic

Euvolemic hypernatremia

↑UOSM UNa< 20

Renal Losses • ATN, diuretic phase • Osmotic diuresis • Postobstructive diuresis

Euvolemic

↓UOSM

Extrarenal • Diabetes Losses insipidus • Diarrhea • Increased insensible losses • Incorrectly mixed formula (diluted)

↑UOSM

Hypervolemic hypernatremia ↑FENa

• Transient • Exogenous Na hypernatremia† administration

nl FENa

• Mineralocorticoid excess • Hyperaldosteronism

Figure 15-1 Evaluation of hypernatremia. † Intracellular osmole generation → osmotic water shift → transient ↑ serum Na, as may be seen after vigorous exercise, seizures, etc.

Workup • History of recent vigorous exercise, trauma, dietary intake, polyuria, renal or CNS insult. • Volume status: Vital signs, orthostatic hypotension, dry or wet mucous membranes, peripheral edema, BUN/serum Cr ratio (>20 indicative of hypovolemia). • Labs: Guided by volume status; see Figure 15-1. • Diagnosis of diabetes insipidus: See Chapter 14 or Ann Intern Med 2006;144(3):186..

Treatment

Method 1 (Calculation Method) 1. Calculate the free water deficit. TBW × [(Serum Na – 145)/145] TBW (L) = [Baseline weight in kg × Age-appropriate constant] (0.8 for premature infants; 0.6 for infants, children, and adult males; 0.5 for adult females). • Divide the deficit by 2 or 3 to correct hypernatremia slowly over 2–3 d to avoid rapid correction and possibility of inducing cerebral edema from rapid water shift from extracellular to intracellular space. 2. Calculate MIVF fluid rate for 24 hour period. 3. Calculate maintenance Na needs for 24 hr period (2-3 mEq/kg/d) 4. Combine values in steps 1-3 to determine daily IVF fluid type, volume and rate: Concentration of Na (mEq/L) in D5W = [Maintenance Na]/[1/3 or ½ of free water deficit volume + Daily maintenance fluid volume] • Round to standard IV fluid solution if possible (see table above, “Composition of Commonly Used IV Fluids”)

P E D I AT R I C S

• Hypervolemic hypernatremia: Correct underlying cause • Euvolemic hypernatremia: Correct underlying cause • Hypovolemic hypernatremia (dehydration): Results from relative loss of free water compared with Na (rarely Na excess); two methods of correction (described in the following sections).

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Pediatrics

Example: (22 kg child, serum Na 165 mEq/L, 10% dehydration = 2.2 L total fluid deficit)

Deficit

Fluid

Na

Free water deficit∗

1800 mL (free water)

0 mEq

Remaining deficit†

400 mL (isotonic saline)

56 mEq (= 140 mEq/L × 0.4 L)

Maintenance per 24 h

1540 mL

55 mEq (2–3 mEq/kg/day)

∗based on free water deficit formula above. † based on estimation of percent dehydration (see beginning of chapter)—free water deficit.

Total Fluid: ½ free water deficit (900 mL) + remaining deficit (400 mL) + maintenance fluid (1540 mL) = Total fluid 2840 mL/day = 118 mL/h Total Sodium: Na in isotonic remaining deficit replacement (56 mEq) + maintenance Na (~55 mEq) = Total (111 mEq/day) IVF Na concentration: 111 mEq Na/2840 mL = 39 mEq Na/1000 cc Final fluid and rate = D5W + 39 mEq Na/L @ 118 cc/h • Hypocalcemia and rarely tetany may occur with correction of hypernatremia and acidosis; monitor blood ionized Ca and add Ca to IVF if necessary • Hyperglycemia is common in patients with hypernatremia. • Monitor serum Na, ionized Ca, glucose, and urine output closely during rehydration. • Serum Na >180 mEq/L requires slow careful correction and rarely dialysis. Dialysis is usually indicated in the presence of renal failure (elevated serum Cr) and poor urine output. • Under no circumstances should serum Na be allowed to decrease at a rate >10–15 mEq/L per 24-h period (~0.5 mEq/h) because of the risk of cerebral edema) Method 2 (Estimation Method) • Based on estimation of expected change in serum Na given 1 L of fluid with a specified concentration of Na. (Intensive Care Med 1997;23:309) [Na]serum per L infused = [ [Na]infusion – [Na]serum ] / [ TBW + 1] • Example (30-kg child, serum Na 160, using D5½NS): Δ Naserum = [77 – 160]/[(0.6 × 30 kg) + 1] = –4.4 mEq serum sodium change after administration of 1 L of fluids • Calculate expected change based on different fluid concentrations (see table below) used for rehydration (must take into account total daily fluid to be delivered; do not lower serum Na >10–15 mEq/L per 24-h period).

HYPONATREMIA (NEJM 2000;342:1581) • Serum Na is a reflection of TBW more than total body sodium caused by free movement of water across cell membranes. Volume status determination is critical to the evaluation and treatment of patients with sodium disturbances. • Symptoms and signs: Headache, nausea, vomiting, muscle cramps, lethargy, restlessness, disorientation, depressed reflexes, seizure, coma, permanent encephalopathy, respiratory arrest, brainstem herniation.

Causes and Workup (Figure 15-2) Treatment • Asymptomatic hyponatremia: See below for treatment based on volume status; rate of correction ≤12 mEq/d (≤ 0.5 mEq/L/h). • Symptomatic hyponatremia: Initial rapid correction of Na to resolve symptomatology (eg, 3 mL/kg 3% NS over 30 minutes to stop seizures, usually accomplished after Na is approximately 120 mEq/L); then continue correct at a rate not to exceed 12 mEq/L/d. • Whether symptomatic or asymptomatic, monitor Na very closely during replacement (eg, Q2–4h) and monitor closely for signs of CNS demyelinization (central pontine myelinolysis): Spasticity, pseudobulbar palsy. Hypervolemic Hyponatremia • Free water restriction, diuretics, increase effective circulatory volume (vasodilators in CHF, colloids in hypoalbuminemic state)

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163

Hyponatremia Hypertonic

Transcellular H2O Shift* (effective osmoles) • Hyperglycemia • Mannitol

Hypotonic

Lab Artifact† (ineffective osmoles) • Hypertriglyceridemia (>1000 mg/dL) • Hyperproteinemia (>10g/dL) • Glycine/sorbitol bladder irrigation (GU procedures)

Hypotonic hyponatremia (true hyponatremia)

Hypovolemic

Hypovolemic hyponatremia FENa>1–2% UNa>20

Isotonic

Hypervolemic

Euvolemic

Hypervolemic hyponatremia

Euvolemic hyponatremia

FENa1–2% UNa>20

FENa 7.45 alkalosis

Step 2

pCO2 7.45

7.40, and a primary alkalosis will never be compensated to a pH 20 or HCO3 5 is significant) • Δ HCO3 ≈ Δ AG → simple AG metabolic acidosis • Δ HCO3 > Δ AG → AG metabolic acidosis with superimposed metabolic alkalosis • Δ HCO3 < Δ AG → AG metabolic acidosis w/ superimposed non anion gap metabolic acidosis Step 4: Perform additional testing (depending on the type of disorder) and generate a differential diagnosis for the disturbance. See “General approach to acid-base disor-

ders” algorithm.

Available at www.AccessPediatrics.com • Hypercalcemia • Hypocalcemia

Gastroenterology GASTROESOPHAGEAL REFLUX

169

P E D I AT R I C S

• Definitions: Gastroesophageal reflux (GER) is normal passage of gastric contents into the esophagus; GERD consists of symptoms and complications of GER; rumination is voluntary habitual regurgitation of gastric contents into the mouth for self-stimulation. • Complications of GERD can be divided into three systems: Respiratory (asthma and / chronic cough, apnea and ALTE, recurrent aspiration pneumonia), ENT (hoarseness, laryngitis, sinusitis, dental erosions, recurrent OM), and GI (esophagitis, esophageal ulcers, esophageal strictures, Barrett’s esophagus). • Prevalence of GER: 50% in infants age 0–3 mo; 67% in children age 4 mo; 5% of children age 10–12 mo; 1.4–8.2% in children age 3–17 yr (Arch Pediatr Adolesc Med 1997;151:569). The prevalence of GERD is unknown. • Diagnosis • History and physical exam: A thorough H&P is sufficient for diagnosis. The clinical presentation is based on the age of the child: • Infants and young children present with recurrent vomiting, arching of the back during feeds (Sandifer syndrome), irritability, and poor weight gain (2° to vomiting or dysphagia). Some present with wheezing or chronic cough, recurrent pneumonia, upper airway symptoms (eg, recurrent stridor), apnea, or ALTE. • Older children and adolescents present with heartburn or retrosternal chest pain and regurgitation. Some present with dysphagia, hoarseness of the voice, weight loss, anemia, wheezing or chronic cough, recurrent pneumonia, food impaction, and Barrett’s esophagus (rare). • History: Ask for details of meals (type, volume and frequency), recent change in appetite, reflux symptoms after feeding (regurgitation, pain, irritable, dyspepsia), presence of blood or bile, force of reflux, blood in the stool, trends in weight gain. Social history: Tobacco or alcohol use, psychological factors (eg, stressors, anxiety, depression). PMH: of eczema, neurologic issues (↑ or ↓ tone, hydrocephalus, presence of shunt), prematurity, surgery, ENT disease. Family history: Helicobacter pylori infection, reflux, atopic disease. • Signs suggesting non-GER cause of vomiting: Bilious or forceful vomiting, GI bleeding, diarrhea, constipation, abdominal pain or distension, fever, lethargy, HSM, seizures, microor macrocephaly, FTT, genetic d/o (trisomy 21), other chronic diseases (eg, HIV). • Laboratory studies/evaluation: None are required for the diagnosis of GERD. Consider H. pylori stool antigen testing for children and adolescents if the patient c/o abdominal pain ± dyspepsia or vomiting. In unclear cases, consider UGI to r/o anatomical abnormalities, esophageal pH monitoring or multichannel intraluminal impedance may be used to correlate reflux with symptoms; esophagoscopy may be used for complications (strictures, esophagitis) and to rule out anatomic abnormalities. • Treatment • Physiological GER: Reassurance • GERD in infants: • Step 1: Thicken formula or breast milk with rice cereal (add 1 Tbsp/oz of formula), reflux precautions (ie, sleeping supine at a 30- to 45-degree angle, small frequent feeds, frequent burping, maintain upright position for 30 min after feeding). • Step 2: If step 1 interventions fail, add pharmacologic therapy: H2R antagonist (eg, ranitidine) or PPI (eg, lansoprazole; not currently FDA approved). PPIs are more effective at acid suppression and are most effective if given 30 min before morning feeds. • Surface agents (eg, sucralfate, sodium alginate) help protect ulcerated gastric mucosa. Consider use in infants with esophagitis or ulcers. • Consider changing to casein hydrolysate formula (ie, Nutramigen, Alimentum, Pregestemil) if milk-protein allergy is strongly suspected as the cause of GER.


C H A P T E R 16

170

Pediatrics

• Prokinetic agents are (eg, metoclopramide, bethanechol, erythromycin) generally not helpful. However, in severe cases a trial of bethanechol may be useful. • Surgical intervention: Nissen fundoplication is indicated in patients who failed or cannot be weaned off medical therapy and those with frequent aspiration related issues. • Children and adolescents with GERD: • Lifestyle changes: Sleep on the left side with the head of the bed elevated; avoid caffeine, chocolate, spicy food, tobacco exposure, alcohol; recommend weight loss for obese children; ± psychological evaluation and support; ± intermittent antacids (eg, magnesium hydroxide, aluminum hydroxide, calcium carbonate). • If above interventions fail, provide a trial of H2RA or PPI. • Surface agents (eg, sucralfate, sodium alginate) help protect ulcerated gastric mucosa. Consider use in children with esophagitis or ulcers.

HEPATITIS Infectious Hepatitis • Most often caused by one of five viruses: Hepatitis A (~30%), B (~50%), C (~20%), D, and E. Other viruses reported to cause hepatitis as part of generalized infection include CMV, HSV, VZV, EBV, rubella, coxsackievirus, and adenovirus.

SEROLOGY AND NUCLEIC ACID TESTING PROFILE FOR HEPATITIS A, B, AND C VIRUSES Scenario

ALT

Acute HAV∗

↑

HBc HBc HCV HCV HBsAg IgM IgG HBeAg HBeAb HBsAb HBVDNA Ab RNA

Prior HAV or Normal vaccine∗ Acute HBV

↑

+

+

–

–

–

–

+

Acute HBV “window period”

↑

–

+

–

–

–

–

±

Chronic HBV, active replication

↑

+

–

+

+

–

–

+

Chronic HBV, “pre core” mutant

↑

+

–

+

–

–

–

+

Chronic HBV, Normal carrier

+

–

+

–

+

–

–

Prior HBV

Normal

–

–

+

–

–

±

–

Prior HBV vaccine

Normal

–

–

–

–

–

+

–

Acute HCV

↑

–

+

Chronic HCV Normal or ↑

+

+

False + Normal HCV or prior infection with eradication

+

–

∗Acute HAV: ALT↑, HAV IgM ¢, HAV IgG ; prior HAV or vaccinated: ALT is normal, HAV IgM Adapted from: Tarascon Internal Medicine and Critical Care Pocketbook, 4th ed. 2009:68.

, HAV IgG ¢.

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Noninfectious Hepatitis NONINFECTIOUS HEPATITIS: CAUSES, DIAGNOSIS, AND TREATMENT Cause

Diagnosis

Treatment

Comments

Hereditary hemochromatosis (HH)

↑ Transferrin saturation, ferritin, genetic testing, liver biopsy

Phlebotomy to keep ferritin 250 mcg/g dry weight); genetic testing; Kayser-Fleischer rings; neurologic d/o (35%)

Chelation: Penicillamine (give pyridoxine); trientine is second line; give 1 h before or 2 h after meals; add PO zinc in maintenance therapy after chelation; liver transplantation for those with fulminant hepatic failure

1 in 30,000 births, AR defect in hepatocellular copper transport

`-1-antitrypsin ↓ serum AAT (1 wk, INR >4, metabolic acidosis with elevated creatinine, factor VII 15–20 mg/dL, ↑ PT/PTT/INR, ↓ Factors V/VII

Fulminant Liver Failure INR >1.5 + encephalopathy or INR >2 ± encephalopathy

Start Investigation for Liver Failure Labs: CBC, ABG, Chem 10, ammonia, fibrinogen, ESR, lipid profile, UA with cx, urine Iytes, osmolality Metabolic screen: SAA; lactate; pyruvate, acylcarnitine profile; AFP; UA and urine organic acids reducing substances, ketones Autoimmune hepatitis screen: Immunoglobulins, ANA, anti-SM antibody, anti-LKM1 antibody Other screen: AAT, copper level, ceruloplasmin, ferritin, iron panel Viral tests: Hepatitis panel, EBV PCR, CMV antigenemia, adenovirus PCR and rapid screen, enterovirus PCR, parvovirus B19 titers Drug screen: Acetaminophen, aminosalicylic acid, urine/blood drug screen Radiology: Abdominal US with Doppler; head CT Other studies: EEG Biopsy: Transjugular liver biopsy + venogram (send tissue for virology, electron microscopy, histology, antibody staining, enzyme assay and copper/iron stains)

Management 1. ABC 2. Supportive care: a. Manage bleeding: FFP, cryoprecipitate, PRBC, Vitamin K, IV PPI b. Manage hypoglycemia (glucose IV) or electrolyte imbalance if present; plan for early nutrition (eg, TPN) c. Manage encephalopathy: Airway management, cerebral edema (elevate head of bed, hyperventilation, mannitol); no sedation, acetaminophen or NSAIDs; hyperammonemia (low-protein diet, Iactulose) d. Manage infections sepsis, spontaneous bacterial peritonitis e. IV N-acetylcysteine may benefit patients with non-acetoaminophen liver failure (Gastroenterology 2009;137(3):856) 3. Admit to ICU 4. Consult GI or Liver team Figure 16-1 Diagnosis, workup, and management guidelines for fulminant liver failure.

END-STAGE LIVER DISEASE (ESLD) • Presentation • Children with ESLD may present well compensated or with acute-on-chronic liver failure (see Figure 16.1). • FTT, abdominal distension, HSM, and jaundice are common signs of ESLD in children. Less commonly, digital clubbing, spider angiomata, palmar erythema, Dupuytren’s contractures, gynecomastia, and testicular atrophy are observed. • Etiology: See noninfectious and infectious causes of hepatitis section. Also see hepatomegaly section. • Diagnostic studies: See fulminant liver failure (Figure 16.1). In general, ↓ Na, ↑ ammonia, ↓ albumin, ↑ bilirubin, ↑ PT/INR, ↓ platelets. AST, ALT, and Alk Phos levels are usually elevated but may fall late in the course of disease. • Treatment: Management of complications (listed below), correction of underlying cause if possible, liver transplantation when indicated. • Complications • Portal HTN • Pathogenesis: ↑ Portal resistance, ↑ portal blood flow, ↑ cardiac output, ↓ systemic vascular resistance. • Management: Follow physical exam for signs of worsening liver failure and development of ascites or varices; assess for GI bleeding (CBC, hemoccult). Consider serial abdominal US with Doppler.

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• Hepatic Encephalopathy • Reversible neurologic abnormalities seen in patients with cirrhosis (see table below). Proposed etiologies include neurotoxicity of ammonia and its metabolites and ↑CNS GABA activity. • May be precipitated by various events, including infection, dehydration, constipation, or medication noncompliance.

STAGES OF HEPATIC ENCEPHALOPATHY Indicator

0

I

II

III

IV

Level of consciousness

Normal

Confused, irritable, loss of spatial orientation

Drowsy, responds to simple commands

Stuporous but arousable, marked confusion

Comatose with or without response to pain

Reflexes

Normal

Normal

Hyperreflexic

Hyperreflexic + Babinski sign

Areflexic

EEG

Normal

Minimal changes

Slow rhythm with triphasic waves

Grossly abnormal

Grossly abnormal with delta waves

Altered sleep habits

Dysarthria, incontinence, asterixis

Marked dysarthria, rigidity

Decerebrate or decorticate posturing

Other

DIAGNOSTIC APPROACH TO RENAL INSUFFICIENCY IN END-STAGE LIVER DISEASE

Clinical

Prerenal

Hepatorenal

Signs of hypovolemia; resolves with volume expansion

No hypovolemia and no response to volume expansion; hypothermia; usually accompanied by ascites and hepatic encephalopathy

ATN


P E D I AT R I C S

• Varices • Pathogenesis: ↑ Portal pressure (>12 mm Hg) causes development of portosystemic collaterals in the stomach and esophagus. Variceal wall tension, diameter, and pressure gradient contribute to the risk of bleeding. • Prevention of bleeding: Propranolol – will ↓ HR, ↓ cardiac output, ↓ portal inflow, and blocks vasodilatation. See GI bleed section for treatment of acute variceal bleeding. • Hepatorenal Syndrome (Lancet 2003;362(9398):1819) • Definition: Cirrhosis or acute liver failure with CrCl 30 >1.1 Normal Proportional ↑ in BUN and Cr

95% accuracy that ascites is portal HTN related (Ann Int Med 1992;117:215). If SAAG ≥1.1 g/dL then concentration of ascites fluid TP (AFTP) helps distinguish between cirrohsis (AFTP 2.5 g/dL) as etiology for ascites. (SAAG = albumin concentration of serum - albumin concentration of ascitic fluid) • Treatment: Treat underlying cause for ascites. Monitor serum and urine lytes, fluid status, daily wt, strict I/Os, low-sodium diet • Diuretics: Spironolactone (for children, 1–3 mg/kg/day divided BID with a maximum of 5–6 mg/kg/day; for adolescents and adults, 100–200 mg/day divided BID with a maximum of 600 mg/day; assess response with spot urine Na (>20 mEq/L considered appropriate naturesis). • If unresponsive to diuretics, consider fluid restriction and albumin infusion followed by furosemide • Large-volume paracentesis is considered for ascites causing respiratory compromise or for refractory cases to treatment. • Complications: Spontaneous bacterial peritonitis, pleural effusions, hepatorenal syndrome. • Bacterial Peritonitis • Definition: Acute infection of the peritoneal cavity. • Presentation: Subtle signs and symptoms: fever, anorexia, vomiting, abdominal tenderness, rigidity, ↓ bowel sounds, fluid wave. • Diagnostic studies: Paracentesis with fluid analysis for cell count, LDH, TP, glucose, Gram stain, and culture. • Management: See table below. Antibiotics, fluid resuscitation, correction of lytes, NG tube placement. Patients with secondary bacterial peritonitis have a intra-abdominal focus of infection (eg, ruptured viscus, abscess) that may warrant surgical intervention.

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175

DIFFERENTIATION AND TREATMENT OF BACTERIAL PERITONITIS Type

Risk Factors

Cell Count

Culture

Treatment

Primary

Ascites: Renal or liver disease (20% of patients with cirrhosis), CHF

PMN count ≥250 cells/mm3 TP 1 g/dL or Glucose upper limit of normal for serum

Polymicrobial

Immediately start empiric 3rd generation cephalosporin (eg, cefotaxime). Modify antibiotic choice based on culture.

Secondary Intraabdominal infection

HYPERBILIRUBINEMIA Direct (Conjugated)

Indirect (Unconjugated) • Definition: Elevation of unconjugated bilirubin, above age-adjusted normal. Majority are caused by hematologic issues. May be caused by bilirubin overproduction (hemolysis), impaired hepatic uptake (drugs, impaired hepatic blood flow eg, CHF), or rarely impaired conjugation (Gilbert syndrome, Crigler-Najjar syndrome, hyperthyroidism, drugs, chronic liver disease). • Diagnosis • History: Age, fever, diet history, jaundice, race or ethnic origin, exposure to medicine, blood products. PMH/FHx of hematologic abnormalities (hemolytic anemia, sickle cell disease, thalassemia), Gilbert or Crigler-Najjar syndrome.

P E D I AT R I C S

• Definition: Conjugated bilirubin >2mg/dL or >20% of total bilirubin. Due to differences in technical methodology, direct bilirubin will be higher than conjugated bilirubin at any given level of hyperbilirubinemia. • Diagnosis: • History: Age, vomiting, diet history (poor feeding), irritability, stool consistency and color (acholic), family history (consanguinity), school or social activity, birth and perinatal history, medications, and recent travel. • Physical exam: Well versus ill appearing and irritable versus drowsy (encephalopathy, infection, metabolic disorder), dysmorphism, eye exam (Kayser-Fleischer rings or posterior embryotoxin), systolic murmur of PPS (suggests Alagille syndrome), hepatomegaly is typically present (small liver may indicate cirrhosis or ESLD), splenomegaly, ascites (suggests portal HTN and CLD), dark urine, ataxia, and asterixis. • Laboratory studies/evaluation: • Fractionation of bilirubin, liver panel (ALT and AST ↑ in hepatocellular injury, GGT ± Alk Phos ↑ in obstructive conditions), PT/PTT/INR, chem 10, UA and cx for UTI. • Abdominal, US (gallstones, choledochal cyst, ascites), Doppler US (portal HTN, portal vein thrombosis). • HIDA scan may help distinguish obstructive versus non-obstructive causes. • ERCP, MRCP, percutaneous transhepatic cholangiography (obstruction proximal to common hepatic duct or ERCP precluded). • Liver biopsy may be required for definitive diagnosis. • Management/Treatment: Treat based on etiology in consultation with GI service.

176

Pediatrics

• Physical exam: General appearance (ill-appearing patients should be promptly evaluated for sepsis), icterus (if no scleral icterus, consider carotenemia), large areas of bruising or hematoma formation, splenomegaly (consider hypersplenic state); usually no hepatomegaly. • Laboratory studies/evaluation: CBC, reticulocyte count, Coombs (direct and indirect), peripheral smear, liver panel (usually AST and ALT are normal with ↑ indirect bilirubin). Consider G6PD assay if appropriate risk factors (Mediterranean, African descent, males + family history). • Management/Treatment: Treat based on etiology in consultation with GI service.

PANCREATITIS • Definition • Acute: Inflammatory process resulting from ectopic activation of pancreatic enzymes characterized by abdominal pain, ↑ amylase and lipase, and supportive radiologic findings (see table below). • Chronic: Permanent structural changes of the pancreas (eg, calcification, fibrosis) or its duct (eg, stricture, dilatation); may lead to exocrine or endocrine insufficiency. • Epidemiology: Generally seen in children and adolescents; boys and girls are equally affected. Most cases of acute and chronic pancreatitis are idiopathic. Trauma is the most common identifiable etiology of acute pancreatitis. CF and other genetic mutations are the most commonly identified etiologies of chronic pancreatitis.

COMPARISON OF ACUTE AND CHRONIC PANCREATITIS IN CHILDREN Acute

Chronic

Etiology

• Idiopathic (23%) • Traumatic (22%) seat belt, bicycle handlebars, NAT • Anatomic or obstructive (15%) pancreas divisum (controversial), anomalous pancreaticobiliary union, choledochal cyst, cholelithiasis, sphincter of Oddi dysfunction, masses • Systemic disease (14%) HUS, Kawasaki, HSP, Crohn disease, SLE • Toxins and drugs (12%) azathioprine, 6-MP, L-asparaginase, valproic acid, corticosteroids, some antibiotics and diuretics, scorpion venom, organophosphate insecticides, cocaine, ethanol • Infection (10%): Usually viral; rarely bacterial, parasitic (eg, Ascaris) • Metabolic (2%) hypertriglyceridemia, hypercalcemia, refeeding syndrome, DM • Familial (2%) (J Clin Gastroenterol 2003;37(2):169) • Miscellaneous: Post-ERCP, malignancy

• Idiopathic • Genetic mutations: Serine protease 1 gene (PRSS1) Serine protease inhibitor Kazal type 1 (SPINK1) CFTR (± clinical manifestations of CF) • Anatomic or obstructive: See list under “acute” • Autoimmune: Secondary forms (eg, SLE) more common in children than primary autoimmune pancreatitis • Tropical calcific pancreatitis

Pathophysiology

Premature activation of pancreatic enzymes in the acinar cell leads to parenchymal inflammation, ischemia, and necrosis

Repeated episodes of acute pancreatitis Protein plugs or calculi causing obstruction and secondary calcification Autoimmune vasculitis (continued on next page)

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177

Chronic

Abdominal pain: Epigastric classically, may radiate to back Nausea and vomiting ± intestinal ileus Anorexia Jaundice (with gallstones or biliary obstruction) Fever, RUQ pain, and jaundice (Charcot’s triad) indicative of cholangitis

Flares of chronic pancreatitis mimic acute pancreatitis Pancreatic exocrine or endocrine insufficiency (late signs) Steatorrhea Excessive appetite Growth failure

Physical exam

Tachycardia, pain, “third spacing” Abdomen: Inspect for signs of trauma, tenderness ± distension, ascites rarely, peritoneal signs, pseudocysts may be palpable, diminished bowel sounds Hemorrhagic necrosis (bluish discoloration): Flanks—Grey Turner’s sign; umbilicus—Cullen’s sign

May be normal Chronic abdominal tenderness may dominate the clinical picture late in the course of disease

Diagnosis

Physical exam findings of epigastric pain along with history of vomiting (afebrile) Serum amylase and lipase >3 times normal (no correlation to disease severity) Secondary hyperglycemia, hypocalcemia, hypoalbuminemia, hypertriglyceridemia, and acidosis may be observed LFTs and bilirubin elevated in gallstone pancreatitis Triglyceride level >1000 mg/dL after the acute episode is suggestive of hypertriglyceridemic pancreatitis Abdominal US, CT if necessary to assess for obstructive causes, pseudocysts, abscesses MRCP (after the acute episode): When an anatomical lesion is suspected

May present as acute pancreatitis; however, amylase and lipase may not be increased with progressive parenchymal destruction Spot fecal fat (increased) Fecal elastase-1 (low) Low vitamins A, D, E, K (rarely) KUB: Helpful when calcific pancreatitis is suspected Abdominal US, CT, MRCP by definition should reflect parenchymal or ductal changes Endoscopic US: Helpful in select cases when noninvasive imaging is equivocal ERCP offers diagnostic and therapeutic abilities (stent placement, sphincterotomy, stone removal) Genetic testing: PRSS1, SPINK1, CFTR (Note: Many CFTR mutation screening panels are “pulmonary centric” and will miss some CFTR mutations associated with pancreatitis but not the pulmonary phenotype) Sweat chloride test (continued on next page)

P E D I AT R I C S

Acute Clinical manifestations

178

Pediatrics Acute

Chronic

Management

• NPO, NG decompression if vomiting; gastric acid suppression until feeding • Fluid resuscitation and electrolyte correction • Nutrition: Jejunal feeds are superior to TPN (↓ complications and cost) when severity of illness is expected to preclude PO feeding for several days (Gastroenterol Clin North Am 2007; 36(1):65). Initiate low fat diet when abdominal pain improves and vomiting resolves • Analgesia: NSAIDs (eg, ketorolac), narcotics; insufficient evidence exists to routinely recommend meperidine over morphine (Am J Gastroenterol. 2001;96(4):1266) • Antibiotics: For severe systemic illness or necrosis (cover aerobic and anaerobic enteric organisms) • ERCP: Only indicated acutely for impacted stone removal, sphincterotomy • Surgical management is reserved for some traumatic injuries, necrotic pancreatitis, recalcitrant pseudocysts

• Pain: • Small, low-fat meals; vitamin (especially fat-soluble) supplementation • Pancreatic enzyme supplements (enteric coated): Restore exocrine enzyme deficiencies and may reduce pain by minimizing CCKmediated secretory drive • Chronic opioids for persistent, significant pain; long-acting agents are more effective • ERCP with sphincterotomy ± stent placement • Surgery

Complications

Shock and multisystem organ failure (rare in children) Pancreatic necrosis, abscess, or pseudocyst formation

Bile duct and duodenal obstruction (5%-10%): Decompression may reverse secondary biliary fibrosis Pseudocysts (~10%): May drain for rapid enlargement, compression of surrounding structures, pain, or infection Splenic vein thrombosis Sequelae of chronic nutrient malabsorption

CONSTIPATION • Definition: Delayed or difficult defecation for >2 wk. • Etiology • Functional: Most cases are functional in older children (ie, withholding of stool that leads to progressive impaction, painful defecation and reinforcement of withholding behavior). • Anatomic: Hirschsprung disease, anal stenosis, malpositioned anus, bowel atresia or stricture, abdominal mass. • Physiologic: Medications (iron, diuretics, anticholinergics, narcotics), milk-protein allergy (infants), CF, neuromuscular disease (CP, MD, hypotonia), spinal cord lesion, hypothyroidism, ↑ Ca++, ↓ K+, botulism, lead poisoning, malnutrition, anorexia nervosa.

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179

MALABSORPTIVE SYNDROMES • Definition: Generalized inability to absorb fat, protein, ± carbohydrates. • Etiology: Any condition resulting in ↓ surface area for absorption, impaired motility or mixing, bacterial overgrowth, decreased pancreatic exocrine function, ↓ bile acid production or secretion, or impaired lymphatic or venous drainage (including inherited diseases). • Presentation: Diarrhea, vomiting, anorexia, abdominal pain, FTT, abdominal distension. • Diagnostic studies • Stool: pH, reducing substances, color, occult blood, microscopy, O&P, culture, fecal fat, lytes, α1-antitrypsin. • Blood: CBC, ESR, LFTs, lytes, PT/PTT/INR, peripheral smear, immunoglobulins (more specific tests as indicated), intestinal biopsy.

INFLAMMATORY BOWEL DISEASE • Definitions • Ulcerative colitis: Chronic, idiopathic superficial inflammation of colonic mucosa starting in the rectum and extending proximally but sparing the upper GI tract. May involve entire colon and the terminal ileum (backwash ileitis) in some patients. • Crohn disease: Chronic, idiopathic transmural inflammation of any part of the GI tract (from mouth to anus), usually occurring in a noncontiguous fashion (skip lesions) and preferentially affecting terminal ileum and colon.

P E D I AT R I C S

• Diagnosis • History: Stool frequency, consistency (hard, soft, diarrheal) and size; blood mixed with or coating stool; abdominal pain; pain with defecation; abdominal distension; time after birth for first bowel movement; urinary incontinence or increased frequency (including enuresis); encopresis; retentive posturing; diet history (especially milk intake, fluid intake); FTT; emesis; fever. Assess for physical, sexual, or emotional abuse; stress; medication use; family history of GI diseases. • Physical Exam • Abdomen: Usually generalized abdominal pain, ± distension, ± palpable stool. No peritoneal signs. Distension, rebound, rigidity (obstruction), palpable stool. • Rectal: Tone, position, presence of anal wink, impacted stool in vault, fissures, perianal abscess, dermatitis to suggest soiling. • Full neurologic exam and back exam (to evaluate for spinal cord lesions, botulism, hypothyroidism); goiter, large tongue, myxedema, skin dryness suggest hypothyroidism. • Management • Successful therapy rests on adequate disimpaction followed by rigorous adherence to a daily maintenance regimen. • Disimpaction • PEG 3350 (Miralax) 0.7–1.5 g/kg/day PO (maximum 17 grams/day) × 2–4 days. • Phosphate enema: 1 yo: 6 mL/kg PR (maximum 135 mL daily) for 2–3 days. The risk of hyperphosphatemia is increased in infants and those with hemodialysis. • PEG-electrolyte solution (GoLytely): NG 25–40 mL/kg/h (maximum 1 L/h) until rectal effluent clear. • Maintenance • PEG 3350 (Miralax) 1 g/kg/day PO and titrate to achieve daily “mashed potato” consistency bowel movements. • Osmotic (adjust to daily bowel movement): Lactulose 1–3 mL/kg/day PO divided BID. • Stimulant (short-term use only): • Senna: 2–6 yo, 2.5–7.5 mL/day PO; 6–12 yo, 5–15 mL/day PO of 8.8 mg/5 mL syrup. • Bisacodyl: >2 yo, 5–10 mg/day PR; 5–15 mg/day PO. • Milk of Magnesia: 12 yo, 30–60 mL/day daily or div BID • Other: Increase dietary fiber and fluids, educate parents, and provide frequent follow-up. Maintain on therapy for ≥2 months after normal stooling has been established.

180

Pediatrics

• Indeterminate colitis: In about 10% of patients with chronic colitis, it is not possible to make a definitive diagnosis by clinical presentation, radiologic, endoscopic, or histopathologic findings. • These are lifelong diseases characterized by unpredictable exacerbations and remissions. • Etiology • The exact cause is unknown, but consensus is that it is caused by permanent inappropriate activation of the immune response. Multiple factors may contribute, including genetic mutation (NOD2/CARD 15 gene implicated in Crohn’s disease), infectious agents, diet, environmental exposures, and enteric luminal flora. • Incidence is highest amongst whites, especially Ashkenazi Jews; however, there is an rising incidence in African American > Hispanics > Asians. • The single greatest risk for the development of IBD is having a first-degree relative with the condition. The estimated risk in these individuals is 30–100 times greater than in the general population. • Pathogenesis • Chronic inflammation, tissue breakdown, and subsequent fibrosis. • Many types of cells of the immune system are implicated: B cells, T cells, macrophages, and PMNs. • Diagnosis: See table below.

EPIDEMIOLOGIC AND CLINICAL FEATURES OF CROHN’S DISEASE AND ULCERATIVE COLITIS Crohn Disease

Ulcerative Colitis

Age at onset

Bimodal, 15–25 yr and 50–70 yr Recent studies show unimodal distribution (peak in the 20s–30s with diminishing incidence later)

16–20 yr

Incidence

4.5/100,000

2.1/100,000

Intestinal symptoms

Abdominal pain, diarrhea with mucus (bloody, if colonic involvement), n/v, perianal disease, fistulas, abscess

Abdominal pain, grossly bloody diarrhea, tenesmus, urgency, painful defecation

Extraintestinal symptoms

Fever (50%), arthralgias, weight loss (90%), stomatitis, gallstones, kidney stones, uveitis (15%), delayed growth or sexual development (30%), erythema nodosum (15%)

Fever (50%), arthralgias, weight loss (70%), sclerosing cholangitis, pyoderma gangrenosum (5%)

Adapted from Pediatr Clin North Am 1988;35(1):189.

• Differential diagnosis: See table below. • Infectious colitis (especially pseudomembranous colitis from Clostridium difficile, other bacterial enteric pathogens, viral colitis in immunocompromised patients) • Allergic colitis • Milk protein intolerance of infancy • Hirschsprung’s colitis (before or within months of surgical correction). • Prestenotic colitis • Diversion colitis • Ischemic colitis • Radiation proctitis or colitis • Eosinophilic gastroenteritis • Congenital or acquired immunodeficiency syndromes • GVHD in transplant patients • Irritable bowel syndrome

• HUS (initially before the renal failure) • Appendicitis • Intestinal lymphoma • Rheumatoid arthritis • Behçet ‘s syndrome (rare in the United States) • Systemic vasculitis ie, SLE or dermatomyositis • Peptic ulcer disease • Typhlitis • Neonatal necrotizing enterocolitis • Laxative abuse • Malabsorptive syndromes • Henoch-Schonlein purpura

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181

• Diagnostic studies: See tables below.

EVALUATION OF CHILD WITH SUSPECTED INFLAMMATORY BOWEL DISEASE Initial Laboratory Evaluation CBC, PT/INR, ESR/CRP UA Stool guaiac, bacterial culture, O&P, total fat, C. difficile toxin, and in immunocompromised patient viral particles or culture Serum total protein, albumin, transferrin, immunoglobulins Serum lytes; Ca; Mag Phos; iron; zinc; folate; vitamins A, E, 25-OH vitamin D, B12 Serum AST, ALT, alkaline phosphatase, bilirubin

Additional Testing Fecal α1-antitrypsin Lactose breath test Plain films, upper GI with SBFT, CT of the abdomen and pelvis Anti-Saccharomyces cerevisiae antibodies (ASCA): Positive in 40%–60% of patients with Crohn disease Perinuclear antineutrophil cytoplasmic antibodies (P-ANCA): Positive in 60%–70% of patients with ulcerative colitis EGD or colonoscopy with biopsy (gold standard)

RADIOGRAPHIC AND ENDOSCOPIC FEATURES OF CROHN’S DISEASE AND ULCERATIVE COLITIS Endoscopic

Longitudinal or transverse deep ulcerations; skip lesions; strictures; fistulae; cobblestone appearance of mucosa; thumbprinting

Asymmetric or discontinuous involvement; deep, longitudinal fissures; cobblestone appearance; mucosal edema; usually no mucosal friability; strictures (not always present)

Ulcerative colitis

Fine, superficial ulcerations; continuous involvement from the rectum; shortening of the bowel; symmetric bowel contour; decreased mucosal pattern; pseudopolyps

Uniform involvement beginning at the anorectal junction; very friable mucosa; diffuse, uniform erythema; rectal involvement if active disease; pseudopolyps

• Treatment • Long-term treatment is required. Goals of therapy are induction and maintenance of remission. Medical treatment is not curative.

P E D I AT R I C S

Radiographic Crohn’s disease

182

Pediatrics

PHARMACOLOGIC THERAPY OF INFLAMMATORY BOWEL DISEASE Crohn Disease

Ulcerative Colitis

Acute exacerbation Methylprednisolone 1–2 mg/kg/day IV divided BID–QID for 7–10 days. Change to PO (same dose) divided BID; then 1 mg/kg PO once daily; then 1 mg/kg PO every other day, continuing for total course of 4–6 wk.

Severe to moderate colitis Methylprednisolone 1–2 mg/kg/day IV divided BID–QID for 7–10 days. Change to PO (same dose) divided BID; then 1 mg/kg/day PO once daily; then 1 mg/kg PO every other day, continuing for a total course of 4–6 wk.

Remission Olsalazine 30–40mg/kg/day PO divided BID Folate 1 mg/day Mesalamine 50 mg/kg/day divided BID or TID

Mild or Localized Distal Colitis Olsalazine 30–40 mg/kg/day oral divided BID Folate 1 mg/day Mesalamine 50 mg/kg/day divided BID or TID Mesalamine enemas or rectal suppositories Hydrocortisone enemas

Perianal Disease or Fistula Metronidazole 15 mg/kg/day divided Q8h Refractory Disease Azathioprine 1–2 mg/kg/day divided BID or 6-Mercaptopurine 1.5 mg/kg/day divided BID or Methotrexate 10 mg/m2/week (PO or subQ) or Inifliximab 5–10 mg/kg wk 0, 2, 6, and subsequently as needed

Refractory disease Azathioprine 2 mg/kg/day or 6-Mercaptopurine 1.5 mg/kg/d divided BID or Cyclosporine IV 1–2 mg/kg/d; then PO 4–8 mg/ kg/d or Tacrolimus or Inifliximab 5–10 mg/kg wk 0, 2, 6, and subsequently as needed

• Correction of nutritional deficiencies with TPN, vitamins (especially vitamin D), or elemental formulas. • Surgery for disease refractory to medical therapy and complications such as perforation, fistulas, strictures, abscesses, fulminant colitis, or toxic megacolon. • Prognosis: Beyond the first decade of having UC, the risk of developing colon cancer begins to increase rapidly. Patients need regular surveillance colonoscopies after 8–10 years of disease.

FAILURE TO THRIVE • Definition: No consensus definition. Working definition: Child younger than 2 yo whose wt has fallen to < below 3rd or 5th percentile or whose weight crosses two percentile growth curves on a gestational corrected growth chart appropriate for the child’s age and gender. • Etiology: Inadequate intake, abnormal utilization, or increased metabolism.

CAUSES OF FAILURE TO THRIVE IN CHILDREN Inadequate Intake

Abnormal Utilization

Increased Metabolic Demand

• Food availability • Feeding technique • Dysphagia • Feeding aversion • Pyloric stenosis

• Short bowel syndrome • Celiac disease • Inflammatory bowel disease • NEC • Malabsorptive syndrome

• Congenital heart disease, CHF • Chronic inflammatory states • Chronic lung disease • Hyperthyroidism • CF (continued on next page)

Gastroenterology

Inadequate Intake

Abnormal Utilization

Increased Metabolic Demand

• Malrotation • GERD • Child abuse or neglect • Food inappropriate for age • Incorrect mixing of formula

• CF • Inborn errors of metabolism • Chromosomal abnormalities • TORCH infections • Lead toxicity

• HIV, toxoplasmosis • Diabetes mellitus • RTA • Malignancy • TB

183

DETERMINING SEVERITY OF MALNUTRITION IN CHILDREN WITH FAILURE TO THRIVE Method

Normal

Mild

Moderate

Severe

Weight for height∗

≥90% of median

80–89% of median

70–79% of median

1000 μM) Citrullinemia

Figure 17-4 Differential diagnosis of neonatal hyperammonemia. THAN, transient hyperammonemia of the newborn. (Modified from Pediatrics 1998;102(6):E69).

• Remove ammonia: Sodium benzoate or sodium phenylbutyrate PO daily. • Arginine: Up to 600 mg/kg/d in ASS and ASL deficiencies; 100–200 mg/kg/d in OTC and CPS deficiencies. • Citrulline: 50–150 mg/kg/d OTC and CPS deficiencies. • Sufficient fluid intake, G-tube placement for neurologically impaired, vigilant adherence to immunization schedule with prophylactic antipyretics.

Available at www.AccessPediatrics.com • Edwards syndrome • Patau syndrome • Urea cycle defects • Carbohydrate metabolism disorders • Organic acidurias and acidemias

P E D I AT R I C S

Citrulline low

CHAPTER 18

Hematology ANEMIA (RED BLOOD CELL DISORDERS) • Definition: ↓ in Hb, HCT, or RBC count Sβ0 thalassemia > SC > Sβ+ thalassemia) • Diagnosis: Often identified on NBS or with positive FHx; send confirmatory test; hemoglobin electrophoresis. • Presentation: Symptoms occur after 6 mo age; persistence of fetal Hb (>30%) have mild or no sickle symptoms.

CLINICAL PRESENTATION AND TREATMENT OF SICKLE CELL DISEASE Name

Signs and Symptoms

Acute Treatment

Pain crises

Extremity, abdominal pain

Hydration, narcotics, NSAIDs (ibuprofen or ketorolac)

Dactylitis (hand– foot syndrome)

Painful swelling of the hands and feet (infancy)

Hydration, narcotics

Splenic sequestration crisis

Hypotension, anemia (caused by pooling of RBC in the spleen), splenomegaly

Transfusion (simple or exchange), oxygen, and splenectomy in severe cases (continued on next page)

P E D I AT R I C S

Intracellular Defects

200

Pediatrics

Name

Signs and Symptoms

Acute Treatment

Acute chest crisis

Fever, tachypnea, hypoxia, infiltrate (CXR)

Hydration, oxygen, transfusion (exchange or simple), incentive spirometry, positive-pressure ventilation if severe

Aplastic crisis

Severe anemia with reticulocytopenia

Transfusion

Stroke

Focal neurologic deficits, altered mental status

Exchange transfusion; chronic transfusion therapy shown to prevent 80% of second strokes in pediatrics

• Secondary manifestations: Gallstones, renal disease, pulmonary hypertension, avascular necrosis, infection, retinopathy (autosplenectomy, 90% by age 6 yr).

HEALTH MAINTENANCE FOR SICKLE CELL DISEASE Age

Recommended Visits

Studies

Vaccines

Screening

0–6 mo

Every 2 mo

CBC, reticulocyte count, Hb electrophoresis, G6PD test

PCV7 per regular schedule

None

6–24 mo

Every 3–6 mo

CBC, reticulocyte count, Hb electrophoresis, LFT, Chem 7, iron studies

Annual influenza

None

2–5 yr

Every 6 mo

CBC, reticulocyte count, LFT, Chem 7, Iron studies, U/A

Pneumococcal 23-valent and a booster at age 5 yr

Transcranial Doppler (TCD) every yr for HbSS and S-β0 thalassemia; dentist

>5 yr

Every 6–12 mo

Same as 2–5 yr

Annual influenza

TCD, retinal exam starting at 8 yr, hip X-ray starting at 10 yr, ECHO starting at 10 yr and repeated every 2 yr, dentist

Reproduced with permission from Pediatr Rev. 2007; 28:259.

• Treatment: Prophylactic penicillin VK (start by age 3 mo), folic acid (start by age 1 yr); chronic therapies → transfusion of RBC (goal HCT50,000/mm3 with an increase in early neutrophil precursors • Etiology: Drugs (glucocorticoids, G-CSF, ATRA), infections (tuberculosis, pertussis), trisomy 21 in infancy

CAUSES OF LEUKOCYTOSIS Neutrophilia

Lymphocytosis

Eosinophilia

Monocytosis

Primary Hereditary MDS CML Secondary Infection Inflammation Drugs (steroids, β-agonists, lithium) Asplenia

Reactive Infectious Mononucleosis Pertussis Bartonella Toxoplasmosis Babesiosis Noninfectious Hypersensitivity Postsplenectomy Malignancy

Infectious Parasites HIV Fungal Allergic disorders Allergic rhinitis Asthma Connective tissue disease Medications Malignancy

Inflammation Infection Brucellosis Tuberculosis Mononucleosis Rickettsia Autoimmune SLE, RA, IBD Malignancy HD, JMML Sarcoidosis Lipid storage disease

PANCYTOPENI DIFFERENTIAL DIAGNOSIS OF PANCYTOPENIA1 Congenital

Acquired

Fanconi anemia Familial aplastic anemia Dyskeratosis congenita Schwachman-Diamond syndrome

Idiopathic Leukemia/Tumor infiltration Drugs Hypersplenism (sequestration) Chemicals and toxins Radiation Infection (ex EBV, CMV, Parvovirus) Hepatitis Thymoma Nutritional –B12, folate

∗Any patient with two or more abnormal cell lines should have a bone marrow exam. Reproduced with permission from Pediatr Rev. 1984;6:46.

Idiopathic Thrombocytopenic Purpura (ITP) • Diagnosis of exclusion (Pediatr Clin North Am 2008;55:393) • Typical presentation: Abrupt onset bleeding and petechiae after viral illness or immunization → patient well at time of presentation → labs: isolated ↓ platelets • Treatment: Observe (80%–90% of self-limited and resolve in 6 mo); consider treatment (IVIG, steroids, anti-RhD) only if massive bleeding or platelets ≤20,000/microL + risk of bleeding

Hematology

203

PLATELET DISORDERS Platelet disorder suspected Send: CBC, peripheral smear, TEG®

Quantitative defect: Thrombocytopenia Consider bone marrow aspirate

Qualitative defect: Thrombocytopathy Acquired: • Drug induced (aspirin) • Uremia • Myeloproliferative disorders

↑ Destruction

↓ Production

Congenital: • Glanzmann’s thrombasthenia • Giant platelet syndromes (Bernard-Soulier, Gray platelet, May-Hegglin)

Immune mediated: • Viral • Drugs (antiepileptics, antibiotics, heparin) • ITP

Bone marrow failure: See pancytopenia Congenital: • Amegakaryocytic thrombocytopenia (eg, TAR) • Wiskott-Aldrich syndrome

Hypersplenism

Acquired: • Vitamin B12 or folate deficiency • Leukemia or myelodysplasia • Liver failure (thrombopoietin) • Sepsis • Dengue fever

DIC TTP

Figure 18-3 Overview of platelet disorders. (Reproduced with permission from Pediatr Clin North Am. 2004;51:1109).

COAGULOPATHIES Diagnosis

PT

Nl

PTT

Nl

Platelets

Nl

↑

Nl

Nl

↑

Nl

Nl

Differential Diagnosis

Possible Follow-up Studies

von Willebrand disease Platelet function disorder Factor XIII deficiency Fibrinolytic defect

PFA-100 von Willebrand studies Platelet aggregation studies

Hemophilia A or B Circulating inhibitor von Willebrand disease Factor XI deficiency Heparin contamination

PTT mixing study Factor assays (VIII, IX, XI) von Willebrand studies

Circulating inhibitor Vitamin K deficiency Warfarin Factor VII deficiency

PT mixing study Factor assays (VII)

P E D I AT R I C S

DIFFERENTIAL DIAGNOSIS OF COAGULATION AND BLEEDING DISORDERS

204

Pediatrics

• History (Pediatr Clin North Am 49:1239) • Does child bruise easily? (↑ sensitivity, ↓ specificity) or is there excess bleeding with separation of umbilical cord, circumcision, tooth eruption, dental extractions, minor trauma, menstrual bleeding, or surgery? • Epistaxis ≥30 min or results in ↓ Hb. • FHx of easy bruising or bleeding? Bleeding during childbirth? • Initial labs: CBC, peripheral smear, PT, PTT

BLEEDING DISORDERS • Thrombocytopenia and platelet dysfunction: See platelet disorders section. • Disorders of clotting factors (Blood 1994;84:3) • Hemophilia A (factor 8 deficiency; one in 5000 males) or hemophilia B (actor 9 deficiency; one in 25,000 males): X-linked recessive; severity based on the percent of factor present; should be suspected in any male with ↑ PTT.

TREATMENT OF ACUTE BLEEDING IN HEMOPHILIA PATIENTS∗† Factor 8 Replacement Factor 9 Replacement Dose (% correction) Dose (% Correction) Comments

Event Hemarthrosis (any joint)

40–50 u/kg (80–100)

100–120 u/kg (80–100)

Ice x 20 minutes, immobilize x 48 hours

Hematoma (soft 25–35 u/kg (50–70) tissue)

60–85 u/kg (50–70)

Local ice pack x 20 minutes

Head trauma

50 u/kg (100)

120 u/kg (100)

Do not wait for neurologic changes CT after first dose Maintain factor >80%

Major surgery

50 u/kg (100)

120 u/kg (100)

Maintain factor >50%

Dental extraction 50 u/kg (100)

120 u/kg (100)

Amicar 100 mg/kg Q6h x 3–5 d

Mucosal, GI, or GU bleeding

85–120 u/kg (70–100)

Search for cause of bleeding

35–50 u/kg (70–100)

∗Early therapy is important to minimize destructive changes from prolonged blood exposures. Treat within 45 minutes of suspected bleeding whenever possible. † Suspect inhibitor if the patient is not having the expected response to therapy.

VON WILLEBRAND DISEASE Type

Frequency

Defect

Diagnosis

Treatment∗

1

80%

Decreased vWF (relative)

vWF antigen decreased

DDAVP effective in almost all

2 (A/B)

10%–15%

Defective vWF

vWF activity decreased

DDAVP response variable

3

Rare

Deficiency of vWF (absolute)

vWF antigen absent

DDAVP ineffective

∗Treatment is based on patient history, response to prior interventions, and severity of bleeding and includes DDAVP, antifibrinolytics (Amicar), plasma-derived vWF concentrates, and cryoprecipitate.

Hematology

205

RISK FACTORS FOR THROMBOEMBOLISM Congenital

Acquired

Common • Factor V Leiden • Prothrombin 20210 Uncommon • Antithrombin III deficiency • Protein C or S deficiency • Dysfibrinogenemia

• Stasis or immobility: Surgery, trauma • Central venous line • Inflammation: Infection, malignancy, SLE, ↑ factor VIII, ↑ vWF • Abnormal endothelium: Hyperlipidemia, ↑ age • Homocystinuria • Nephrotic syndrome

THROMBOPHILIA • Diagnosis: Patient history: Idiopathic DVT, PE, stroke; multiple or recurrent thrombotic events even if other risk factors present. FHx of DVT, PE, stroke, or MI before 50 yr; multiple pregnancy loss; known thrombophilia. Thrombophilia testing is controversial; consider clinical utility.

ANTICOAGULATION THERAPY∗ Mechanism

Route

Monitoring

Pros

Cons

UFH

Complexes with antithrombin to inactivate thrombin

IV drip Rarely sub-Q

PTT goal, 70–100 sec Anti-Xa activity goal 0.3–0.7 U/mL

Can be stopped quickly or reversed with protamine

Frequent monitoring Risk of HIT

LMWH

Inactivates factor Xa

Sub-Q

Anti-Xa goal 0.5–1.0

Does not require frequent monitoring Patient or parent dosing

Not fully reversible

Warfarin

Inhibits production of vitamin K–dependent factors (II, VII, IX, X)

PO

INR goal, 2–3†

Oral dosing Best option for long-term treatment

Drug and food interactions Difficult to titrate in small patients Teratogen

∗Typical course for DVT or PE: UFH or LMWH for 7–10 d followed by 3–6 mo of LMWH or oral anticoagulants. Therapy should be tailored to the clinical situation. † This is a general range; INR should be based on the degree of anticoagulation needed for the clinical situation. Reproduced with permission from Chest 2008;133(suppl):887S–968S.

BLOOD PRODUCTS Definitions • Blood type: Detection of the presence or absence of ABO and Rh antigens • Cross-match: Determine compatibility of donated unit of blood with intended recipient • Ab screen (indirect Coombs): Test for presence of alloantibodies (not bound to RBCs) to blood antigens • Clinically significant major antibodies (those which are likely to cause hemolytic transfusion reactions), in addition to A and B antibodies, include: D, C, c, E, e, K, k, Fya, Fyb, Jka, Jkb, S, s. These may be due to pregnancy, previous transfusions, etc. Antigen-negative, fully crossmatched PRBCs should be ordered in the presence of any of these antibodies. • Clinically insignificant antibodies (M, N, P1, Lea, Leb ) rarely cause transfusion reactions. While a full crossmatch with recipient serum and donor PRBCs is still recommended, antigen-negative units are not absolutely required.

P E D I AT R I C S

Drug

206

Pediatrics

• Direct Coombs: Test for autoantibodies (bound to RBCs) to blood antigens • Extended phenotype matching: Test to prevent alloimmunization against C, D, E, and Kell antigens in patients receiving frequent transfusions (eg, sickle cell disease)

Blood Component Therapy • Potential risks of blood transfusion • Infectious: HIV-1 (one in 2.1 million), hepatitis C (one in 1.9 million), hepatitis B (one in 205,000–488,000), bacterial contamination (cultured apheresis platelets, one in 75,000; random donor platelets tested by surrogate methods, one in 33,000) • Noninfectious: Acute include hemolysis, fever, allergy, TRALI, transfusion associated circulatory overload (TACO); delayed include hemolysis, transfusion-associated GVHD, transfusion-associated hemosiderosis • Premedication: Diphenhydramine (0.5 mg/kg; maximum dose, 50 mg), acetaminophen (10–15 mg/kg; maximum dose 650 mg), hydrocortisone (1–2 mg/kg, maximum dose 250 mg) (Note: There is no definitive evidence to advocate for empiric use.)

Red Blood Cells INDICATIONS FOR PACKED RED BLOOD CELL TRANSFUSION Hemoglobin (g/dL)

Patients Who May Benefit from Transfusion Intervention

>10

• Neonates • Underlying pulmonary disease or prolonged ventilation • Underlying cardiac disease

8–10

• Undergoing radiation therapy

100,000

• Active bleeding with known or suspected platelet dysfunction • Unexplained, excessive bleeding during cardiopulmonary bypass • Bleeding while undergoing ECMO therapy

50,000–100,000

• Active bleeding • ECMO • Undergoing invasive procedure

20,000–50,000

• Underlying brain tumor • Undergoing radiation therapy

4 mo = 70 mL/kg Plasma volume (PV): TBV × (1 – Hct/100) RBC replacement: Volume (mL) = TBV × desired HCT increase ÷ 57% (average HCT of PRBC) • Number of units cryoprecipitate needed for fibrinogen replacement Desired fibrinogen level∗ – Initial fibrinogen level) mg/dL × PV (mL) ÷ 100 250 mg (fibrinogen of 1 U of cryoprecipitate) • Antithrombin (AT) replacement (for patients receiving unfractionated heparin therapy)† [(Desired AT level‡ – Current AT level) × Body weight (kg)]/1.4

∗200 is usually used as the goal fibrinogen level. †

May round up to a maximum of 300% of the calculated dose or the amount in one vial, whichever is less.

‡

120 is usually used as the desired AT level.

Available at www.AccessPediatrics.com • Folate deficiency • Vitamin B12 deficiency

C H A P T E R 19

High-Yield Ambulatory Care ROUTINE HEALTH SUPERVISION OVERVIEW The American Academy of Pediatrics has established guidelines for routine health supervision visits. Priorities of these visits are the physical exam, developmental observation, universal screening, immunizations (Figures 19-1 and 19-2) and anticipatory guidance (including nutrition, healthy habits, injury prevention, and evaluation of milestones). For complete AAP bright futures universal screening recommendations see: http://brightfutures.aap.org/3rd_Edition_Guidelines_and_Pocket_Guide.html

Age

Developmental Universal Observations Screening

Nutrition

Healthy Habits

Injury Key Prevention Milestones

Allow umbilical cord to air dry, avoid ill contacts, use rectal thermometer (fever ≥100.4 ºF)

Should First stool sleep on and void by the back, age 24 h Rear-facing car seat

Newborn

Periods of wakefulness, responsive to parent voice and touch

State metabolic screen, newborn hearing screen, height, weight, FOC

Breastfeeding or formula, start vitamin D for exclusively breastfed infants

1 wk

Briefly fixes on faces, primitive reflexes

Height, weight, FOC

Breastfeed- Avoid ing or direct formula sunlight

1 mo

Lifts head while Height, prone weight, FOC

Breastfeed- Tummy Set water ing or time while heater formula awake temp to 20 lb

15 mo

Speaks two or three words, scribbles, walks well

Height, weight, FOC

Three Avoid meals/d nighttime with snacks bottle

18 mo

Speaks six words, runs, stacks blocks, uses spoon

DevelopLimit juice ment, auintake tism, height, weight, FOC

2 yr

Speaks 50 words, twoword phrases, follows twostep command, pretend play

Autism, lead (high prevalence, Medicaid), height, weight, FOC, BMI

2.5 yr

Speaks threeto four-word phrases, language understandable 50% of the time, throws ball, puts on clothes with help, points to body parts

Development, height, weight, FOC, BMI

Continue to read with child

Fire safety

3 yr

Self-care skills (feeding, dressing), language understandable 75% of the time, rides tricycle, walks upstairs, alternating feet, copies circle

Visual acuity, measure blood pressure, height, weight, FOC, BMI

Use “timeouts” for discipline, interactive play

Switch to beltpositioning booster seat when >40 pounds and ears reach top of car seat

Consistent discipline for teaching and protecting, avoid punishing

Prevent burns (eg, hot liquids on stove), no spanking

Transition Limit TV Supervise from whole viewing to child to low-fat 20 mg/d if patient >10 kg and duration of steroids 4 wk) • 100% oral bioavailability • Active against some atypical mycobacteria

Bacteriostatic: Inhibit protein synthesis via binding to 30S ribosomal subunit Examples • Tetracycline (PO) • Doxycycline (IV, PO) • Minocycline (PO)

Antimicrobial Spectrum • Rickettsia spp. • Chlamydia spp. • Mycoplasma spp. • Entamoeba histolytica • Ehrlichia spp. • Anaerobes • Streptococcus Group A, B, viridans • S. aureus (MSSA & MRSA) • Gram-negative organisms: M. catarrhalis, H. influenzae, E. coli, Legionella spp., Brucella spp. • Propionibacterium acnes

Adverse Effects

Miscellaneous

• GI disturbance • Phototoxic dermatitis • Renal toxicity • Hepatic toxicity • Staining of permanent teeth • Fanconi syndrome • Teratogenic

• Absorption is impaired by food, milk, Ca, Mg • Avoid use in children 99%

Catheterization

>100,000 10,000–100,000 1000–10,000 10,000 >100,000 in three specimens >100,000 in two specimens >100,000 in one specimen 10,000–100,000

Infection likely 95% 90% 80% Suspicious, especially if symptomatic; repeat culture Infection unlikely

Clean Catch Boy Girl

6 mo age), amoxicillin (neonates), nitrofurantoin

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Pediatrics

IMAGING MODALITIES FOR CHILDREN WITH URINARY TRACT INFECTION OR PYELONEPHRITIS Imaging Modality

Pros

Cons

Renal US

• Most useful modality for imaging kidneys, ureters, bladder; defines renal structure; evaluates for dilatation in collecting system

• Does not provide functional information or detect renal scarring

VCUG and RNC

• Defines the presence and grade of VUR; VCUG detects PUV in male infants

• VCUG has increased radiation exposure (VCUG 10-fold compared with RNC), RNC will not detect PUV in male infants

Technetium-99m DMSA scan∗

• Assessment of renal function and scarring

• Positive findings may not change management

∗ DMSA scan should be done if identification of renal scarring will change management.

IMAGING RECOMMENDATIONS AFTER FIRST FEBRILE URINARY TRACT INFECTION Age and Gender

Imaging Modality

Indication

Birth–24 mo

RUS + VCUG∗

First UTI

Girls 2–6 yr and Boys 2–12 yr

RUS; if RUS is abnormal or the patient has any risk factor (sibling with VUR, ↓ renal function, proteinuria, HTN) also obtain VCUG

First UTI

Girls 6–12 yr

RUS

Consider based on clinical findings suggestive of upper tract disease or history of repeated infections

∗VCUG can be performed as an outpatient; if inpatient, VCUG can be performed after the patient is on appropriate antibiotics and fever is improving (do not need a repeat UA).

MENINGITIS • Definitions • Aseptic meningitis: Inflammation of meninges with no identification of bacterial or fungal organism • Bacterial meningitis: Acute bacterial infection of the meninges and subarachnoid space • Etiology • Bacterial meningitis: See table below for agents that cause bacterial meningitis

Infectious Disease

243

ETIOLOGY OF BACTERIAL MENINGITIS ACCORDING TO AGE AND RISK FACTORS Age or Risk Factor

Organism

Neonatal (0–3 mo)

GBS, E. coli and other gram GNR, L. monocytogenes, S. pneumoniae (usually in infants >1 mo)

Non-neonatal (3 mo–18 yr)

S. pneumoniae, N. meningitidis, Hib∗

Trauma

Penetrating trauma: Staphylococci spp., skin flora organisms, GNRs (including Pseudomonas aeruginosa) Animal bites: Pasteurella multocida CSF leak: S. pneumoniae, Hib, GNRs

Immunodeficiency

Terminal complement deficiency: N. meningitidis Asplenia: S. pneumoniae, N. meningitidis, Salmonella spp. Transplant recipients, T-cell defects: L. monocytogenes HIV: S. pneumoniae, N. meningitidis, Hib, fungal (Cryptococcus)

Direct extension

Dermal sinus tracts, meningomyelocele, urinary tract abnormalities: Staphylococci (S. aureus, CONS), GNR, intestinal organisms Otic fistula, cochlear implants: S. pneumoniae

Neurosurgery

Postneurosurgery: S. aureus, CONS, enteric GNR (including E. coli, Klebsiella spp., P. aeruginosa) VPS: Staphylococci (CONS and S. aureus), fungal, S. pneumoniae, Hib, N. meningitidis, diphtheroids

∗ Less common after introduction of Hib vaccine; unlikely in patients with at least two doses of Hib vaccine

P E D I AT R I C S

• Aseptic Meningitis • Common pathogens: Enteroviruses, arboviruses, HSV, Lyme disease, partially treated bacterial meningitis • Uncommon pathogens: M. tuberculosis, fungi, HIV, adenoviruses, influenza, parainfluenza, EBV, CMV, VZV, mumps, measles, lymphocytic choriomeningitis, Bartonella spp., spirochetes, Leptospira spp., Brucella spp., Mycoplasma spp., rickettsia, parasites, parameningeal bacterial infection • Noninfectious causes: Drugs (NSAIDs, IVIG), systemic immune-mediated diseases, neoplastic diseases, Kawasaki disease • Diagnosis • History • Infants: Inconsolable crying or irritability, lethargy, fever or hypothermia, vomiting, poor feeding, respiratory distress, grunting, apnea, seizures • Children: Fever, lethargy, irritability, myalgia, malaise, headache, anorexia, vomiting, neck and back pain or stiffness, photophobia, mental status changes, seizures (20%–30%; bacterial: S. pneumoniae and Hib; viral: HSV) • Symptom progression: Slow (2–5 d), rapid (1–2 d), or fulminant (shock early in the course of illness) • Physical exam • Infants (1 yr): Neck stiffness, photophobia, Kernig’s sign (patient supine, hip and knee flexed to 90° + if passive extension of knee meets resistance) and Brudzinski’s sign (patient supine and limbs extended + if passive neck flexion results in involuntary hip or knee flexion), cranial nerve palsies (III, VI, VII), ataxia; papilledema (suggestive of brain abscess, epidural or subdural empyema, or sinus venous thrombosis); petechial or purpuric rash (classically N. meningitidis; also found with S. pneumoniae and Hib; 25% of patients with N. meningitidis with nonspecific maculopapular rash very similar to viral exanthem)

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Pediatrics

• Laboratory studies/evaluation: Electrolytes (r/o SIADH), CBC, DIC evaluation, blood cultures (x 2); CSF analysis (cell count with differential; glucose, protein; Grams stain and bacterial culture; viral studies (see “aseptic meningitis” below). See table below for interpretation of CSF. • Contraindications to urgent LP: Hemodynamic instability, coagulopathy, evidence of increased ICP (focal deficit on physical exam, papilledema), h/o recent or chronic CNS lesion, h/o recent CNS trauma, h/o CNS shunt (if VPS in place, CSF should be obtained after neurosurgery evaluation from reservoir or by LP) • Aseptic meningitis CSF evaluation considerations: Enterovirus and HSV PCR, viral culture, AFB stain and culture, acute and convalescent arbovirus titers, EBV, CMV, VZV serologies, HIV ELISA, Mycoplasma and Bartonella serologies, influenza or adenoviral screens

CEREBROSPINAL FLUID ANALYSIS WBC/ CSF Finding mm3

PMN Protein Cells (%) (mg/dL)

Glucose (mg/dL)

Preterm newborn

1000 >80

60–150

8.0), ammonium compounds or detergents, gross hematuria or pyuria • False-negative results: Very dilute and acidic (pH 180 mg/dL in patients with normal renal function • False-negative results: Ketones, high SG, elevated ascorbic acid • False-positive results: Oxidizing agents (bleach)

Hematuria

Negative

GN, nephrolithiasis, urinary tract malignancy or trauma

• Detects intact RBCs, hemoglobin, myoglobin • Dipstick detects 5–10 RBC per μL or 1–2 RBC per hpf (as sensitive as microscopy) • False-positive results: Alkaline urine, oxidizing agents used to clean perineum, semen, ascorbic acid • False negative: excess nitrites, high SG, captopril

Leukocyte esterase

Negative

Infectious pyuria • False-positive results: Drugs or Noninfectious (sterile) pyuria: foods rendering urine red • False-negative results: AntibiInterstitial nephritis, renal otics (gentamicin, cephalexin, TB, nephrolithiasis (see table tetracycline), proteinuria, below) glucosuria (continued on next page)

P E D I AT R I C S

Protein

256

Pediatrics

Normal Component Value

Differential Diagnosis of Abnormalities

Nitrite

Negative

Infection with Enterobacteriaceae (convert nitrate to nitrite)

Crystals

Negative

PRECIPITATE IN ACIDIC URINE: Uric acid: Acidic urine, tumor lysis syndrome (in setting of ARF), hyperuricosuria Calcium oxalate: Hyperoxaluria, hyperglycinuria, ethylene glycol ingestion (in setting of ARF)

Notes and Limitations • False-positive results: Vaginal secretions, phenazopyridine, red dyes or other chromogens, prolonged air exposure • False-negative results: Increased urine SG, increased urobilinogen, urine pH 8.0, chlorhexidine or benzalkonium contamination, concentrated urine (if SG >1.025, dipstick should be 2+); false-negative results: very dilute urine. • Dipstick testing detects albumin only (proteinuria on dipstick testing = albuminuria); glycoproteins, immunoglobulins, Tamm-Horsfall protein, are also excreted by the kidney but not detected by UA. 24-h Urine Collection for Protein • Gold standard; must verify reliability of collection based on expected creatinine in sample

P E D I AT R I C S

Category

258

Pediatrics

Spot Urine Collection (J Pediatr 1990;116:243) • Ratio of urinary protein (mg/dL):creatinine (mg/dL) in random specimen • Directly correlates to 24-h collection sample; often much more practical • Reference ranges: 38.3°; mechanism unknown; UA dipstick ≤2+, resolves with defervescence • Exercise-induced proteinuria: ± Hematuria after vigorous exercise; UA dipstick ≤2+; resolves in 48 h • Orthostatic (postural) proteinuria: Most common cause of persistent asymptomatic proteinuria in school-aged children and adolescents; 32°C. c. There is no spontaneous respiratory effort. d. There is an absence of brainstem function as defined by: (1) Midposition or fully dilated pupils that do not respond to light. (2) Absence of eye movements by oculocephalic and caloric testing. (3) Absence of movement of bulbar musculature including facial and oropharyngeal muscles. The corneal, gag, cough, sucking, and rooting reflexes are absent.

P E D I AT R I C S

Infectious

Environmental Botulism toxins Tick Lead paralysis Mercury Organophosphates n-Hexene Neurotoxic drugs

288

Pediatrics

2. The clinical criteria identified above must be present for the following minimum periods of time depending on the patient’s age: a. Birth–1 mo postterm: 48 h b. 1 mo postterm–1 yr: 24 h c. 1–16 yr: 12 h d. >16 yr: 6 h 3. In any situation in which there is a possible drug effect or a reversible metabolic condition involving the patient’s altered state of consciousness and when there is doubt as to the nature of the pathological process responsible for the cessation of neurologic function, the condition of brain death may be established by studies of cerebral perfusion that document the absence of intracranial circulation. 4. The performance of an EEG is not a prerequisite for a clinical determination of brain death.

EVALUATION OF GLOBAL DEVELOPMENTAL DELAY (Neurology 2003;60:367)

Criteria • Typically applied to children >5 yr • Delays in two or more of the following areas (Figure 22-4): • Fine gross motor delays • Speech and language • Cognition

• Social and personal • Activities of daily living

First-Line Evaluation • History and physical exam: Important components include assessing for regression in development, consanguinity, miscarriages in other pregnancies, family history of developmental delay, dysmorphic features. • Hearing and vision screening exams. • Obtain results of newborn screen or send initial screen if not previously completed. • EEG (if seizures present). • Screen for autism spectrum disorders (see Neurology 2000;55:468).

Second-Line Evaluation • MRI brain without contrast in all patients with global developmental delay (with contrast if seizures present). • Genetic testing • Chromosomes and CMA in all patients • Fragile X testing • Rett testing • Metabolic testing should be completed in the context of historical or physical exam findings suggestive of a specific etiology, including serum amino acids, urine organic acids, acylcarnitine profile, mucopolysaccharides, serum glucose, bicarbonate, lactate, pyruvate, ammonia, and creatine kinase. • Serum lead level.

Available at www.AccessPediatrics.com • Ataxia • Stroke • Weakness

C H A P T E R 23

Oncology ONCOLOGIC EMERGENCIES Fever and Neutropenia • Often the first and only sign of serious infection. It should be evaluated immediately. • Definition • Fever: Single temperature ≥38.3°C (101°F) or a temperature ≥38.0°C (100.4°F) on two occasions 1 h apart. (Note: The temperature should not be taken rectally for immunocompromised patients!) • Neutropenia: ANC 400,000 cells/mm3 without symptoms • Child with AML (M4/M5): WBC >150,000 cells/mm3 • Child with all other AML: WBC >200,000 cells/mm3 with symptoms or >300,000 cells/mm3 without symptoms

NAUSEA MANAGEMENT

EMETOGENIC POTENTIAL OF CHEMOTHERAPY AGENTS Level

Agents

High

Actinomycin-D, cisplatin (>40 mg/m2), cyclophosphamide (>1 g/m2), cytarabine (>1 g/m2), ifosfamide

Moderate

Anthracyclines (daunorubicin, doxorubicin), carboplatin (25% lymphoblasts on BMA • Epidemiology: 30% of childhood cancers; ~2500–3500 cases/yr; peak incidence 2–5 yr of age; Risk is ↑ 14-fold in children with Down syndrome; minor risk ↑ in siblings with ALL and in children with immunodeficiencies

Oncology

293

• Diagnosis • History and physical exam: ↓ Energy, fever (60%), bone pain (25%), night sweats, weight loss, pallor, petechiae or bruising (50%), HSM (66%), lymphadenopathy (50%; definition: epitrochlear or supraclavicular LN >5 mm, inguinal LN >15 mm, and >10 mm for all other LN; malignant LN are typically rubbery, firm, and nontender). May also have headache, nausea or vomiting, AMS, testicular enlargement (2%–5%). Older children may have wheezing, cough, or SVC syndrome caused by a mediastinal mass. • Labs/evaluation: • CBC (hyperleukocytosis; ~50% with a WBC >10,000 and ~20% >50,000); 95% present with ≥1 cell line down (~90% with a Hb 3 SD above age-specific norms) • If suspicion is high, then tissue biopsy (see below), skeletal survey, MIBG scan or bone scan, CT or MRI of the abdomen, CXR ± chest CT (if suspect extraabdominal metastasis), head CT if clinically indicated • Tissue biopsy (small, round, blue cells on histology; immunohistochemical staining; and test for MYCN amplification, hyperdiploidy, CD44 expression, specific translocations for other cancers) and bilateral BMA • Treatment • Surgery: Improved prognosis. Patients with low-risk disease: (stage 1–2b) are treated with surgery alone (J Clin Oncol 1988;6:1271). • Chemotherapy: Cyclophosphamide, carboplatin or cisplatin, etoposide, and adriamycin. The goal is to reduce tumor size. Intermediate risk dz is tx with chemotherapy followed by resection if possible (NEJM 1999;341:1165). For patients with high risk disease highdose chemotherapy with autologous transplant is indicated. • Radiation therapy: For tumors that are unresectable or unresponsive to chemotherapy. • Prognosis • Prognosis is worse compared with other pediatric malignancies (eg, ALL). NB accounts for ~15% of all pediatric cancer fatalities. Five-year EFS varies greatly according to risk group (low risk, 90%–95%; intermediate risk, 85%–90%; high risk, 30%) (Lancet 2007;369:2106). • Favorable features: Age 2 yr, male gender, ↑ ferritin, advanced stage euploidy, MYCN amplification. Malignant CNS Tumors

CHARACTERISTICS OF CENTRAL NERVOUS SYSTEM TUMORS IN CHILDREN YOUNGER THAN 20 YEARS OF AGE International classification of childhood cancer (ICCC) group Astrocytoma

Brainstem Medulloblastoma Glioma

Ependymoma

Site

Cerebrum, cerebellum

Cerebellum

Midbrain, pons, medulla

Incidence (%)∗

52

21

15

9

Peak age (yr)∗

5, 13

3

8, 17

2

M:F ratio∗

1:1

2:1

1:1

1:1

Ventricles or spinal cord; fourth ventricle most common in 3yr, 80% at 5 yr M1: 55%–76% at 5 yr

Grade I: 100% at 5 yr Grade II–IV: 7 yr of age)

13

Repeat tetanus titer to evaluate antibody response; if response is adequate, continue reimmunization; if not, repeat DTaP in 3 mo and follow repeat titer for response

14

DaPT #2, IPV #1, pneumococcal (PCV7) #1, HBV #1 (continued on next page)

P E D I AT R I C S

ELIGIBLE CONDITIONS FOR BONE MARROW TRANSPLANT

300

Pediatrics

Months After BMT

Titers or Vaccines

15

Meningococcal, HIB #1, HAV #1, HBV #2

16

DaPT #3, IPV #2, pneumococcal (PCV7) #2

18

Pneumococcal (PCV7) #3, HIB #2

20

Pneumococcal (PCV7) #4, HBV 3

22

Pneumococcal (23PS) #1, IPV #3, HAV #2

24

MMR, Hib #3

25

MMR #2 Pneumococcal (23PS) #2 (3–5 yr after #1) Influenza vaccine yearly starting ~9 mo after SCT

Adapted from http://www.cdc.gov/vaccines/Pubs/downloads/b_hsct-recs.pdf. ∗For this schedule, HSCT recipients are presumed immunocompetent at ≥24 months after HSCT if they are not on immunosuppressive therapy and do not have GVHD

LATE EFFECTS OF CANCER TREATMENTS Treatment

Important Factors

Common Complications

Chemotherapy

Drug type and doses

Endocrine dysfunction (eg, GH, thyroid deficiency) Gonadal dysfunction Cardiopulmonary toxicities Obesity Neurocognitive delay Psychiatric disorders

Radiation

Total dose and involved field of radiation

Neurocognitive delay with craniospinal XRT or TBI (especially if age 18 mm for boys; >14 mm for girls), 4. open fracture, 5. neurovascular compromise, 6. respiratory compromise, 7. skin tenting, 8. type 2 distal clavicle fracture • Proximal 1/3rd fracture: • Most occur due to high energy impact ie, MVA • Must exclude mediastinal injury (mimics sternoclavicular dislocation) • Consider orthopedic consult for all these types of fracture especially if 1. due to high energy impact, 2. significant displacement >1 cm, 3. posterior diplacement, 4. posterior sternoclavicular dislocation

304

Pediatrics Considerations and management Proximal fracture • Complete displacement, ≤3 cm overriding and 3 mm, or completely displaced → ORIF

306

Pediatrics

ULNA

Considerations and management Olecranon fracture • Nondisplaced → splint • Displaced >3 mm + extra-articular → closed reduction with immobilization/splint • Displaced >3 mm + intra-articular and/or comminuted → ORIF

Splinting: • Posterior splint with elbow flexed at 75°–80° (leave partially extended) F/U: • PCP or ortho consult

Both radius and ulna fracture • Nondisplaced → splint + orthopedic clinic f/u • Plastic deformation (bowing fracture): If >20° angulation or unable to fully rotate → reduction under general anesthesia → ortho consult • Greenstick fracture + rotation and angular deformity present → closed reduction by reversing deforming forces → sugar tong splint or bivalved long arm cast → ortho consult • Complete shaft fracture → closed reduction→ ortho consult if parameters are greater than age appropriate limits (below) • Age 5° → ORIF

Splinting: • Non–weight-bearing • Long leg splint/cast with knee flexed • Ankle at 90°

Calcaneous fracture • Most immobilize with splint • If severely displaced or intra-articular → ORIF Sever’s disease (calcaneal apophysitis) • ⊕ heel squeeze on exam and pain at Achilles insertion • Resolves as growth plate closes → symptomatic treatment

FOOT

Kohler’s disease • Self-limiting AVN of the navicular bone from repetitive stress • Ages 5–7 yr • Symptomatic treatment

• Tuli’s heel cups 4–6 wk • Ice daily • Calf stretch • Rest and ice daily • Persistent pain treated with non–weight-bearing and short leg cast

Metatarsal fracture • Most shaft/neck Fracture → short leg walking cast • Base of 5th metatarsal: (1) avulsion fracture of tuberosity → walking boot; (2) “Jones” fracture of proximal metaphyseal–diaphyseal → ORIF if displaced (poor healing)

Lisfranc fracture • Fracture at base of 2nd MT with ligament injury • Frequently missed in ER and on X-ray, especially without fracture • Poor prognosis if no treatment • Late midfoot collapse a common sequelae • Obtain three-view with oblique X-ray series (weightbearing) • If >2 mm displacement or MT dislocated → ORIF

Splinting: • Non–weight-bearing • Short leg cast for 6 wk • Consider casting even when no fracture seen


FOOT

Orthopedics and Sports Medicine

311

Considerations

Management

Freiberg’s infarction • Self-limiting AVN of 2nd MT head → MT pad • Typically females age >13 yr • Persistent pain → surgery

Splinting: • Short leg cast

Proximal and distal phalanx fracture • Attempt reduction using digital block for anesthesia

Splinting: • Buddy tape to adjacent toe • Walking boot if necessary

Prognosis • Children’s fractures remodel and heal quickly. The younger the patient and the closer the fracture is located to a growth center, the greater the amount of angulation is tolerated. • Children tolerate casting better than adults and develop less stiffness. Often, they are most likely to regain normal ROM and strength, requiring very little to no physical therapy. • Complex regional pain syndrome (late complication) often follows an acute injury and is characterized by hypersensitivity (allodynia), altered blood flow (mottled skin appearance), temperature changes (typically cooler), and swelling.

Available at www.AccessPediatrics.com • Ankle Examination • Knee Examination • Shoulder Examination • Elbow Examination

P E D I AT R I C S

CHAPTER 25

Pulmonology WHEEZING AND ASTHMA Wheezing • Definition: Continuous high-pitched sound with musical quality emitting from the chest during expiration. • Differential diagnosis: Modified asthma predictive index (API; J Allergy Clin Immunol 2004;114(6):1282): Identifies children with wheezing who are at risk for persistent asthma; requires h/o four or more episodes of childhood wheezing and one major and two minor criteria

DIFFERENTIAL DIAGNOSIS FOR A CHILD WITH WHEEZING Age 5 Years

• Asthma • Infection (viral upper or lower respiratory infections, bronchiolitis, tuberculosis, pertussis) • Bronchopulmonary dysplasia • Sinusitis • Foreign body aspiration • Anatomic abnormality (vascular ring, mediastinal mass) • Tracheobronchomalacia • Aspiration due to swallow dysfunction or GERD • Cardiac disease with congestive heart failure • Immunodeficiency, immotile cilia • CF

• Asthma • Vocal cord dysfunction • GERD • CF

MODIFIED ASTHMA PREDICTIVE INDEX Major Criteria

Minor Criteria

Parental history of asthma Physician-diagnosed atopic dermatitis Allergic sensitization to at least one aeroallergen

Allergic sensitization to eggs, milk, or peanuts Wheezing apart from viral illness Blood eosinophilia >4%

Asthma • Definition: Chronic inflammatory disorder of the airways associated with airway hyperresponsiveness that leads to recurrent episodes of combinations of wheezing, breathlessness, chest tightness, or coughing. Usually associated with diffuse but variable airflow obstruction within the lung that is often reversible spontaneously or with treatment. • Pathophysiology: Combination of bronchial smooth muscle constriction and obstruction of the lumen (by inflammatory exudates and airway wall edema).

312

Pulmonology

313

Diagnosis

CLINICAL HISTORY AND PHYSICAL EXAM FOR DIAGNOSING ASTHMA History

Physical Exam

• Age at onset of wheezing

• Respiratory: Evaluate degree of air entry; evaluate for prolonged expiratory phase, tachypnea, retractions (subcostal, sternal, intercostal), wheezing vs stridor • Signs of atopy: Eczema, rhinorrhea, transverse nasal crease (allergic salute), Dennie’s lines (folds inferior to the lower eyelid), allergic shiners (periorbital darkening), conjunctivitis, pale or edematous nasal turbinates • Absence of clubbing • Normal growth

• Frequency, timing (day or night) • H/o prematurity, BPD • Use of urgent care ED visits, hospitalizations, need for O2 or intubation • Comorbid conditions: Eczema, allergies, chronic rhinitis, sinusitis, GERD • FHx of asthma and allergic conditions • Triggers for wheezing: URI or infection, exercise, cold air, allergens (eg, dust mite, animal dander, grass or tree pollen, molds, cockroach), irritant exposures (eg, tobacco smoke, air pollution), emotional stress • Environmental exposures: Pets, tobacco smoke, carpet, dust, cockroaches, central air or heat, use of pillow or mattress covers (dust mite exposure) • Positive response to short-acting bronchodilator • Current medications (check adherence and drug delivery technique)

P E D I AT R I C S

• Diagnostic studies • CXR: May be normal or may show hyperinflation (flattened diaphragm ↑ chest A/P diameter) or peribronchial thickening. • Peak flow monitors: May be useful in children ≥6 yr, especially those with poor symptom perception. Used to assess the severity of exacerbations, monitor response to therapy (compare with personal best at baseline or can estimate goal PEF using predicted table by gender, height, age). Limitations include that it is effort dependent and requires training (can have false high and low values). • Spirometry: Obstructive pattern with ↓ FEV1 and ↓ FEV1/FVC ratio; scooped or concave pattern on expiratory flow–volume loop. • Positive bronchodilator (BD) response = ↑of ≥12% in FEV1 after inhaled short-acting -agonist. • Bronchial provocation tests: May use exercise, histamine, or methacholine to provoke airway hyperresponsiveness with recovery post-BD. • Management: There are four components to asthma management (NAEPP EPR-3, 2007, http://www.nhlbi.nih.gov/guidelines/asthma): • Component 1: Asthma assessment (see table below) and objective monitoring (see table “Assessing control and adjusting therapy”) • Component 2: Education for a partnership in asthma care • Train families in self-management skills, including monitoring, treatment, and communication. • Define goals for good asthma control. Goals should include (1) control symptoms, allowing normal levels of activity and undisturbed sleep; (2) prevent exacerbations; (3) maintain normal lung function; and (4) use minimal therapy necessary to minimize side effects. • Provide a written asthma action (management) plan and emergency information. • Printable asthma action plan template: NHLBI EPR3, page 402(http://www.nhlbi.nih. gov/guidelines/asthma/asthgdln.pdf) • Printable MDI instructions: NHLBI EPR3, page 403(http://www.nhlbi.nih.gov/ guidelines/asthma/asthgdln.pdf)

314

Pediatrics

• Component 3: Control of environmental factors and comorbid conditions that affect asthma • Define specific allergy sensitization (skin testing or RAST/ImmunoCap) and avoid exposure; consider immunotherapy. • Eliminate exposure to active or passive tobacco smoke; limit exposure to air pollutants. • Manage comorbid conditions (GERD, allergic rhinitis). • Annual influenza vaccine, good handwashing. • Component 4: Pharmacologic therapy • All patients need access to quick-relief bronchodilator (short-acting β2-agonist). • Daily controller therapy based on severity and control; consider both impairment and risk (See tables “Medications commonly used for treating Asthma” and “Stepwise approach to management of Asthma” below). • Inhaled drug delivery technique is important to avoid frequent errors; check regularly. • MDI with a spacer has similar or better efficacy, more portability and a shorter administration time compared to a nebulizer. • Spacer (valved holding chamber) with MDI improves lower airway delivery at all ages (including adults) and reduces side effects (thrush with ICS).

CLASSIFYING ASTHMA SEVERITY AND INITIATING THERAPY∗

Risk

Impairment

Components of severity

Persistent Intermittent

Mild

Moderate

Severe

Symptoms

≤ 2 days/wk

>2 days/ wk

Daily

Throughout the day

Nighttime awakenings

0-4 yr: 0 ≥5 yr: ≤ 2x/mo

0-4 yr: 1-2x/mo ≥5 yr: 3-4x/mo

0-4 yr: 3-4x/mo ≥5 yr: >1x/wk

0-4: >1x/wk ≥5 yr: Often, 7x/wk

SABA use for symptoms

≤2 days/wk

>2 days/wk

Daily

Several times per day

Interference with normal activity

None

Minor

Some

Extreme

Lung function

Normal FEV1 between exacerbations FEV1 >80% predicted 5-12 yr: FEV1/FVC >85% ≥12 yr: FEV1/FVC normal

FEV1 >80% predicted 5-12 yr: FEV1/ FVC >80% ≥12 yr: FEV1/ FVC normal

FEV1: 60%-80% predicted 5-12 yr: FEV1/ FVC 75%-80% ≥12 yr: FEV1/ FVC reduced 5%

FEV1 < 60% predicted 5-12 yr: FEV1/FVC< 75% ≥12 yr: FEV1/ FVC reduced >5%

Exacerbations requiring oral corticosteroid

0-1/yr

0-4 yr: ≥2 in 6 mo OR ≥4 wheezing episodes per 1 year lasting >1 day ≥5 yr: ≥2/yr (continued on next page)

Pulmonology

Components of severity Recommended Initial Therapy (see table “Stepwise approach to management of asthma” below)

315

Persistent Intermittent

Mild

Moderate

Severe

Step 1

Step 2

Step 3

0-4 yr: Step 3 5-12 yr: Step 3 or 4 ≥12 yr: Step 4 or 5

and consider short course of oral systemic steroids In 2-6 weeks, evaluate level of asthma control and adjust therapy accordingly. ∗ Some criteria vary by age. Adapted from NHLBI, EPR 3, 2007:41

ASSESSING CONTROL AND ADJUSTING THERAPY∗ Components of Severity

Well Controlled

Impairment

Symptoms

>2 d/wk

Very Poorly Controlled Throughout the day

0–11 yr: ≤1x/mo ≥12 yr: ≤2x/mo

0–11 yr: ≥2x/mo ≥12 yr: 1–3x/wk

0–11 yr: ≥2x/wk ≥12 yr: ≥4x/wk

Interference with normal activity

None

Some limitation

Extremely limited

SABA use for symptoms

≤2 days/wk

>2 days/wk

Several times per day

>80% predicted or personal best >80%

60%–80% predicted 95% Females: ≤20%

Semen analysis Testicular or pelvic US

Genetic counseling Microsurgical epididymal sperm aspiration

Focal biliary cirrhosis

2%–5%

Abdominal US, LFT, liver biopsy

Ursodeoxycholic acid Liver transplantation

Cholelithiasis

≤12%

Abdominal US

Ursodeoxycholic acid Cholecystectomy for recurrent severe symptoms

CF-Related Liver Disease

• Complications • Pulmonary exacerbation: Typically caused by lower respiratory infection. • Systemic signs and symptoms: Anorexia, weight loss or poor gain, ↓ exercise tolerance, fever (atypical). • Symptoms: ↑ Cough, ↑ sputum production (wet cough), change in sputum color/ viscosity, shortness of breath. • Signs: New or ↑ crackles or wheezing, hemoptysis (infrequent). • Objective measures: ↓ in lung function from baseline (FEV1 >10% decrease), new CXR findings, ↓ O2 saturation (infrequent). • Acute treatment: 10–21 d of antibiotics (oral/inhaled or IV/inhaled), supplemental O2 if required, increased airway clearance ± hospitalization. Consider hospitalization with IV antibiotics for multiply resistant bacteria or severe or refractory exacerbations. (If hospitalized, provide contact isolation for MRSA and multiresistant P. aeruginosa and B. cepacia complex.) • Noninvasive positive pressure ventilation has been used for patients with advanced CF lung disease and hypercapnia (≥50 mmHg).

P E D I AT R I C S

∗Goal BMI percentile for patients 2-20 yr is ≥ 50th percentile for age. Data obtained from Am J Clin Nutrition, 2008;88(1):161. CF Patient Registry Annual Data Report 2008. Available at http://www.cff.org.

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Pediatrics

• Lung transplantation: Indications for lung transplant referral include progressive pulmonary function impairment (FEV1 8 yr: 2–4 mg/kg/d divided Q12–24h; max, 100 mg/dose or 200 mg/d

Tissue hyperpigmentation, enamel hypoplasia, or permanent tooth discoloration

S. aureus (MRSA) H. influenzae S. maltophilia

Trimethoprim/ sulfamethoxazole

10–20 mg/kg/d TMP component divided Q6–8h

GI upset, rash, urticaria, myelosuppression

A. fumigatus (ABPA)

Itraconazole

2–5mg/kg/dose Q12–24h

Liver dysfunction

S. aureus (MRSA)

Linezolid

10 mg/kg Q8–12h; max, 600 mg Q12h

Headache, myelosuppression Second-line; very expensive

P. aeruginosa

Tobramycin (TOBI)

300 mg BID in 28-day cycles

Dysphonia, bronchospasm

P. aeruginosa

Aztreonam (Cayston)

75mg once/day x 28days using Altera e-flow device

Bronchospasm, cough

Organism Oral

Inhaled


Pulmonology

323

Drug

Dosage∗

Adverse Effects

P. aeruginosa

Aztreonam

50 mg/kg/dose Q6–8h; max, 50 mg/kg/dose or 2 g/dose

Rash, diarrhea, nausea, vomiting (1%–10%)

P. aeruginosa

Ceftazidime

50 mg/kg/dose Q8h; max, 50 mg/kg/dose or 6 g/d

Diarrhea, may ↑ INR in nutritionally deficient children, hepatic or renal disease

P. aeruginosa

Colistimethate sodium

5 mg/kg/dose divided Q6–8h, increase 1 mg/ kg/dose until 8 mg/kg/ dose (max, 160 mg/dose) is reached or side effects noted

Nephrotoxicity Neurologic abnormalities (primarily paresthesias, may also see dizziness, slurred speech, vertigo, headache, fever)

P. aeruginosa

Gentamicin/ tobramycin

3 mg/kg/dose Q8h (titrate to peak of 10–12 μg/L and trough 75% • Progression to ESRD in 1%–5%, with its development occurring in days up to 20 yr after disease onset • Treatment • Most patients who are clinically well appearing, tolerating PO, and have sufficient pain control can be sent home with close follow-up with their PCP. Recommend checking UA with micro q2 weeks and then space out to q month over a period of 6 months to year. Also annual BP monitoring ± BUN/Cr. • Supportive care with bland diet and outpatient management for mild GI involvement; bowel rest and TPN for moderate to severe GI involvement (severe abdominal pain, bloody stools) • Consider gastric protection with H2 blocker or PPI and H1 blocker if pruritis is present. • Acetaminophen for joint manifestations (NSAID therapy may exacerbate GI symptoms and potentially affect kidneys, although they can be used). • Corticosteroids • Controversial for the prevention of persistent renal disease • Possible benefit in severe GI involvement • Severe renal involvement, CNS disease, or significant pulmonary disease may require stronger immunosuppressive therapy, such as cyclophosphamide.

336

Pediatrics

DISEASES AND DISORDERS WITH SIMILAR CLINICAL FINDINGS TO KAWASAKI DISEASE Viral infections (eg, measles, adenovirus, enterovirus, EBV, CMV) Scarlet fever Staphylococcal scalded skin syndrome Toxic shock syndrome Bacterial cervical lymphadenitis Drug hypersensitivity reactions Stevens–Johnson syndrome Juvenile idiopathic arthritis Rocky Mountain spotted fever Leptospirosis Mercury hypersensitivity reaction (acrodynia)

DIAGNOSTIC CRITERIA FOR COMPLETE KAWASAKI DISEASE ≥5 d of fever1, plus four of the five principal features 1. Changes in extremities • Acute: Erythema and edema of hands and feet • Convalescent: Membranous desquamation of fingertips 2. Polymorphous rash 3. Bilateral, painless bulbar conjunctival injection without exudate 4. Changes in lips and oral cavity (ie, erythema and cracking of lips, strawberry tongue, diffuse injection of oral and pharyngeal mucosa 5. Cervical lymphadenopathy (≥1.5 cm in diameter), typically unilateral

Other clinical findings Cardiovascular findings

• Congestive heart failure, myocarditis, pericarditis, valvular regurgitation, and myocardial infarction • Coronary artery abnormalities • Aneurysms of medium-sized noncoronary arteries • Raynaud’s phenomenon • Peripheral gangrene (usually under 1 year of age)

Musculoskeletal

• Arthritis, arthralgia (15%; usually less than 2-mo duration)

GI tract

• Paralytic ileus • Diarrhea, vomiting, abdominal pain (61%) • Hepatic dysfunction, obstructive jaundice caused by hydrops of the gallbladder

Central nervous system

• Extreme irritability (50%; usually responsive to IVIG therapy) • Aseptic meningitis (related to KD or combination of disease and IVIG therapy) • Sensorineural hearing loss, facial palsy • Anterior uveitis (mild)

Genitourinary system

• Urethritis/meatitis (continued on next page)

Rheumatology

337

Other clinical findings Other findings

• Perineal rash and desquamation • Erythema, induration at previous BCG inoculation site • Beau lines: transverse furrows of fingernails in convalescence phase • Macrophage activation syndrome

1 A diagnosis of KD can be made on the fourth day of fever when four out of five criteria are present, or if coronary abnormalities are present in the absence of full criteria. Experienced clinicians who have treated many KD patients may establish diagnosis before day 4. Adapted from Pediatrics 2004;114(6):1708.

• Incomplete Kawasaki Disease: If diagnostic criteria for complete KD are not met, incomplete KD should be strongly considered in the appropriate clinical scenario (see table and “Evaluation of suspected incomplete KD” algorithm below.)

LABORATORY FINDINGS SUGGESTIVE OF INCOMPLETE KD Elevated acute-phase reactants (CRP ≥3.0 mg/dL or ESR ≥40 mm/hr) White cell count ≥15,000/cells/mm3 Normocytic, normochromic anemia for age (see Hematology chapter) Pyuria: ≥10 WBCs/high-power field (obtained by bag or clean-catch method, not catheterization, since cells are of urethral origin) Serum alanine aminotransferase level >50 U/L Serum albumin ≤3.0 g/dL After 7 d of illness, platelet cell count ≥450,000/μL CSF pleocytosis (LP is not part of the routine KD evaluation) Adapted from: Pediatrics 2004;114(6):1708.

P E D I AT R I C S

• Treatment • IVIG, 2 g/kg given as a single infusion over 10–12 hr. • Note: Infants with cardiac compromise may not tolerate volumes, and dividing dose over several days is an appropriate consideration. • Repeat IVIG dose (2 g/kg) if fever does not abate 48 hr from completion of initial IVIG infusion. • If after second dose of IVIG, patient continues with fever, consider corticosteroid therapy with 1–3 days of pulsed IV methylprednisolone (30 mg/kg). • Limited data suggest benefit from anti-TNF agents (infliximab or etanercept) for KD refractory to IVIG and corticosteroids. • Aspirin 80–100 mg/kg/day in four divided doses until afebrile for 48 hr → then aspirin 3–5 mg/kg/day once daily until repeat negative echo at 6–8 wk into illness, then stop aspirin. • Caveats • Therapy prior to day 5 of illness may be associated with an increased need for IVIG retreatment, but this should not dissuade the physician from prompt therapy, as this usually indicates ongoing vasculitis/inflammatory response. • Administration of IVIG should occur for those presenting after the 10th day of illness (ie, earlier missed diagnosis of KD) if persistent signs of inflammation (ie, fever, ↑ ESR or CRP) or of evolving CAD. • Follow-up imaging • For uncomplicated cases of KD (negative echocardiogram at presentation, adequate response to treatment), obtain repeat echocardiogram at 1–2 wk from 1st day of fever and 6–8 wk from the first day of fever. Consider other modalities of imaging (eg, cardiac MRI) if quality of echocardiogram is suboptimal.

338

Pediatrics Fever ≥ 5 days and 2 or 3 clinical KD criteria 1

Assess Patient Characteristics 2 Inconsistent with KD

Consistent with KD

KD Unlikely

Assess Laboratory Tests

CRP ≥ 3.0 mg/DL and/or ESR ≥ 40 mm/hr

CRP < 3.0 mg/DL and ESR < 40 mm/hr

< 3 Supplemental Laboratory Criteria 3

≥ 3 Supplemental Laboratory Criteria 3

Fever resolves

Echo

Treat and Echo 4

No Typical Peeling Peeling 7

Echo +

Follow Daily

Fever continues for 2 days

Persistent Fever

No f/u

Fever Persists

Echo 5

Repeat Echo, Consult KD Expert

Echo + 5

Fever Abates

Treat 6

KD Unlikely

Figure 26-3 Evaluation of suspected incomplete KD. (Reprinted with permission from Pediatrics 2004;114(6):1708.) 1 Infants ≤6 mo on day ≥7 of fever without other explanation should undergo laboratory testing; if evidence of systemic inflammation is found, obtain echocardiogram (even if infant has no other clinical criteria). 2 Characteristics suggesting KD are listed in the table “Diagnostic Criteria for Complete Kawasaki Disease”. Characteristics suggesting disease other than KD include exudative conjunctivitis, exudative pharyngitis, discrete intraoral lesions, bullous or vesicular rash, or generalized adenopathy → Consider alternative diagnoses (see the table, “Diseases and Disorders with Similar Clinical Findings to Kawasaki Disease”). 3

See the table LABORATORY FINDINGS SUGGESTIVE OF INCOMPLETE KD.

4

Do not delay treatment for echocardiogram.

5

Echocardiogram is considered positive for purposes of this algorithm if any of the following three conditions are met: (1) z score of LAD or RCA ≥2.5, (2) coronary arteries meet Japanese Ministry of Health criteria for aneurysms, or (3) ≥3 other suggestive features (perivascular brightness, lack of tapering, decreased LV function, mitral regurgitation, pericardial effusion, or z scores in LAD or RCA of 2–2.5) exist. 6 If the echocardiogram is positive, treatment should be given to children within 10 d of fever onset and those beyond day 10 with clinical or laboratory signs (CRP, ESR) of ongoing inflammation. 7

Typical peeling begins around nail bed of fingers and then nail bed of toes.

Rheumatology

339

Coronary Thrombosis Treatment (see Pediatrics 2004;114(6):1708) • Coronary thrombosis management in Kawasaki Disease (see Pediatrics 2004;114(6):1708) • Cardiology consult • Acute therapy includes fibrinolytic therapy (streptokinase, urokinase, or tPA) in a tertiary care center under the supervision of a cardiologist. • Chronic therapy for coronary artery aneurysm (CAA): • Aspirin 3–5 mg/kg/day once daily • Some add dipyridamole or clopidogrel to ASA in those considered high risk. • Some add anticoagulant therapy with warfarin or heparin in combination with antiplatelet therapy for children with severe coronary involvement or past coronary thrombosis.

BENIGN HYPERMOBILITY SYNDROMES • Epidemiology • 10%–25% of the population has hyperflexible joints, not necessarily indicative of congenital syndrome or connective tissue disorders • Age affected: 3–10 yr • ♀:♂ ratio 2:1 • Familial tendency • Diagnosis • Clinical manifestations • Varying degrees of joint laxity without instability or disability • Arthralgia and/or transient mild swelling during the afternoon or night or after activities • Commonly involves lower extremities and larger joints but can involve any joint • Frequent ankle or wrist sprains • Absence of underlying systemic or rheumatic disease

COMMON GENETIC CONDITIONS ASSOCIATED WITH HYPERMOBILITY • Osteogenesis Imperfecta • Williams syndrome • Trisomy 21 (Down syndrome)

• Ehlers–Danlos syndrome • Treatment • Reassurance (symptoms diminish during musculoskeletal maturation occuring in puberty) • Supportive footwear • Strength training and guided exercise • Bracing • Avoidance of hyperextension of joints, but physical activity should not be discouraged • ± NSAIDs or acetaminophen

Available at www.AccessPediatrics.com • Approach to the child with suspected rheumatic disease • Seronegative spondyloarthropathies • Childhood fibromyalgia • Complex regional pain syndrome • Juvenile dermatomyositis

P E D I AT R I C S

• Marfan syndrome • Homocystinuria • Stickler syndrome

CHAPTER 27

Toxicology • One to two tablets or 1 to 2 tsp (standard pediatric “swallow” ~5–10 cc) of the standardstrength preparations of the following medications are potentially fatal to a 10-kg child: • Antiarrhythmics, antimalarials (chloroquine, hydroxychloroquine), benzocaine, β-blockers, CCBs, camphor, clonidine, Lomotil (diphenoxylate/atropine), lindane, methyl salicylate, opioids (codeine, morphine, pentazocine), phenothiazines (thioridazine, chlorpromazine), quinine, theophylline, TCAs (imipramine, desipramine) • Nontoxic pharmaceuticals: Antacids, antibiotics, corticosteroids, contraceptives, laxatives, mineral oil, ACE inhibitors • Nontoxic household products: Cosmetics, deodorants, dehumidifying (silica gel) packets, matches, candles

COMMONLY INGESTED AGENTS IN POISONINGS IN CHILDREN YOUNGER THAN 6 YEARS (2005) Cosmetics or personal care substances

13.4%

Cleaning products

9.8%

Analgesics

8.2%

Foreign bodies

7.4%

Topicals

7.2%

Cough and cold medicines

5.7%

Plants

4.0%

Pesticides

4.0%

Vitamins

3.9%

Antihistamines

2.9%

Data from Clin Toxicol. 2006;44:803.

INITIAL INGESTION EVALUATION (Emerg Med Clin North Am 2007;25:249) Poisoned patient Diagnosis History Physical examination Toxidrome recognition Diagnostic testing

Treatment Airway Breathing Circulation Decontamination Enhanced elimination Focused therapy Get tox help: Call poison control (800-222-1222)

Figure 27-1 Simultaneous diagnostic and treatment approach to a poisoned patient. (Adapted from Emerg Med Clin North Am 2007;25:249)

340

Toxicology

341

• History: Should address the questions what?, when?, how?, how much?, what symptoms?, and why?: Timing of ingestion, substance(s) taken, amount taken (pill count or volume quantification, if bottle available), location of ingestion (kitchen vs bedroom vs other), reason or circumstances of exposure or ingestion, symptoms before arrival, other substances in the home (possible co-ingestions), concomitant illness or trauma (possible supratherapeutic or chronic dosing of acetaminophen or aspirin), history of depression or prior suicide attempts (in adolescents), history of street drug use.

STREET NAMES OF COMMON DRUGS OF ABUSE Marijuana: Acapulco gold, bhang, doobie, ganja, grass, joint, Mary Jane, pot, rope, reefer Amphetamines: Black beauties, crank, crystals, cat (methcathinone), ice, ecstasy, meth, pep pills, smart drug (Ritalin), speed, uppers Ecstasy: Adam, E, lollies, love drug, smarties, vitamin E, XTC Heroin: Boy, China white, dust, Harry, horse, junk, monkey, smack, speed ball (with cocaine), atom bomb (with marijuana)

PCP: Angel dust, goon, horse tranquilizer, hog, Sherman, tank, wickie stick (with marijuana) δ-hydroxybutyrate (GHB): Bioski, Georgia home boy, grievous bodily harm, liquid G, liquid ecstasy, somatomax, cow growth hormone Cocaine: All-American drug, coke, crack, girl, mother of pearl, nose candy, Peruvian powder, snow, toot, white lady LSD: Acid, blotters, microdots, paper acid, pyramids, window pane, Zen

AGENTS OF INTOXICATION WITH UNIQUE ODORS Odor

†

Agent

Wintergreen

Methyl salicylate

Garlic

Arsenic, DMSO, organophosphate insecticides, selenium

Rotten eggs

Hydrogen sulfide, sulfur dioxide

Fresh hay

Phosgene

Mothballs

Camphor, naphthalene, paradichlorobenzene

Bitter almond†

Cyanide

Pears

Chloral hydrate, paraldehyde

Fruity

Isopropyl alcohol, ketones (diabetic ketoacidosis), phenol

Carrots

Water hemlock (cicutoxin)

30% of the population is unable to detect; Bitter almonds are not readily available in the United States

P E D I AT R I C S

• Anticipate: Seizures, altered mental status, multi-organ dysfunction, respiratory failure, cardiac failure, arrhythmias, or unexplained metabolic acidosis. • Primary survey: See Figure 27-1. • Secondary survey, laboratory investigation: More detailed history and physical exam (including possible abnormal odors), including pulse oximetry and cardiac monitoring, laboratory testing (glucose, electrolytes, anion gap, osmolar gap, baseline CBC and liver panel, urinalysis for hemoglobinuria or myoglobinuria, urine pregnancy test), blood gas (with carboxyhemoglobin if carbon monoxide toxicity is possible), urine drug screen (detects limited number of agents; rarely, color of urine may be helpful), serum drug levels (only to guide therapy or predict toxicity, eg, acetaminophen, salicylates, theophylline, lithium, lead, iron, methemoglobin, toxic alcohols, anticonvulsants, digoxin), chest radiography (if respiratory symptoms present, may identify selected radiopaque objects), baseline ECG (evaluate QRS duration for TCA, β-blocker, CCB toxicity).

342

Pediatrics

RAPID URINE TESTING FOR DRUGS OF ABUSE Drug

Duration of Urine Detectability

Drugs Causing False-Positive Preliminary Results

Amphetamines

2–3 d

Amantadine, bupropion, chlorpromazine, desipramine, ephedrine, phenylephrine, pseudoephedrine, ranitidine, selegiline, trazodone

Cocaine

2–3 d

Topical anesthetics containing cocaine-like structure

Marijuana

1–7 d (light use); 1 mo with chronic moderate to heavy use

Dronabinol, efavirenz, hemp seed oil, ibuprofen, naproxen

Opiates

1–3 d

Fluoroquinolones, poppy seeds, quinine (tonic water), rifampin

Phencyclidine

7–14 d

Dextromethorphan, ketamine

Data from Med Lett 2002;44 (1137):71

URINE COLOR CHANGE ASSOCIATED WITH POISONINGS Color

Substance Responsible

Orange to red-orange

Deferoxamine, hydroxocobalamin, lead (chronic poisoning), mercury, phenazopyridine, rifampin

Pink

Ampicillin, cephalosporins

Brown

Carbon tetrachloride, chloroquine

Blue-green

Copper sulfate, methylene blue

ITEMS OF INGESTION DETECTABLE WITH CONVENTIONAL RADIOGRAPHY (CXR, KUB) Chloral hydrate Calcium Opiate or cocaine packets

Iron and other heavy metals Neuroleptic agents Sustained-release or enteric-coated agents

PHYSICAL EXAM FINDINGS OF SELECTED TOXINS* Symptoms

Intoxications

Withdrawals

Tachycardia

Anticholinergics, sympathomimetics, theophylline, levothyroxine, TCAs

Barbiturates, benzodiazepines, ethanol

Bradycardia

β-blockers, CCBs, cholinergics, cholinesterase inhibitors, clonidine, digoxin, ethanol,∗ opiates

Tachypnea

Alcohols, hydrocarbons (chemical pneumonitis), nerve agents, PCP, phosgene, salicylates

Bradypnea

Barbiturates, benzodiazepines, ethanol,∗ marijuana, opioids (continued on next page)

Toxicology

343

Symptoms

Intoxications

Pulmonary edema (noncardiogenic)

Meprobamate, methadone, opioids, paraquat, phenobarbital, phosgene, propoxyphene, salicylates

Withdrawals

Hypoxia

Benzocaine (secondary to methemoglobinemia), carbon monoxide, opioids, sedative–hypnotics

Hypertension

Anticholinergics, caffeine, nicotine, PCP, sympathomimetics, levothyroxine

Ethanol

Hypotension

Antihypertensives, barbiturates, benzodiazepines, ethanol,∗ iron, opioids, rodenticides (containing arsenic, cyanide), TCAs


Miosis

Cholinergics, clonidine, organophosphates, opioids, oxymetazoline, phenothiazines, pilocarpine

Mydriasis

Anticholinergics, antihistamines, marijuana, sympathomimetics, TCAs

Opioid

Hyperthermia

Amphetamines, anticholinergics, cocaine, MAOIs, neuroleptic malignant syndrome, PCP, salicylates, serotonin syndrome

Ethanol

Hypothermia

Barbiturates, benzodiazepines, carbon monoxide, clonidine, ethanol, opioids, oral hypoglycemics, phenothiazines, TCAs

Seizures

Amphetamines, anticholinergics, atropine, bupropion, caffeine, camphor, carbon monoxide, chlorinated hydrocarbons (lindane), cocaine, cyanide, INH, inhalants, insulin, lead, lidocaine, lithium, methanol∗, mushrooms, nicotine, oral hypoglycemics, organophosphates, PCP, salicylates, strychnine, TCAs, theophylline

Anion gap metabolic acidosis (see Fluid, Electrolyte, and Acid–Base Balance, Chapter 15, for more detail)

Alcohols∗ (ethanol,∗ ethylene glycol,∗ methanol∗), carbon monoxide, cyanide, ibuprofen, INH, iron, metformin, salicylates

Hypoglycemia

Alcohols,∗ β-blockers, insulin, oral hypoglycemics, salicylates

Acute ataxia or nystagmus

Alcohols,∗ anticonvulsants, (especially phenytoin) antihistamines, barbiturates, carbon monoxide, organic solvents, PCP (rotary nystagmus, versus vertical nystagmus of brainstem lesion)

Emesis, severe

Boric acid, corrosives, fluoride, iron, salicylates, theophylline

Renal failure

Carbon tetrachloride, ethylene glycol,∗ methanol,∗ mushrooms, oxalates

Skin Color

Red: Anticholinergics, boric acid, carbon monoxide, cyanide Blue: Methemoglobinemia


P E D I AT R I C S

Opioids


344

Pediatrics

Symptoms

Intoxications

Withdrawals

Diaphoresis

Organophosphates, PCP, salicylates, sympathomimetics

Opioids

Altered mental status

Anticonvulsants, antihistamines, antipsychotics (risperdone), benzodiazepines (Rohypnol), carbon monoxide, clonidine, cyanide, GHB, ethanol,∗ ethylene glycol,∗ heavy metals (lead), hemlock, INH, insulin, lithium, opioids, oral hypoglycemics, TCAs, thallium, toluene, toxininduced hepatic encephalopathy

Ethanol

∗ Associated with an increased osmolar gap.

TOXIDROMES

HR Adrenergic ↑

Anticholinergic

↑

Cholinergic

↓

RR Pupils

Other Skin and Neurologic Symptoms Mucous or Mental or Membranes Status Mnemonic Agents

↑

Dilated, reactive

Diaphoretic

Agitated, psychosis, delirium

± Hyperthermia, tremors, hypertension, hyperreflexia

Amphetamine, caffeine, cocaine, ephedrine, sympathomimetics, theophylline

↔

Dilated, sluggishly reactive

Dry, flushed skin; dry mucous membranes

Depressed, confused, hallucinations, EPS

HOT as a hare, MAD as a hatter, RED as a beet, DRY as a bone, BLIND as a bat: Hyperthermia; delirium or hallucinations; flushed, dry skin; dilated pupils

Antihistamines, atropine, belladonna alkaloids, benztropine, mushrooms, phenothiazines, scopolamine, TCAs

Constricted

Diaphoretic skin; salivation, tearing

Depressed, confused

DUMBBELLS: Diarrhea and GI upset, urination, miosis, bradycardia, bronchoconstriction, emesis, lethargy, lacrimation, secretions or salivation

Black widow spider bites, mushrooms, pesticides (carbamates, organophosphates), pilocarpine


Toxicology

345

Other Skin and Neurologic Symptoms Mucous or Mental or Membranes Status Mnemonic Agents

HR

RR Pupils

Opioid

↓/↑

↓

Constricted

Dry or no change

Depressed, confused

Constipation Narcotics or urinary retention, ± hyporeflexia, pulmonary edema

Salicylism

±↑

↑

Normal

No change

Confusion, seizures, tinnitus

Nausea, metabolic acidosis

Sedative– ↔/↓ ↓ hypnotics

Normal or constricted

No change

Depressed, confused

± urinary Benzodiazretention, epines constipation, hypotonia

Normal

No change

Confusion, coma, seizures

Ventricular arrhythmia, ↓ BP, anticholinergic effects

TCAs

↑

↑

Aspirin, oil of wintergreen

Imipramine, amitriptyline

OVERVIEW OF ANTIDOTES AND RECOMMENDED INITIAL DOSING IN PEDIATRICS Antidote

Acetaminophen

NAC PO: 140 mg/kg loading dose Then 70 mg/kg Q4h x 17 doses; see acetaminophen toxicity treatment section for IV dosing

Arsenic, mercury, lead, other heavy metals

Dimercaprol: 3–5 mg/kg/dose Q4h (duration depends on degree of intoxication)

Benzodiazepines

Flumazenil 0.01 mg/kg IV over 30 sec; may repeat q1 min (max dose 0.2 mg or 0.05 mg/kg, maximum of 5 dose series q 30 min; lack of response after first 5-dose series suggests alternate etiology)

β-blockers

Glucagon 0.05 mg/kg bolus followed by infusion of 0.05 mg/kg/h with vasopressors as needed

Carbon monoxide∗

Mild to moderate (COHb 20%): Hyperbaric oxygen chamber therapy

Carbon tetrachloride

NAC: Generally same dosing schedule as acetaminophen overdose

Digoxin

Anti-digoxin antibody: Estimate digoxin antibody vials (each vial is 38 mg) to administer once digoxin reaches steady state (~8h postingestion): (Serum digoxin [ng/mL] × Body weight [kg])/100

Ethylene glycol, methanol

Fomepizole: No dosage data in pediatrics, may use: 15 mg/kg IV load; maintenance dose 10 mg/kg Q12h × 4 doses

Iron

Deferoxamine 15 mg/kg/h infusion

Lithium

Sodium replacement as needed; low-dose dopamine; hemodialysis (continued on next page)

P E D I AT R I C S

Toxin

346

Pediatrics

Toxin

Antidote

Methemoglobinemia

Methylene blue: 1–2 mg/kg

Neuroleptics (resulting in acute dystonia)

Diphenhydramine: 5 mg/kg divided Q6–8h or benztropine 1–2 mg IV/IM (adults), 0.02 mg/kg IV/IM (children >3 yrs, max 1 mg; use in children 2 d

Ibuprofen

400 mg/kg

Iron

50–60 mg/kg elemental iron; >500 μg/dL serum value at 6 h postingestion

Salicylates

150 mg/kg (SI units) μM/L μg/mL 1000 6000 5000 4000 3000

500

1300

200

1000 900 800 700 600 500 400

150

Probable hepatic toxicity

P E D I AT R I C S

50 ib

ss le

300 250 200

100

Po tic

No hepatic toxicity

ity

xic

to

100 90 80 70 60 50

pa

he

Acetaminophen plasma concentration

2000

10

40 30

25%

5

20

10

0

4

8 12 16 Hours after ingestion

20

24

Figure 27-2 Nomogram for prediction of acetaminophen hepatotoxicity after acute overdose. Note: The upper line defines serum acetaminophen concentrations likely to be associated with hepatotoxicity; the lower line defines serum levels 25% below those expected to cause hepatotoxicity. (Reprinted with permission from Olson KR: Poisoning & Drug Overdose, 5th ed. New York: McGraw-Hill. Copyright © The McGraw-Hill Companies, Inc. All rights reserved.)

Available at www.AccessPediatrics.com • Acetaminophen toxicity • GI decontamination, including activated charcoal, orogastric lavage, whole-bowel irrigation

Section 4: Neonatology CHAPTER 28

Newborn Nursery Neuromuscular maturity 0

-1

1

2

3

4

5

Posture

Square window (wrist)

>90°

90°

60°

45°

180°

0°

45°

140° to 180°

Arm recoil

30°

110° to 140°

90° to 110°

Popliteal angle 100

Respiratory effort

Absent

Slow, irregular

Good, crying

Muscle tone

Limp

Some flexion

Active motion

Response to catheter in nostrilb

No response

Grimace

Cough or sneeze

Color

Blue or pale

Body pink; extremities blue

Completely pink

Figure 28-2 Infant evaluation at birth—Apgar score. (Reproduced with permission from Hay WW Jr, Levin MJ, Sondheimer JM, Deterding RR: Current Diagnosis & Treatment: Pediatrics, 19th ed. New York: McGraw-Hill. Copyright © The McGraw-Hill Companies. All rights reserved.) a

One minute and 5 minutes after complete birth of the infant (disregarding the cord and the placenta), the following objective signs should be observed and recorded.

b

Tested after the oropharynx is clear.

• Term babies take 0.5–1 oz q2–4hr initially. • Breastfeeding should occur q1-3hr (see Chapter 4).

Eyes • 0.5% erythromycin ointment to both eyes within 2 hr after birth for prophylaxis against ophthalmia neonatorum.

Vitamin K • 0.5–1 mg IM within 6 hr of life to prevent hemorrhagic disease of the newborn.

Stool • Meconium within 48 hr of life.

Urine • Void within 30 hr of life.

Umbilical cord • Triple dye, then keep cord clean and dry until stump falls off (average 6–14 DOL).

Nails • Keep short with emery board or baby nail clippers.

Sleep • Back to sleep to reduce the risk of SIDS.

• 37-41 completed weeks’ gestation • No findings that require continued hospitalization. • Vital signs documented as normal (see “Newborn Exam” above).

NE ON AT OLO GY

EARLY DISCHARGE CRITERIA (47–48

95

51

Low

Yes

No

FLM-TDx >55 mg/g

96–100

47–61

Moderate Yes

Yes

Lamellar ≥50,000 body count

97–98

29–35

Low

Unknown

Blood only

Adapted from ACOG Practice Bulletin No. 97: Fetal Lung Maturity. Obstet Gynecol 2008;112:717.

THE FETAL ENVIRONMENT: MATERNAL MEDICAL CONDITIONS Diabetes Mellitus During Pregnancy

NE ON AT OLO GY

• Fetal complications of maternal DM • Congenital anomalies • Incidence, 5%–10%; two to four times the control population • Caudal dysplasia (sacral agenesis): 200- to 400-fold increase • Neural tube defects: 10-fold increase; anencephaly, myelomeningocele • Congenital heart disease: Fivefold increase; hypertrophic cardiomyopathy, VSD, transposition of the great arteries • Macrosomia: Increased risk of cephalopelvic disproportion, traumatic delivery, Erb’s palsy • Polyhydramnios: 15% incidence • IUGR: Only in mothers with very poorly controlled diabetes, with a diabetic vasculopathy of the placental vessels, leading to poor placental growth • Hypoxia • Intrauterine fetal demise: Stillbirth rate increases when postprandial glucose level is >120 mg/dL • Perinatal mortality • Increased risk of developing type II DM later in life

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Neonatology

Hypertension in Pregnancy • Definitions • Preeclampsia: New-onset hypertension (BP >140/90 mm Hg or MAP >105) with proteinuria (>30 mg/dL or +1 in a random specimen or 300 mg in a 24-h specimen) ± edema • Eclampsia: Development of convulsions or coma in the setting of preeclampsia • Gestational hypertension: Hypertension occurring after the 20th week of pregnancy or during the first 24 hours postpartum without evidence of preeclampsia • Chronic hypertension: Hypertension diagnosed before pregnancy or before the 20th week of pregnancy; or hypertension diagnosed during pregnancy but persisting beyond the 42nd postpartum day • Superimposed preeclampsia: Worsening of hypertension (increase in SBP >30 mm Hg or DPB >15mm Hg) or increase in the degree of proteinuria in a pregnant patient with chronic hypertension

SEQUELAE OF MATERNAL HYPERTENSION IN NEONATES System

Findings

Clinical Manifestations

Cardiovascular

Increased cardiac output, systemic vasoconstriction, and hypertension Increased hydrostatic pressure

Systemic hypertension, intravascular hemolysis Generalized edema

Uteroplacental

Uteroplacental insufficiency

Fetal somatic growth deficiency; fetal hypoxemia and distress Abruptio placentae; placental infarcts Thrombocytopenia

Decidual ischemia Decidual thrombosis Renal

Decreased renal blood flow and GFR; endothelial damage High AT-II responsiveness of tubular vasculature All of the above

Proteinuria; elevated Cr, decreased Cr clearance; oliguria Elevated uric acid

Cerebrovascular

Cerebral motor ischemia High CPP with regional ischemia Cerebral edema Regional ischemia

Eclampsia (seizures) Cerebral hemorrhage Coma Central blindness; loss of speech

Hepatic

Ischemia; hepatocellular injury Mitochondrial injury

Elevated LFTs Intracellular fatty deposits

Hematologic

Intravascular hemolysis

Schistocyte burr cells; elevated free hemoglobin and iron; decreased haptoglobin levels Thrombocytopenia; antiplatelet antibodies

Decidual thrombosis, release of fibrin degradation products

ATN, renal failure

• Fetal and neonatal complications • Prematurity (often caused by medical intervention) • Uteroplacental insufficiency → fetal somatic growth deficiency, fetal hypoxemia or distress • Abruptio placentae

Fetal Assessment and Prenatal Diagnosis

357

FETAL AND NEONATAL COMPLICATIONS OF MATERNAL MEDICAL CONDITIONS Maternal Medical Condition

Maternal Complications During Pregnancy

Fetal and Neonatal Complications

DM

Hypoglycemia; DKA; hypertension; progression of diabetic retinopathy; polyhydramnios; cephalopelvic disproportion; increased risk of malpresentation, cesarean section, and operative vaginal delivery

Hypoglycemia, macrosomia, birth trauma, IUGR, hypoxia, caudal dysplasia, congenital heart defects, neural tube defects, intrauterine fetal demise

Hypertension

Generalized edema, intravascular hemolysis, thrombocytopenia, renal insufficiency, proteinuria, seizures, cerebral hemorrhage, coma, central blindness, elevated liver enzymes, abruption placentae, preterm labor

IUGR, fetal hypoxemia and distress, prematurity

Hypothyroidism

First trimester miscarriage, preeclampsia, placental abruption, preterm delivery, postpartum hemorrhage

IUGR, suboptimal neurodevelopmental outcome

Substance abuse

Tobacco: Perinatal death, spontaneous abortion, stillbirth, abruption placentae, placenta previa, premature delivery Cocaine: Abruption placentae, spontaneous abortion, premature rupture of membranes

Tobacco: Growth restriction, increased risk of SIDS and respiratory difficulties Cocaine: IUGR, adverse neurodevelopmental outcomes

SONOGRAPHIC AND FETAL ECHOCARDIOGRAPHIC ASSESSMENT Antenatal Ultrasonography

NE ON AT OLO GY

• Indications: • Dating pregnancy: If no reliable dating is available, discrepancy between uterine size and date (suspected IUGR; suspected large-for-dates pregnancies) • Fetal and placental location: Confirm pregnancy, suspected missed abortion, suspected ectopic pregnancy, suspected placenta previa, before prenatal diagnostic or interventional procedures • Survey of fetal anatomy: History of malformation, advanced maternal age, diabetic pregnancy, polyhydramnios and oligohydramnios, abnormal presentation, exposure to teratogens, abnormal maternal serum screen • Fetal anomalies detected by antenatal US • CNS: Fetal ventriculomegaly, meningomyelocele and type II Chiari malformation, anencephaly, encephalocele, Dandy-Walker cyst, choroid plexus cyst, holoprosencephaly • Spine: Diastematomyelia, sacrococcygeal teratoma • Head and neck: Gross craniofacial abnormalities, cystic hygroma, increased nuchal translucency, teratoma and lymphangioma • Thorax and heart: Intrathoracic mass (cystic adenomatoid malformation, pulmonary sequestration and diaphragmatic eventration), diaphragm hernia, laryngeal atresia, CHAOS, cardiac malformations and tumors • GI tract: Esophageal atresia and tracheoesophageal fistula, bowel obstruction, anterior abdominal wall defects, diaphragmatic hernia, choledochal cyst, heterotaxy • GU tract: Pelviectasis, hydronephrosis, renal agenesis, multicystic kidneys, renal dysplasia or duplication, lower urinary tract obstructions, ambiguous genitalia • Musculoskeletal system: Skeletal dysplasias, arthrogryposis, phocomelia

358

Neonatology

Fetal Echocardiography • Specific familial, fetal, and maternal indications for prenatal fetal echocardiograms exist (J Am Soc Echocardiogr 2004;17(7):803).

SERUM SCREENING FOR FETAL CHROMOSOMAL ABNORMALITIES CHANGES OBSERVED IN ANEUPLOIDIES AND OTHER FETAL ANOMALIES Serum Marker

Trisomy 21

Trisomy 13

Trisomy 18

Neural Tube Defects

↑

Second Trimester AFP

↓

Normal

↓

hCG

↑

Normal

↓

∗

Unconjugated estriol

↓

Normal

↓

∗

Inhibin-A

↑

Normal

∗

∗

PAPP-A

↓

∗

↓

∗

hCG

↑

∗

↓

∗

First Trimester

∗Value not typically used for estimating the risk of this disorder. D, decreased; I, increased; N, no change; N/A, not applicable.

• US finding of aneuploidy • Nuchal measurements: Nuchal translucency at 11–14 wk (sagittal view) and nuchal fold at 15–20 wk (axial view); the size of fluid collection at the back of a fetus’s neck is increased in several chromosomal abnormalities. • Short femur and/or humerus. • Absent nasal bone. • Congenital heart defects. • Other findings are more commonly seen in aneuploidy but are neither sensitive or specific: Hydrops, major malformation (omphalocele, holoprosencephaly, CDH, ONTD), echogenic bowel, intracardiac echogenic focus, dilated renal pelvis.

PRENATAL GENETIC DIAGNOSIS Amniocentesis • Typically performed at 16–18 wk GA. • Success rate of single procedure, >95%; success rate of establishing cell cultures from amniotic fluid cells, >99%. • Cytogenetic analysis can be completed 7–12 d after procedure. • Complications: Chorioamnionitis (1.6 mmol/L) • Decrease Ca:Phos to 0.5:1–0.8:1. • If iCa is >1.8 mmol/L, remove Ca from TPN, and discontinue or decrease phosphorus intake by 50%. • Measure iCa every 12 h until levels are back to normal. • Resume IV calcium when iCa is below 1.45 mmol/L. • Symptomatic hypercalcemia • Volume expansion if cardiac function is normal (can give 10–20 mL/kg of normal saline over 15–30 min). • Furosemide may be given to promote calciuria. • Discontinue thiazide diuretics (if applicable). • Inorganic phosphate may lower serum calcium in hypophosphatemic patients. • Glucocorticoids are effective in hypervitaminosis A and D and in subcutaneous fat necrosis; they are ineffective in hyperparathyroidism. • In older children and adults, calcitonin has been used as an adjunctive therapy; very little data on the use of calcitonin in neonates exist. • In severe cases, parathyroidectomy with autologous reimplantation may be helpful.

NEONATAL HYPOCALCEMIA Definition • In infants >1500 g, hypocalcemia is defined as iCa concentration 5–7 d to maintain normoglycemia will require more extensive evaluation and may require pharmacologic interventions. An endocrine consult should be considered at that time. The following laboratory studies should be considered (see the table below).

WORKUP OF PERSISTENT NEONATAL HYPOGLYCEMIA When to Obtain Test

Laboratory Study

During period of hypoglycemia

• Insulin • Glucose (to calculate insulin:glucose ratio; a value >0.30 indicates a nonhyperinsulinemic cause) • Cortisol • Glucagon • Growth hormone • Serum and/or urine ketones (should be absent in cases of hyperinsulinism) • Lactate

At any time

• Fatty acid profile • Thyroid function studies (TSH, T4) • Uric acid • Plasma amino acids • Urine organic acids • Urine-reducing substances • Abdominal imaging

• Pharmacologic therapy may be required for persistent hypoglycemia: intramuscular glucagon (assumes the infant has adequate glycogen stores), diazoxide (for hyperinsulinism). • Surgical intervention with subtotal pancreatectomy may be required for infants with insulin-producing tumors.

HYPERNATREMIA Definition • Serum sodium >150 mmol/L

Etiology • Increased insensible water losses • Inadequate water intake

Endocrinology

407

• Excess sodium administration • Following administration of sodium bicarbonate during resuscitation • Increased free water losses from the urinary tract

APPROACH TO THE NEONATE WITH HYPERNATREMIA BASED ON VOLUME STATUS ECF Volume Status

Possible Etiologies

Findings

Treatment

Hypovolemia or Euvolemia

• Increased renal and insensible free water loss • Diabetes insipidus (DI; central or nephrogenic)

• Weight loss • Tachycardia • Hypotension • Metabolic acidosis • Polyuria if DI; eventual ↓ in UOP if not DI

• Increase free water administration to decrease serum sodium at a rate no greater than 1 mEq/kg/h. • May need to use intranasal desmopressin for central DI. • Note: Correction of hypernatremia should be done over 48 h to decrease the propensity of cerebral edema.

Hypervolemia

• Excessive isotonic or hypertonic fluid

• Weight gain with edema • Normal heart rate, blood pressure, and urine output, with an elevated FENa

• Restrict sodium intake

HYPONATREMIA Definition • Serum sodium 120 mEq/L • With SIADH: • If neurologic symptoms decreased UOP and are present: increased urine • Furosemide (1 mg/ osmolarity kg q6h) with replace• NOTE: Diagnosis ment of urinary of SIADH assumes sodium losses with no volume-related hypertonic saline (3%) reason for ADH • Return to simple free release. water restriction once serum sodium >120 mEq/L and neurologic signs improve

Hypervolemic

• Sepsis with decreased cardiac output • Necrotizing enterocolitis • Heart failure • Impaired lymphatic drainage • Neuromuscular blockade

• Weight gain with • Treatment of underlying edema disorder • Decreased UOP • Restrict free water as clinically allowed with • For infants with some degree of sodium mature renal function, ↑ BUN, ↑ urine restriction SG, and ↓ FENa

Available at www.AccessPediatrics.com • Disorders of sexual differentiation • Congenital hypothyroidism • Infant of diabetic mother • Disorders of magnesium

Treatment

C H A P T E R 35

Gastroenterology DELAYED PASSAGE OF MECONIUM • The delayed passage of meconium may be the result of a mechanical or functional bowel obstruction. • Virtually all infants, term and preterm, will have passed meconium by 48 h of age. • The evaluation of any infant with delayed passage of meconium should begin with a thorough physical examination and history. • For infants who are unstable, the infant must first be stabilized before the investigation begins as to the cause of delayed stooling.

DIFFERENTIAL DIAGNOSIS, EVALUATION, AND MANAGEMENT OF DELAYED PASSAGE OF MECONIUM Diagnosis

Management

Anorectal abnormality (imperforate anus, anal stenosis)

Imperforate anus should be evident on physical examination Anal stenosis may be evident on physical examination

Surgical consult Repogle to low intermittent suction IV fluids Remember that high anal atresias may be associated with GU abnormalities

Meconium plug

Contrast enema shows the meconium plug with a normal-caliber colon

Enema is usually therapeutic If abnormal stooling continues, consider diagnosis of Hirschsprung’s disease and rectal biopsy

Meconium ileus

Contrast enema reveals a microcolon from ileal obstruction with stool

Enema can be therapeutic but may need surgical intervention

Hirschsprung’s disease

Contrast enema shows a distally narrowed segment (aganglionic) leading to a dilated proximal segment (normal colon) If suspected, rectal biopsy will provide the definitive diagnosis

Surgical consult Repogle to low intermittent suction IV fluids Diverting colostomy is standard; end-to-end anastomosis if affected segment is very short

Ileal atresia

Contrast enema shows no reflux of contrast into the terminal ileum

Surgical consult Repogle to low intermittent suction IV fluids

Malrotation

Infants usually present with bilious emesis Upper GI or contrast enema shows a malpositioned cecum

Surgical consult Repogle to low intermittent suction IV fluids


409

NE ON AT OLO GY

Cause

410

Neonatology

Cause

Diagnosis

Management

Volvulus

Surgical emergency because the ischemic gut may progress to frank necrosis Plain film of the abdomen reveals a massively dilated proximal colon Contrast enema reveals a midtransverse colon obstruction

Emergent surgical consult Repogle to low intermittent suction IV fluids

Ileus

May be secondary to a number of factors: • Sepsis • NEC • Hypokalemia • Hypothyroidism • Hypermagnesemia • Narcotic analgesia therapy

Bowel rest IV fluids Treatment of underlying condition causing the ileus

GASTROESOPHAGEAL REFLUX DISEASE (GERD) • GER is defined as passage of gastric contents into the esophagus. • GER is very common in term and preterm neonates. • A very large majority of infants exhibit no signs of clinical compromise and hence do not warrant specific treatment(GER) vs. infants who experience feeding difficulties, poor weight gain, failure to thrive, etc as a direct result of reflux (GERD). • For infants who have persistent “spitting up,” care must be taken to ensure that there is no true underlying pathology as a cause. • Apnea and GER are common occurrences in premature infants, but studies have shown no temporal relationship between the two.

Risk Factors • Congenital diaphragmatic hernia • Esophageal atresia repair • Gastroschisis • Omphalocele

• Short bowel syndrome (SBS) • Bronchopulmonary dysplasia • Neurologic deficits

Differential Diagnosis • Differential diagnosis for GER should include: • GI tract anatomic abnormalities: Esophageal webs or stenosis, duodenal web or stenosis, malrotation, gastric outlet obstruction

• Inborn errors of metabolism • Renal dysfunction • Neurologic deficits • Bronchopulmonary dysplasia

Clinical Presentation • Dysphagia • Odynophagia • Arching of the back with feeds

• Irritability • Failure to thrive • Hematemesis

Management • Initial management should be aimed at nonpharmacologic maneuvers (eg, GERD precautions): • Positioning of the infant with the head of the bed elevated. • Changing the rate of feeding (slow rate of bolus feeds to 60-90 minutes, feed less volume more frequently).

Gastroenterology

411

• Consider thickening feeds with commercially available thickeners. • Discontinuation of caffeine for apnea of prematurity may ameliorate symptoms of GER. • Pharmacologic therapy can be used if the above measures fail, but data on efficacy in this patient population are very limited. • Use of H2 blockers or PPIs to decrease gastric acid release have been reported with mixed results • Use of promotility agents is not advocated because of potentially serious side effects (eg, Parkinsonian reaction and tardive dyskinesia with the use of metoclopramide, may not be reversible). • Infants with severe symptoms as above have GERD. • Infants with GERD often have true esophagitis or gastritis and have been shown to benefit from gastric acid reduction. • Surgical intervention with fundoplication may be considered for infants who fail to respond to pharmacologic therapy and continue to display signs of GERD.

INDIRECT (UNCONJUGATED) HYPERBILIRUBINEMIA • 25%–50% of term neonates and a larger percentage of preterm neonates will develop clinical jaundice. • Clinical exam is not a reliable tool to estimate serum bilirubin level. • The major source of bilirubin is from the breakdown of hemoglobin in the RES. • Bilirubin is also produced from other heme-containing proteins in the peripheral tissues (eg, cytochromes, myoglobin) • The majority of fetal unconjugated bilirubin is cleared via the placenta to the maternal circulation.

RISK FACTORS FOR INDIRECT (UNCONJUGATED) HYPERBILIRUBINEMIA Major Risk Factors

Minor Risk Factors

• Predischarge TSB or TcB in high-risk zone • Jaundice in the first 24 h • Known hemolytic disease • GA 35–36 wk • Older sibling received phototherapy • Cephalhematoma or significant bruising • Exclusive breastfeeding (especially with poor feeding or excessive weight loss) • East Asian race

• Predischarge TSB or TcB in high-intermediaterisk zone • GA 37–38 wk • Jaundice observed before discharge • Older sibling with h/o jaundice • Macrosomic IDM • Maternal age 72 h

Physiologic Unconjugated Hyperbilirubinemia

NE ON AT OLO GY

• Generally, whereas bilirubin peaks in term neonates at 3–4 d, it peaks at 4–5 d in preterm neonates. • Physiologic hyperbilirubinemia occurs for several reasons: • Increased bilirubin production caused by decreased RBC survival in the neonate compared with adults • Increased enterohepatic recirculation caused by ↑ levels of β-glucuronidase and decreased gut motility • Decreased hepatic ligandin leading to decreased bilirubin uptake in the liver • Decreased UDPG-T activity leading to decreased conjugation of bilirubin • Decreased hepatic excretion

412

Neonatology

NONPHYSIOLOGIC (PATHOLOGIC) UNCONJUGATED HYPERBILIRUBINEMIA CAUSES OF NONPHYSIOLOGIC (PATHOLOGIC) UNCONJUGATED HYPERBILIRUBINEMIA Cause

Examples and Points to Consider

Hemolysis

• Intrinsic RBC membrane defects (elliptocytosis, spherocytosis) • ABO, Rh, or minor blood group incompatibility • Increased production of bilirubin caused by accelerated breakdown of RBCs

Polycythemia

• Increased bilirubin load caused by increased RBC mass • See Chapter 37 chapter for further details

Bleeding into body cavity

• Sequestered blood turnover contributes to bilirubin load • May be seen with bleeding into any body cavity (cephalhematoma, GI bleeding, pulmonary hemorrhage)

Conjugation defects

• Crigler-Najjar syndrome (types I and II; caused by relative deficiencies in UDP glucuronyl transferase) • Gilbert syndrome (caused by mild form of UDP glucuronyl transferase deficiency) • Lucey-Driscoll syndrome (caused by an undefined maternal hormone that interferes with bilirubin conjugation)

Breast milk jaundice

• Jaundice that appears around 5–7 d of life, peaks later at 10–15 d, and declines over several weeks • Caused by an undefined factor in breast milk that promotes intestinal re-absorption of bilirubin • Cessation of breast milk for 24–48 h causes a rapid decrease in serum bilirubin level

Breastfeeding jaundice

• Early onset jaundice caused by poor caloric intake and relative dehydration • Resolves as breastfeeding improves and milk supply increases

Metabolic disorders

• Galactosemia, hypothyroidism, maternal DM

Increased enterohepatic recirculation

• From any cause that delays transit through the gut (bowel obstruction at any level, CF, ileus from any cause)

Displacement of bilirubin from albumin

• Drugs (aspirin, sulfonamides, ceftriaxone) • Increased fatty acid concentration • Acidosis • Hypothermia

Evaluation • Do not delay therapy while awaiting results of laboratory tests. • Obtain total and direct bilirubin levels (most data correlating TSB with incidence of kernicterus are based on capillary samples of TSB). • CBC with reticulocyte count. • Blood type and Rh of mother and infant with direct Coombs test. • Peripheral blood smear. • Serum albumin. • Adjunct tests such as urine for reducing substances, metabolic tests, thyroid function, blood and urine cultures, imaging studies (cranial US for bleeding, abdominal radiographs for evidence of obstruction) if the index of suspicion is high. Management • See nomograms below for the American Academy of Pediatrics’ guidelines for phototherapy and for exchange transfusion in infants (≥35 wk gestation). • Standard phototherapy for infants whose TSB is not approaching exchange transfusion level may be used as a first-line therapy.

Gastroenterology 25

428

20

342 95th percentile

High risk zone 15

te r

edia

h

Hig

rm inte

Low

10

isk

te r

dia rme

e zon

257

e zon isk

μmol/L

Total Serum Bilirubin (mg/dL)

413

inte

171

Low risk zone 5

85

0

0 0

12

24

36

48

60 72 84 96 Postnatal Age (hours)

108

120

132

144

Figure 35-2 Bhutani Nomogram: Nomogram for designation of risk (likelihood) of a subsequent bilirubin level exceeding the 95th percentile, based on the hour-specific serum bilirubin value, for well newborns at least 36 wk gestation and 2000 g at birth, or 35 wk gestation and 2500 g at birth. Note: These values are NOT to be used to represent the natural history of unconjugated neonatal hyperbilirubinemia. (Reproduced with permission from Hay WW JR, Levin MJ, Sodheimer JM, Deterding RR: Current Diagnosis & Treatment: Pediatrics, 19th ed. New York: McGraw-Hill. Available at http://www.accessmedicine.com. Copyright © The McGraw-Hill Companies, Inc. All rights reserved.) For more information: Pediatrics 2004;114(1):297 and Pediatrics 1999;103:6.

25

428

20

342

15

257

10

171

5

μmol/L

Total Serum Bilirubin (mg/dL)

• Irradiance should be >18 μW/cm2 per nm. • As much of the infant’s skin surface area as possible should be exposed to the phototherapy light. • Intensive phototherapy should be used for infants whose TSB is approaching exchange transfusion level. • This involves overhead bili-lights combined with a bili-blanket. • Overhead lights should be positioned to deliver an irradiance of >30 μW/cm2 per nm.

85

Infants at lower risk (≥38 wk and well) Infants at medium risk (≥38 wk + risk factors or 35–37 6/7 wk and well)

0

Infants at higher risk (35–37 6/7 wk + risk factors)

Birth

24 h

24 h

72 h

96 h

5 days

6 days

0 7 days

Age

Figure 35-3 Guidelines for phototherapy in hospitalized infants ≥ 35 weeks’ gestation. (Reproduced with permission from Hay WW JR, Levin MJ, Sodheimer JM, Deterding RR: Current Diagnosis & Treatment: Pediatrics, 19th ed. New York: McGraw-Hill. Available at http://www.accessmedicine.com. Copyright © The McGraw-Hill Companies, Inc. All rights reserved.) For more information: Pediatrics 2004;114(1):297.

NE ON AT OLO GY

• Use total bilirubin, do not subtract direct reacting or conjugated bilirubin. • Risk factors = isoimmune hemolytic disease, G6PD deficiency, asphyxia, significant lethargy, temperature instability, sepsis, acidosis, or albumin < 3.0 g/dL (if measured). • For well infants 35–37 6/7 wk can adjust TSB levels for intervention around the medium risk line. It is an option to intervene at lower TSB levels for intants closer to 35 wks and at higher TSB levels for those closer to 37 6/7 wk. • It is an option to provide conventional phototherapy in hospital or at home at TSB levels 2–3 mg/dL (35–50 mmol/L) below those shown but home phototherapy should not be used in any infant with risk factors.

414

Neonatology

• As much of the infant’s skin surface area as possible should be exposed to the phototherapy light. • IVIG administered to infants with isoimmune hemolysis leading to hyperbilirubinemia has been shown to decrease the need for exchange transfusion. • Administer IVIG if TSB continues to increase in the presence of known isoimmune hemolysis despite intensive phototherapy or if TSB is within 2–3 mg/dL of the exchange transfusion level. • The dose is 0.5–1 g/kg over 2 h; the dose may be repeated in 12 h if needed. • Exchange transfusion is pursued for infants according to the figure below. (See Chapter 37 for guidelines on how to perform an exchange transfusion.) • Scant data exist to provide guidelines for the treatment of indirect hyperbilirubinemia in premature infants 90% of infants who develop NEC have received at least one enteral feeding. • Hyperosmolar formula or medications alter mucosal permeability and cause direct injury. • Breast milk significantly lowers the risk of NEC. Breast milk has been shown to decrease the incidence of NEC. • Immunoprotective factors are lacking in commercial formula. • All other interventions at this time remain experimental (use of immunoglobulins, prophylactic enteral antibiotics, probiotics). • Asphyxia or hypotension: Lead to low perfusion of mesenteric bed, causing intestinal ischemia and mucosal injury. • Hyperviscosity syndromes. • Exchange transfusions. • Use of H2 blockers. • PAF and other inflammatory mediators. • Clinical evidence for feeding volume and timing of initiation of enteral feeding remain controversial.

CLINICAL PRESENTATION OF NECROTIZING ENTEROCOLITIS Systemic Signs and Symptoms Lethargy Apnea Bradycardia Respiratory distress Irritability Feeding intolerance Hypotension Temperature instability Acidosis Oliguria Coagulopathy

Poor perfusion Bloody stools Abdominal distension Abdominal tenderness Gastric residuals Ileus Abdominal wall erythema Localized abdominal mass Ascites Vomiting (bloody or bilious)

STAGING OF NECROTIZING ENTEROCOLITIS Stage

Systemic Signs

Abdominal Signs

Radiographic Signs

Stage 1: Suspected NEC

• Apnea • Lethargy • Bradycardia • Temp instability

• Gastric residuals • Feeding intolerance • Guaiac-positive stools

• Normal or nonspecific


Gastroenterology

417

Stage

Systemic Signs

Abdominal Signs

Radiographic Signs

Stage IIA: Mild NEC

• As in Stage 1

• Abdominal distension • Abdominal tenderness • Absent bowel sounds • Bloody stools

• Ileus • Dilated loops of bowel • Focal pneumatosis intestinalis

As in Stage 1, plus:

• Further abdominal distension • Abdominal tenderness • Abdominal wall edema • Abdominal mass

• Extensive pneumatosis intestinalis • Ascites • ± Portal venous gas

Stage IIB: Moderate NEC

• Mild acidosis • Thrombocytopenia

Stage IIIA: Advanced NEC

• Mixed acidosis • Need for mechanical ventilation • Hypotension • Oliguria • Neutropenia • Coagulopathy

• Worsening abdominal wall edema • Worsening abdominal wall tenderness • Worsening abdominal wall discoloration (erythema or ecchymotic changes)

• Prominent ascites • Paucity of bowel gas • Sentinel loop of bowel

Stage IIIB: Advanced NEC

• Generalized edema • Intractable hypotension • Shock • DIC • Electrolyte abnormalities

• Tight abdominal distension • Significant discoloration of abdomen

• Absent bowel gas • Pneumoperitoneum

Differential Diagnosis • Ileus associated with infection (sepsis) • Malrotation with obstruction or midgut volvulus • Intussusception • Perforation (gastric or isolated small bowel) • Mesenteric vessel malformations leading to hypoperfusion or thrombosis • Infectious or allergic enterocolitis • Severe forms of metabolic disease (eg, galactosemia may produce vomiting, abdominal distension, hypotension, shock)

Diagnosis

NE ON AT OLO GY

• Clinical examination: Systemic and abdominal signs/symptoms as outlined above • Laboratory studies: • CBC • Culture of blood, urine, CSF, ± stool • Blood gas measurement (preferably arterial) • Serum electrolytes • BUN/creatinine • Radiographic studies • Flat plate radiograph of abdomen • Add a left-lateral decubitus (ie, left side down) film of abdomen if pneumoperitoneum is suspected (bowel perforation commonly occurs within 48-72 h of symptom onset)

418

Neonatology

MANAGEMENT OF INFANTS WITH NECROTIZING ENTEROCOLITIS Stage I

Stages IIA and IIB

Stages IIIA and IIIB

• Make NPO • Decompress bowel (place repogle tube to low intermittent suction) • Continuous cardiorespiratory monitoring • Controversial: Removal of umbilical catheters and placement of peripherally inserted venous and arterial lines • Broad-spectrum antibiotics (gentamicin, and ampicillin or vancomycin; clindamycin may be added if perforation or bowel necrosis is suspected) • Monitor for pathologic bleeding • Strict monitoring of “ins and outs” • Remove potassium from all fluids • Cultures as outlined above • Other labs listed above can be followed serially as indicated • Serial abdominal plain films ± decubitus views to evaluate for free air Q6–12 h for the first 48–72 h; then as needed

• All stage I management plus: • Constitute TPN to deliver 100–120 kcal/kg/d • Adjust fluids to allow for possible frequent transfusion of blood products, third-space losses, and renal failure • Respiratory support as required • Cardiovascular support as required with pressors (eg, dopamine) • Strongly consider obtaining a surgical consultation

• All stage I and II management plus: • Refractory hypotension may become an issue; will need support with pressors, intravascular volume expansion with blood products or crystalloid solutions • Common findings in this stage include severe thrombocytopenia, DIC, leukopenia or neutropenia • Surgical intervention may be necessary at this stage

Surgical Management • Surgical options include exploratory laparotomy (with resection of nonviable bowel and creation of an enterostomy) or placement of peritoneal drains. • Relative indications for surgical intervention: • Bowel perforation and pneumoperitoneum • Intractable metabolic acidosis • Severe abdominal distension leading to inability to ventilate • Abdominal wall erythema • Fixed, sentinel loop on abdominal radiographs (suggesting a segment of gangrenous bowel)

Prognosis • Infants with NEC who sustain an intestinal perforation have mortality rates of ≤40%. • Stenoses or strictures of the small or large bowel may develop, leading to symptoms of obstruction; contrast enema is usually diagnostic. • Infants who develop NEC that requires surgical intervention have higher risk of serious sequelae, including increased morbidity and mortality, hepatic disease associated with prolonged parenteral nutrition, and developmental delay

DIRECT (CONJUGATED) HYPERBILIRUBINEMIA • DH is a sign of hepatobiliary dysfunction. • An absolute level of >2.0 mg/dL or a value that is >20% of total serum bilirubin (TSB) warrants further investigation. • Consulting the GI or liver service would be appropriate at any point during the workup.

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ETIOLOGIES OF DIRECT (CONJUGATED) HYPERBILIRUBINEMIA Pathophysiology

Examples

Extrahepatic biliary disease

• Biliary atresia • Choledochal cyst

Intrahepatic biliary disease

• Paucity of bile ducts (syndromic form is Alagille’s syndrome) • Progressive intrahepatic cholestasis • Inspissated bile

Hepatocellular disease

• α1-Antitrypsin (A1AT) deficiency • CF • Tyrosinemia • Hereditary fructose intolerance (HFI) • Galactosemia • Rotor and Dubin-Johnson syndromes • TPN-associated cholestasis (TPNAC) • Other metabolic or genetic causes of conjugated hyperbilirubinemia • Infectious causes • Neonatal hemochromatosis • Hypoperfusion state • Infants on ECMO

Diagnosis • Infants who present with cholestasis may be evaluated according to the guidelines shown in the following table. The workup can be tailored to specific diagnoses based on clinical presentation and history.

EVALUATION OF DIRECT (CONJUGATED) HYPERBILIRUBINEMIA Rationale for Study

Diagnostic Studies US of liver or biliary tree

Evaluate liver function

ALT, AST, Alk Phos, GGT, unconjugated and conjugated bilirubin, albumin

Evaluate liver synthetic capacity

Glucose, PT, PTT

Evaluate for viral hepatitis

Hepatitis panel, EBV, CMV, adenovirus, enteroviruses, parvovirus

Evaluate for genetic or metabolic causes

A1AT with Pi phenotype, serum and urine amino acid profile, urine organic acid profile, ammonia, urine succinylacetone, urine ketones, serum lactate and pyruvate, serum ferritin, urine reducing substances, urinalysis, echocardiography (suspicious for cardiac disease)

Evaluate for CF

Genetic testing or sweat testing

Evaluate for hemolytic diseases

Peripheral blood smear, blood typing of infant and mother, Coombs test

Evaluate for hepatic excretory capacity

HIDA scan Liver biopsy

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Evaluate for anatomic causes

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Management • The primary treatment should be aimed at the underlying cause of cholestasis. Adjunctive therapies are listed in the table below.

ADJUNCTIVE THERAPIES FOR DIRECT (CONJUGATED) HYPERBILIRUBINEMIA Adjunct Therapy

Purpose

Enteral feeding

• Establishment of enteral feeds to the maximum that the patient will tolerate may help ameliorate cholestasis related to TPNAC. • Fortified human milk or standard premature formula is appropriate for premature neonates with cholestasis. • In infants with significant cholestasis, fat absorption may be impaired, and formulas (eg, Pregestimil or equivalent) containing a majority of fats as MCTs may be helpful in aiding fat absorption.

Ursodeoxycholic acid (UDCA)

• UDCA is a potent choleretic, increasing bile flow and hence helping to improve cholestasis. • Its efficacy in neonatal cholestasis is not proven. • If a bile acid synthetic defect is suspected, UDCA should be held until that workup is completed.

Fat-soluble vitamins

• TPN should provide adequate amounts of vitamins A, D, and E. • Vitamin K should be supplemented if bleeding occurs or PT is prolonged. • When full enteral feeds are achieved, consider giving vitamin E supplement plus BID doses of Poly-Vi-Sol ± PO vitamin K supplement. • If infant is on full enteral feeds but still with cholestasis, consider measuring serum levels of vitamins A, D, and E every 2–3 mo.

Copper and manganese

• Cu and Mn are excreted in bile. • In infants with cholestasis, Cu and Mn may accumulate in the liver and cause further hepatocellular injury. • As such, infants with conjugated bilirubin ≥4 mg/dL should have Cu and Mn limited in the TPN (eg, should be given “trace elements” twice weekly to prevent subsequent deficiencies of these nutrients).

Unproven therapies

• These include: changing the amino acid mixture, reducing or withholding lipid infusion, TPN “cycling”, or injection of cholecystokinin.

Available at www.AccessPediatrics.com • Upper GI bleeding • Lower GI bleeding • Short bowel syndrome

C H A P T E R 36

Genetics NONIMMUNE FETAL HYDROPS Definition • Presence of excess extracellular fluid in at least two fetal compartments (ascites, pleural effusion, pericardial effusion, skin edema, polyhydramnios) without any identifiable circulating antibody to red-cell antigens. • For a discussion on immune-mediated disease, see Chapter 37. • Prevalence is estimated at 1:1500-4000. • Highest prevalence in Southeast Asian population.

Diagnosis • Increased uterine size for dates • Decreased fetal movements • Generalized maternal edema (mirror syndrome)

• Polyhydramnios (AFI >24) • Placentomegaly

Management • Infants with nonimmune hydrops are at very high risk for fetal demise. • Intrauterine therapy is aimed at treating underlying causes (maternal digitalis therapy for fetal tachyarrhythmias); if this is not possible, the risks of intrauterine death versus premature delivery have to be weighed. • By organ system (see table below).

MANAGEMENT OF NONIMMUNE FETAL HYDROPS BY ORGAN SYSTEM System

Potential Difficulties/Management

Pulmonary

• Difficult intubation due to severe edema of head/neck/oropharynx. → Consider ENT or anesthesia support for intubation as needed. • Varying degrees of pulmonary hypoplasia due to large pleural effusions or other extrinsic in utero compression of lungs → May need emergent thoracentesis in delivery area to alleviate lung compression from large effusions. • Chest tube placement for rapidly reaccumulating pleural effusions.

Cardiovascular

• Hypotension should be treated with appropriate inotropic support. • Remember that most hydropic infants are euvolemic intravascularly, often with depressed cardiac function → Avoid large fluid shifts. • Pericardiocentesis may be necessary if cardiac tamponade from pericardial effusion is suspected → Should ideally be done under US guidance. • Arterial access is helpful to follow invasive blood pressures. • Echocardiogram should be obtained to evaluate for structural abnormalities as a cause of hydrops.

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System

Potential Difficulties/Management

Fluids and electrolytes

• Infants are total body fluid overloaded but are usually euvolemic (intravascular status). • Fluid intake should be based on a “dry” weight (ie, the 50th percentile for gestational age). • Fluids should be restricted (40–60 mL/kg/d) to avoid further fluid overload and to allow diuresis. • Vigilant attention to all fluid intake and output to maintain an adequate circulating volume. • Electrolyte losses should be replaced accordingly in the IV fluids. • Diuretics should be used very judiciously. • Abdominal US should be obtained to evaluate for an intra-abdominal mass/process that is compressing the IVC and other vascular structures.

Hematology

• For infants who are very anemic (hematocrit 7 d: 25 mg/kg/dose q8h • >37 wk PMA: • ≤7 d: 25 mg/kg/dose q12h • >7 d: 25 mg/kg/dose q6h Meningitis: • Use 50 mg/kg/dose at same interval as listed above

Empiric treatment for early- or late-onset (age >72 hrs) sepsis: • ≤7 d old: 150 mg/kg/dose IV q12h • >7 d old: 75 mg/kg/dose IV q6h Treatment >48 h: • Meningitis or no CSF obtained: 75 mg/kg/dose IV q6h • Sepsis without meningitis: 75 mg/kg/dose IV q12h Moderate CSF penetration


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Cleared primarily by the liver → monitor LFTs on treatment Can cause interstitial nephritis → monitor renal function weekly on treatment Can cause bone marrow suppression → monitor CBC weekly on therapy

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Antibiotic

Organisms Covered

Dose

Notes

Vancomycin

Aerobic and anaerobic Gram-positive cocci and bacilli Methicillin-resistant S. aureus (MRSA) Coagulase-negative staphylococci Clostridium difficile Bacillus spp. Ampicillin-resistant Enterococcus

• 7 d: 20 mg/kg/dose IV q18h • 30–37 wk PMA: • ≤7 d: 20 mg/kg/dose IV q18h • >7 d: 15 mg/kg/dose IV q12h • >37 wk PMA: • ≤7 d: 15 mg/kg/dose IV q12h • >7 d: 15 mg/kg/dose IV q8h • >44 wk PMA (meningitis): • 15 mg/kg/dose IV q6h

Only 10%–15% of serum concentration enters CSF. Optimal serum concentration: • Trough: 15–20 mcg/mL

Gentamicin, amikacin, tobramycin

Broad Gramnegative bacillus coverage Synergistic against S. aureus, GBS, L. monocytogenes, enterococci

Gentamicin • Indications: early- or late-onset sepsis (age >72 h); covers Gram-negative rods; use for synergy • 48 h (>2 doses), draw gentamicin trough before and peak level after the third dose. Monitor BUN/Cr: • Optimum levels: peak= 5–10 mcg/mL, trough = 7 d: 2.5 mg/kg/dose q12h • >37 wk PMA: • ≤7 d: 2.5 mg/kg/dose q12h • >7 d: 2.5 mg/kg/dose q8h • Optimum levels: peak = 8–10 mcg/mL; trough = 7 d: 15 mg/kg/dose q12h

CSF penetration depends on meningeal inflammation. Monitor peak and trough levels, as these antibiotics can cause nephrotoxicity and ototoxicity.


Infectious Diseases

Antibiotic

Organisms Covered

Dose

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Notes

• >37 wk PMA: • ≤7 d: 15 mg/kg/dose q12h • >7 d: 15 mg/kg/dose q8h • Optimum levels: peak = 15–40 mcg/mL; trough = 7 d: 10 mg/kg/dose q8h • >37 wk PMA: • 13 mg/kg/dose q8h

Poor CSF penetration Cleared by the liver → monitor LFTs while on therapy

First-generation cephalosporins (cefazolin, cephalexin)

Susceptible Staphylococcus, Streptococcus, and pneumococci

Cefazolin: • ≤7 d postnatal age: • 20 mg/kg/dose q12h • >7 d postnatal age: • 2000 g: 20 mg/kg/dose q8h

Poor CSF penetration

Secondgeneration cephalosporins (cefuroxime, cefoxitin, cefotetan, cefprozil)

Same as first generation plus Haemophilus influenzae E. coli Citrobacter Klebsiella Enterobacter cloacae

Improved activity over firstgeneration against β-lactamase– producing organisms Little data in neonates, so use is limited

Thirdgeneration cephalosporins (ceftriaxone, cefdinir, ceftazidime, cefotaxime, cefixime)

Gram-negative Cefotaxime: enterics • 2000 g: 50 mg/kg/dose q8–12h • >7 d postnatal age:

Ceftazidime can be used for adequate coverage of Pseudomonas aeruginosa. Ceftazidime, cefotaxime, and ceftriaxone all achieve good CSF penetration. Ceftriaxone displaces bilirubin from albumin, raising serum levels of free unconjugated bilirubin → not recommended for use in neonates except in treating gonococcal infection in nonjaundiced neonates. In jaundiced neonates, use cefotaxime.

• 1200–2000 g: 50 mg/kg/ dose q8h • >2000 g: 150–200 mg/kg/d divided q6–8h Ceftazidime: • 7 d postnatal age: • ≤2000 g: 50 mg/kg/d q24h • >2000 g: 50–75 mg/kg/d q24h • Gonococcal prophylaxis: • 25–50 mg/kg as a single dose (maximum: 125 mg/dose) • Gonococcal infection: • 25–50 mg/kg/d (maximum: 125 mg/dose) q24h for 7 d, up to 10–14 d if meningitis is documented Macrolides Gram-positive (azithromycin) bacteria MRSA Neisseria spp. T. pallidum Chlamydia trachomatis Bordetella pertussis

Infants 7 d postnatal age: • Weight 1200–2000 g: • Non-meningitis: 20 mg/kg/ dose q12h • Meningitis: 40 mg/kg/dose q12h • Weight >2000 g: • Non-meningitis: 20 mg/kg/ dose q8h • Meningitis: 40 mg/kg/ dose q8h

Use must be approved by infectious disease service

Use must be approved by infectious disease service Good CSF penetration Resistant organisms: Burkholderia cepacia, E. faecium, Stenotrophomonas maltophilia

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NEONATAL MENINGITIS OVERVIEW OF ETIOLOGY AND TREATMENT OF NEONATAL MENINGITIS Age of Infant

Most Common Etiologies

3 mo

S. pneumoniae, N. meningitidis, H. influenzae, above neonatal pathogens

Cefotaxime or ceftriaxone Add vancomycin if possible PCN-resistant S. pneumoniae until susceptibilities return

Same as above

Consider less common forms of neonatal meningitis if index of suspicion is high based on history or physical examination findings: viral (HSV (meningoencephalitis), enterovirus, VZV), tuberculosis, fungal, and noninfectious causes (eg, leukemic infiltrates).

Clinical Features • Neurologic: seizures, lethargy, irritability, decreased tone; full fontanelle • Fever (>38°C) or hypothermia (more common in preterms) • Respiratory distress: grunting, tachypnea, nasal flaring • Poor feeding • Diarrhea, vomiting • Apnea

Risk Factors • Premature rupture membranes, maternal fever/infection, fetal hypoxia, birth trauma, galactosemia (E. coli sepsis), low birth weight, preterm infants (100 WBC/μL, predominantly polymophonuclear cells evolving to lymphocytes.

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• Viral meningitis in neonates: picture may be similar but with a less dramatic pleocytosis. • Maternal investigation: If possible, send placenta for pathology and, if indicated, cultures/PCR. • MRI/CT: to identify focal areas of infection, infarction, hemorrhage, edema, hydrocephalus, or abscess formation. Consider with focal neurologic abnormalities, persistent infection, or clinical deterioration. • Eye exam may be helpful to evaluate for chorioretinitis.

CSF PROFILES FOR INFANTS WITH BIRTH WEIGHT 7 d–3 mo of life Mean time: 36 d

>3 mo of life

Commonly affected population

• All infants

• Predominantly term infants

• Predominantly neonates 80 mm Hg; DBP >50 mm Hg

DIFFERENTIAL DIAGNOSIS OF HYPERTENSION IN NEONATES System

Examples

Vascular

Renal artery or vein thrombosis Renal artery stenosis Aortic thrombosis Coarctation of aorta

Renal

Obstructive uropathy Renal failure Renal neoplasia (Wilms tumor) (continued on next page)

Nephrology System

Examples

Neurologic

Drug withdrawal Pain or agitation Increased ICP (very rare manifestation in neonates) Seizures

Endocrine

Congenital adrenal hyperplasia Hyperthyroidism Adrenal neoplasia (neuroblastoma, pheochromocytoma)

Medications

Pancuronium Corticosteroids Dopamine Vitamin D Epinephrine

Other

Bronchopulmonary dysplasia ECMO Fluid overload Idiopathic Spurious (improper BP cuff size)

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Evaluation • Physical exam: Check femoral pulses and examine the abdomen to evaluate for masses or enlarged kidneys. • Laboratory studies: Determine renal function with serum creatinine and BUN levels, urinalysis, serum electrolytes, and plasma renin activity. • Radiologic studies: Abdominal US, head US, echocardiogram. • Adjunct studies may be obtained based on clinical suspicion: Renal vessel angiography to evaluate for renal artery stenosis, renal scan to determine the extent of renal scarring or loss of function, renal biopsy (not routinely done for isolated hypertension), 24-h urine collection to determine catecholamine levels.

Management

Available at www.AccessPediatrics.com • Congenital syndromes and disorders with renal manifestations

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• Treat any underlying cause of hypertension (eg, fluid overload, pain or agitation, medications). • Management guidelines for hypertension in neonates are controversial. • For hypertension that is symptomatic (nonspecific cardiac findings of tachypnea, cardiomegaly, or heart failure or neurologic symptoms such as irritability, lethargy, or seizures), immediate treatment to lower BP is indicated. • Hydralazine has been used for control, titrating to the lowest effective doses. • Labetalol and nicardipine starting with lowest doses (limited use in neonates reported) • For moderate hypertension (asymptomatic) • Diuretics • Vasodilators (hydralazine, nicardipine) or β-blockade (labetalol, propranolol) • ACE inhibitors may be used but with caution because use may lead to oliguria, hyperkalemia, or renal failure. Bilateral renal artery stenosis must be excluded before use. Check serum electrolytes and creatinine within 24 h after initiation and dose escalations. • For mild hypertension, simple observation may all be that is needed. If hypertension does not improve or worsens, treat as above.

CHAPTER 40

Neurology NEONATAL SEIZURES ETIOLOGIES OF NEONATAL SEIZURES Underlying Defect

Points to Remember

Hypoxic–ischemic encephalopathy

• Leading cause of neonatal seizures in full-term infants. • This diagnosis should never be one of exclusion. • Data supporting this diagnosis (history of prolonged labor, perinatal depression, prolonged resuscitation) should be obtained and documented.

Focal ischemia/ infarction

• Second most common cause of neonatal seizures in full-term infants. • Most common presentation is right-sided clonic seizures due to infarction in the left middle cerebral artery territory. • Cerebral vein thromboses can lead to venous infarcts.

Intracranial hemorrhage

• In term infants, subarachnoid hemorrhage is more associated with subsequent seizure than subdural hemorrhage. • Infratentorial subdural hemorrhages require urgent evaluation due to risk of brainstem compression. • In preterm infants, intraventricular hemorrhage is the most common type of ICH (see below).

Infections of the CNS

• Can occur in utero (CMV, toxoplasmosis) or perinatally (herpes simplex, bacterial meningitis with GBS or Escherichia coli being most common). • Prognosis can be very grim.

Metabolic derangement

• Transient causes (hypoglycemia, hypocalcemia, hyponatremia); see Chapter 34 for further information. • Inborn errors of metabolism (pyridoxine dependency, nonketotic hyperglycinemia, urea cycle defects, glutaric aciduria (type II), maple syrup urine disease, organic acidurias, cofactor deficiencies, mitochondrial defects , Zellweger Syndrome).

Structural defects

• Defects of neuronal migration (heterotopias). • Defects of neuronal organization (polymicrogyria). • Cerebral malformation (holoprosencephaly). • Usually will display associated dysmorphic features on physical examination.

COMMON CAUSES OF NEONATAL SEIZURES BY AGE AT PRESENTATION Day of Life

Possible Causes

1

Hypoxic ischemic encephalopathy Infection Hypocalcemia

Maternal drug use Hypoglycemia

2–3

All of the above Inborn errors of metabolism Drug withdrawal

CNS malformation Hypernatremia Hyponatremia

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CLASSIFICATION OF NEONATAL SEIZURES Seizure Type

Clinical Manifestation

Subtle

Oral–buccal–lingual or ocular movements Autonomic dysfunction (Δ in HR, BP, SpO2)

Stereotypical stepping/swimming

Clonic

Rhythmic, slow jerking Facial, extremity, or axial involvement

Focal or generalized

Tonic

Sustained limb posturing Asymmetric position of trunk/neck

Focal or generalized

Rapid isolated jerks

Generalized, multifocal, or focal

Myoclonic

Limb/trunk involvement

NEONATAL SEIZURE MIMICS Seizure Mimic

Clinical Manifestation

Jitteriness

Spontaneous or provoked by stimulus Flexion/extension are equivalent Diminished by repositioning Abolished with containment

Benign neonatal sleep myoclonus

Bilateral or unilateral Synchronous or asynchronous Occurs during sleep Not due to a stimulus

Stimulus-evoked myoclonus

Focal or generalized Severe CNS dysfunction EEG may show cortical spike-wave discharge

Hyperekplexia (stiff-man syndrome)

Generalized stiffness Autosomal dominant and recessive forms Excessive startle responses to unexpected stimuli Excessive stiffness following startle response Benzodiazepines reduce symptoms

Management

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• Support respiratory and cardiovascular function (may require endotracheal intubation and mechanical ventilation). • Place on continuous cardiorespiratory monitoring. • Correct any known causes of seizures (see above). • If seizures continue after correction of transient metabolic derangements, load with phenobarbital (20 mg/kg IV); can be followed by repeat doses of 5–10 mg/kg IV to a total dose of 40 mg/kg IV, if needed. • If seizures continue, load with a benzodiazepine such as midazolam (0.1 mg/kg IV; repeat doses can be given to a total dose of 0.3 mg/kg IV). • If seizures continue, load with fosphenytoin 20 mg/kg. • If seizures continue, can consider loading with pyridoxine (100 mg/kg IV), preferably with EEG monitoring. Maintenance dosing can be 50-100 mg/dose once daily (orally) • Newer agents that are used in older children, adolescents, and adults may sometimes be given (in consultation with the neurology service), but no data exist on the safety and efficacy of these medications in neonates.

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INTRAVENTRICULAR HEMORRHAGE • Definition: an intracranial hemorrhage that originates in the paraventricular subependymal germinal matrix and may extend into the lateral ventricular system. • 50% of IVH occurs in the first 24 h after birth, and 90% by 7-10 days of life. • Incidence and severity are inversely proportional to gestational age. • Antenatal steroid administration is associated with a lower incidence. • Major risk factors: • Extreme prematurity • Need for assisted ventilation • Pneumothorax • Clinical presentation can be very diverse: • May be asymptomatic • May have nonspecific symptoms (bulging fontanelle, sudden drop in hematocrit, apnea/bradycardia, metabolic acidosis, seizures, change in level of consciousness) • May present with a catastrophic constellation of symptoms (profound hypotension, severe neurogenic pulmonary edema, rapid deterioration in neurologic exam, decerebrate posturing) • All infants with birth weight distal

Normal or ↑ DTRs

↓ or absent DTRs

↓ or absent DTRs

Normal DTRs

↓ DTRs

± Seizures

Fasciculations

± Fasciculations

No fasciculations

Central

Persistent primitive reflexes

Muscle

Sensation loss

• Diagnostic workup may include: • Cranial imaging (US, CT scan, MRI) • EEG • Genetics studies (karyotype, chromosome microarray, specific gene tests, or other specialized studies [eg, DNA methylation study for Prader–Willi] after consultation with genetics service) • TORCH screen if clinical suspicion and other stigmata of congenital infection • Metabolic workup • Molecular genetics (CTG repeats, deletions in SMN gene) • CK (levels tend to be high at birth and increase in the first 24 h; false-positive increase with acidosis)

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• Nerve conduction studies • Muscle biopsy

NEUROMUSCULAR DISORDERS NEUROMUSCULAR DISORDERS PRESENTING WITH NEONATAL HYPOTONIA Disorder

Genetics

Presentation

Prognosis

Congenital muscular dystrophy

AR, M=F

Difficulties with suck, swallow, and respirations; joint contractures

Normal intellect; severe disability

Central core disease

AD

Generalized weakness

Normal life span

Spinal muscular atrophy type 1 (Werdnig–Hoffmann disease)

AR; rarely AD

Absent DTRs, weak cry, poor suck; facial muscles spared

1–2 yr life expectancy

Hereditary motor and sensory neuropathy type III (Dejerine– Sottas disease)

AD; rarely AR

Generalized weakness, delayed motor milestones, ataxia, sensory loss, decreased DTRs

Variable disability; usually live to adulthood

Nemaline rod myopathy

AD or AR

Generalized weakness, weak respiratory muscles

Early death due to respiratory failure

Riley–Day syndrome

AR

Sweating with feeds, no response to painful stimuli, decreased DTRs

Few live beyond adolescence

Congenital fiber type disproportion myopathy

Unknown

Generalized weakness, delayed motor milestones, hip dislocation, contractures

Nonprogressive after 2 yr; normal life span

HYDROCEPHALUS Definition • Progressive dilation of the cerebral ventricular system caused by an imbalance in CSF production/absorption • Obstructive/noncommunicating: ventricles are dilated proximal to site of obstruction • Communicating: excessive secretion or inadequate absorption of CSF

CAUSES OF CONGENITAL AND ACQUIRED HYDROCEPHALUS Acquired Hydrocephalus

• Aqueductal stenosis • Neural tube defects (up to 80% of these infants have hydrocephalus) • Dandy–Walker malformation • Syndromes with vermian agenesis

• Posthemorrhagic (see “Intraventricular Hemorrhage” section) • Hydrocephalus ex vacuo from cerebral atrophy after severe insult • Postinfectious

• Vein of Galen malformation • Arachnoid cysts • Achondroplasia • Osteopetrosis

• Neoplasms of the CNS

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Congenital Hydrocephalus

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Presentation • Bulging or full fontanelle • Split sutures • Rapid head growth • Macrocephaly • Vomiting

• Apnea • Bradycardia • Irritability • Altered level of consciousness

Diagnosis • Many of the congenital causes of hydrocephalus may have accompanying physical findings (ie, cranial bruit for vein of Galen malformations). • Head US, CT, or MRI.

MANAGEMENT OF NEONATAL HYDROCEPHALUS Temporizing Measures

Permanent Measure

• Placement of a temporary reservoir or ventriculo-subgaleal shunt until more definitive CSF diversion can be performed (most common therapy) • Serial LPs (can be done for communicating hydrocephalus) • Medications to reduce CSF production (acetazolamide ± furosemide; may lead to significant metabolic derangements)

• Ventriculoperitoneal shunting

RETINOPATHY OF PREMATURITY (ROP) Diagnosis • Screening for ROP is by indirect ophthalmoscopic retinal examinations for all infants born at 34 wk GA on mechanical ventilation has an oxygen index (OI) > 25 on two separate measurements OI = ([Mean airway pressure x FiO2] / PaO2) x 100 • Consider ECMO for OI >40 on two separate measurements • ECMO has been used pre-, intra-, and postoperatively to improve survival in patients with poor pulmonary function • In severe cases, fetal ex utero intrapartum treatment (EXIT) to ECMO may be considered • In surgery, hernia is reduced by withdrawing viscera from the chest, and the diaphragm is either repaired primarily or with a patch • LHR 1.4 has near complete survival • Survival rates vary from 40%–79%, depending on case selection • Mortality is largely related to pulmonary hypoplasia, pulmonary hypertension, and CHD • Late complications: Recurrence of hernia, small bowel obstruction, GER, and thoracolumbar scoliosis

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Congenital Cystic Adenomatoid Malformation • If fetal surgery is not an option, maternal steroids have been reported to arrest the growth of fetal CCAMs • Those that do not self-resolve are resected later to prevent infection and malignant potential within the malformation • CXR should be taken after delivery and a CT scan of the chest should be done at 6–8 wk of age, even if the CXR is normal; any residual lesion is surgically resected, as there is a risk for malignancy.

Laryngomalacia Presentation • Most common cause of stridor in neonates and infants. • Intermittent, recurrent inspiratory stridor. • Stridor worsens with crying, feeding, supine positioning, agitation. • Stridor may improve with crying in very mild laryngomalacia. • May be associated with difficulty feeding, failure to thrive, dyspnea, tachypnea, apnea, cyanosis, pectus excavatum, and sleep apnea. If severe cases are untreated, cor pulmonale and pulmonary hypertension may develop. • 50%–100% association with GERD. • ~20% with neurologic compromise or multiple congenital anomalies. • ~20 % with second airway lesion.

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Course • Typically present within first 2 weeks of life. • 50% resolve by age 9 mo. • 75% resolve by age 18 mo. Evaluation and Management • Consider treatment for GERD with a PPI or an H2 receptor blocker. • Lansoprazole 0.3–3.3 mg/kg/day or ranitidine 2 mg/kg/dose PO Q8h. • Note that safety and efficacy data for use of these medication in neonates is very limited.

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• Routine referral to pediatric otolaryngologist if the patient has isolated, uncomplicated stridor. • Urgent evaluation to pediatric otolaryngologist for “severe symptoms”: Failure to thrive, recurrent hospitalization for dyspnea, apnea, cyanosis, pectus excavatum, severe reflux, cor pulmonale, pulmonary hypertension. • Flexible fiberoptic laryngoscopy by pediatric otolaryngologist. • Direct laryngoscopy and bronchoscopy if: • Complicated by “severe symptoms” above • Stridor or symptoms are out of proportion to the fiberoptic exam • History and exam suggest a second airway lesion • Surgical correction: Supraglottoplasty. • 20% of patients require surgical intervention for failure to resolve symptom or severe symptoms” listed above. • Performed with either surgical instruments or lasers. • Division of the aryepiglottic folds. • Trimming or lasering of redundant supraglottic mucosa. • 1–3 d postoperative hospitalization.

Available at www.AccessPediatrics.com • Obstructive lesions of the large bowel

Index Page numbers followed by f or t indicate figures or tables, respectively.

A AAT (α-1-antitrypsin) deficiency, 171t Ab screen, 205 Abbott powder formulas, 46–47t Abdominal pain, acute, 139–140 Abdominal reflex, 269t Abdominal wall defects, 471–472t Abducens nerve, 268t ABPA (allergic bronchopulmonary aspergillosis), 320t Abscess brain, 245t retropharyngeal, 136–137t skin characteristics, 114t, 233t incision and drainage, 32–33, 33t treatment, 114t Absence epilepsy, 278t AC (assist-control) ventilation, 87t, 375t ACE (angiotensin-converting enzyme) inhibitors, 73 Acetaminophen antidote, 345t dosing, 8–9t, 12t, 456 for migraine headache, 282t in neonates, 456 Acid–base disorders differential diagnosis, 164–165t, 166–167t, 166f in inborn errors of metabolism, 189f, 191t mixed, 168 neonatal, 189, 189f nomogram, 166f with partial compensation, 166–167t Acne, neonatal, 386t Acrodermatitis enteropathica, 391t Acropustulosis of infancy, 387t Acute chest crisis, in sickle cell disease, 200t Acute disseminated encephalomyelitis (ADEM), 284t Acute generalized exanthematous pustulosis (AGEP), 121t Acute infusion reaction, 290–291t Acute kidney injury (acute renal injury, acute renal failure) categories, 264 classification, 263 definition, 263 etiologies, 264 evaluation, 264–265 management, 265 in neonates, 445–446 prognosis, 265

Acute lymphoblastic leukemia (ALL) definition, 292 diagnosis, 293 epidemiology, 292 immunologic classification, 293t morphologic classification, 293t prognosis, 295 treatment, 293–294 Acute otitis media (AOM), 125–126 Acute postinfectious glomerulonephritis, 259t Acyclovir, 55t Acylcarnitine analysis, 193 ADEM (acute disseminated encephalomyelitis), 284t Admission orders (ADC VANDALISM), 1 Adolescents eating disorders in, 56–59, 57–58t emergency contraception for, 59 gynecologic examination, 51–52, 51t human papillomavirus infection in, 56 pediculosis pubis in, 56 pelvic inflammatory disease in, 55–56t scabies in, 56 sexually transmitted infections in, 53–54t vulvovaginal disorders in, 52–53t Adrenal hemorrhage, bilateral, 392t Adrenal insufficiency primary, 392–393 secondary, 393–294t Adrenergic toxidrome, 344t Adrenocortical dysgenesis, 392t AGEP (acute generalized exanthematous pustulosis), 121t Albumin 5%, 158t Albuterol adverse effects, 316t for anaphylaxis, 61t for asthma, 316t for status asthmaticus, 91t Alclometasone dipropionate, 124t Aldosterone synthase deficiency, 393t Alkylating agents, 298t ALL. See Acute lymphoblastic leukemia (ALL) Allergic bronchopulmonary aspergillosis (ABPA), 320t Allergic contact dermatitis, 111t Allergic rhinitis, 62–63t, 127 Allergic salute, 62 Allergic shiners, 62 Allergy testing, 60 Alloimmune thrombocytopenia, 426–427t α-1-antitrypsin (AAT) deficiency, 171t

479

480

Index

α-thalassemia, 199t ALTE (apparent life-threatening event), 323–324 Altered mental status, 141 Ambulatory care health supervision overview, 209–211t screening recommendations, 212t Amcinonide, 123t American Society of Anesthesiologists (ASA), physical status classification, 15t Amikacin, 224t, 438–439t Amino acid analysis, 193t Amino acid metabolic disorders, 191t Amino acid transport disorders, 191t Aminoglycosides, 224t Aminopenicillins, 218t Aminophylline, 92t Amitriptyline, 282t Amniocentesis, 358 Amoxicillin, 218t for acute otitis media, 126t for community-acquired pneumonia, 237t for infective endocarditis prophylaxis, 80t Amoxicillin–clavulanate, 219t for acute otitis media, 126t for community-acquired pneumonia, 237t Amphetamines, 341t, 342t Ampicillin, 218t, 437t Ampicillin–sulbactam, 219t AMPLE history, 15 Anal stenosis, 409t Anaphylaxis, 60–61, 61t, 121t Anemia of chronic disease, 197t definition, 196 diagnosis, 196, 197f, 198f iron-deficiency, 197t, 198–199 macrocytic, 197f microcytic, 197f morphologic classification, 197f neonatal, 431–433, 432t screening, 212t Aneuploidy, screening for, 354, 358, 358t Angioedema, 61–62, 121t Angiotensin-converting enzyme (ACE) inhibitors, 73 Anion gap metabolic acidosis, 168 Ankle fractures, 310t Ankle sprain, 310t Annular pancreas, 140 Anorectal abnormality, 409t Anorexia nervosa, 56–57, 57–58t Antiarrhythmics overview, 77t poisoning/overdose, 346t Antibiotics for acute otitis media, 125–126t aminoglycosides, 224t for bacterial tracheitis, 136t for bite wounds, 135t carbapenems, 221t cephalosporins, 220–221t

Antibiotics (cont.) in cystic fibrosis, 322–323t empiric choices for bacterial meningitis, 245t based on organism, 228f for community-acquired pneumonia, 237t, 238t for epiglottitis, 136t for fever without localizing signs, 130f, 131f fluoroquinolones, 224–225t lincosamides, 223t lipopeptides, 226t macrolides, 222t for mastoiditis, 127t monobactams, 221t for neonatal infections, 437–440t nitroimidazoles, 226t oxazolidinones, 223t penicillins, 218–219t for retropharyngeal abscess, 136t rifamycins, 226t for septic arthritis, 134t for sinusitis, 128 for skin and soft tissue infections, 234 sulfonamides, 225t tetracyclines, 223t vancomycin, 222t Anticholinergic toxidrome, 344t Anticoagulants, 205t Antiemetics, 292t Antiepileptics in increased intracranial pressure, 101 for migraine headache prophylaxis, 282t in pregnancy, neonatal effects, 399t, 423t for seizures, 279–280t Antihistamines for allergic rhinitis, 62t poisoning/overdose, 346t Antimetabolites, 298t Antipseudomonal penicillins, 219t Antithrombin (AT) replacement, 208 α-1-Antitrypsin (AAT) deficiency, 171t Antitumor antibiotics, 298t AOM (acute otitis media), 125–126 Apgar score, 349t Aplastic crisis, 200t Apnea, neonatal definition, 465 differential diagnosis, 468–469 evaluation, 468 incidence, 465 management, 469–470 monitoring, 468 Apophysitis, 301, 302 Apparent life-threatening event (ALTE), 323–324 Appendicitis, 139 Apple juice, 157t Aprepitant, 292t Argininemia, 194t Argininosuccinic aciduria, 194f, 194t

Index Arrhythmias, 75t, 76f, 77t Arsenic poisoning, antidote, 345t Arterial blood gases in congenital heart disease, 382 in mechanical ventilation, 89 normal values, 164t SI laboratory value conversion table, 8t Arterial switch operation, 81 Arthritis in cystic fibrosis, 320t differential diagnosis, 328f juvenile idiopathic, 329–330 synovial fluid evaluation in, 327t Ascites, 174 Asparaginase, 299t Aspergillosis, 227 Aspiration pneumonia, 235t, 237t, 238t Asplenia, secondary immunodeficiency in, 64t Assist-control ventilation (AC), 87t, 375t Asthma definition, 312 diagnosis, 313 exacerbation, 137f history and physical exam, 313t management assessment of control, 315t components, 313–314 initial therapy, 315t medications, 316t stepwise approach, 316–317t therapy adjustment, 315t modified predictive index, 312 pathophysiology, 312 severity classification, 314t Astrocytoma, 296–297t Athlete’s foot (tinea pedis), 118t Atopic dermatitis, 110–111 Atrial septal defect (ASD), 66 Atrial switch operation, 82, 82f Atrioventricular septal defect, 67, 67f, 81 Atrophy, 109 Auditory nerve, 268t Augmented betamethasone dipropionate, 123t Autoimmune diseases, secondary immunodeficiency in, 65t Autoimmune thrombocytopenia, 426t Avulsion fracture, 302, 308t Azalides, 222t Azithromycin, 222t for acute otitis media, 126 for chancroid, 54t for chlamydia, 54t for community-acquired pneumonia, 237t, 238t in cystic fibrosis, 322t for infective endocarditis prophylaxis, 80t for neonatal infections, 440t Aztreonam, 221t, 322t, 323t B Babinski sign/reflex, 269t, 272t Bacterial peritonitis, 174, 175t

481

Bacterial tracheitis, 136–137t Bacterial vaginosis, 52–53t Ballard score, 348t Barbiturates, 18, 18t Barium enema, pre-procedural diet, 4t Basal metabolic rate, 41t Behçet syndrome, 332t Benign childhood epilepsy with centrotemporal spikes, 278t Benign neonatal sleep myoclonus, 449t Benign Rolandic Epilepsy (BRE), 278t Benzodiazepines. See also specific drugs antidote, 345t overview, 17, 17t Berger (IgA) nephropathy, 259t Bernard-Soulier syndrome, 427t β-blockers as antiarrhythmics, 77t antidote, 345t for dilated cardiomyopathy, 73 for hyperthyroidism, 149 β-lactamase resistant penicillins, 219t β-lactamase susceptible penicillins, 218t β-thalassemia major, 199t β-thalassemia minor, 197t, 199t Betamethasone, equivalencies, 10t Betamethasone dipropionate, 123t Betamethasone valerate, 124t Bicarbonate, in fluid replacement, 154 Bilevel positive airway pressure (BiPAP), 85t Biliary cirrhosis, in cystic fibrosis, 319t Biophysical profile, 360–361t Birth weight, 3 Bisacodyl, 179 Bites, 134–135 Bitter almond odor, 341t Blalock-Taussig shunt, 81, 81f Blastomycosis, 227 Bleeding disorders differential diagnosis, 203t history, 204 laboratory evaluation, 204 treatment of acute bleeding in, 204t Bleomycin, 298t Blood component transfusion CMV-seronegative, 207 cryoprecipitate, 207 definitions, 205 granulocyte, 207 irradiated blood, 208 leukocyte-reduced blood, 207 in neonates, 433t, 434–436t packed red blood cells, 206t, 433t, 434t plasma, 207, 435t platelets, 206t, 207, 435t risks, 206 Blood gases in mechanical ventilation, 89 normal values, 164t SI laboratory value conversion table, 8t Blood pressure screening, 212t. See also Hypertension

482

Index

Blood transfusion. See also Blood component transfusion; Exchange transfusion for shock, 98 typing and cross-matching for, 430–431t Blood type, 205 Bloodstream infections central venous catheter-related, 247–249, 247t, 248f, 249t intravascular catheter-related, 246–247, 246t Body mass index (BMI), 49 Body surface area, 4 Bone marrow transplantation, 299–300 Bone scan, pre-procedural diet, 4t Boost, 156t Borrelia burgdorferi, 115t Brachial muscles, actions and nerve root supply, 272–273t Bradycardia, 76f Brain abscess, 245t Brain death, 287–288 Brainstem glioma, 296–297t Breast milk jaundice, 412t Breastfeeding, 35–36. See also Human milk Breastfeeding jaundice, 412t Bronchiolitis, 138–139 Bronchopulmonary dysplasia (BPD), 465–468t Bronchoscopy, pre-procedural diet, 4t Buckle (torus) fracture, 302 Bulimia nervosa, 57–58t Bulla, 109 Bullous congenital ichthyosiform erythroderma, 388t Bupivacaine, 12t Busulfan, 298t Buttock laceration, 31t C Caffeine, 466t, 469 Calcium conversion, 43t daily requirement, 43t ECG changes and, 77 for neonatal hypocalcemia, 401t in shock, 98 Calcium channel blockers, as antiarrhythmics, 77t Calcium deficiency, 152t Candida infections, 52–53t, 119t, 227, 390t Carbamazepine, 279t Carbamoyl-phosphatase synthetase (CPS) deficiency, 193t, 194f Carbapenems, 221t, 440t Carbenicillin, 219t Carbohydrates, in total parenteral nutrition, 42, 43t Carbon monoxide, antidote, 345t Carbon tetrachloride, antidote, 345t Carbuncle, 233t Cardiac ischemia, 75 Cardiogenic shock, 94t, 96t, 98 Cardiomyopathy dilated, 72–73, 72t in inborn errors of metabolism, 192 Caries, dental, 106–107

Carrot odor, 341t Casts, in urine, 257t Cat bites, 135t, 233t Cataracts, 192 Cefaclor, 220t Cefadroxil, 220t Cefamandole, 220t Cefazolin, 80t, 220t, 439t Cefdinir, 220t Cefepime, 221t Cefixime, 53t, 220t Cefonicid, 220t Cefotaxime, 220t, 238t, 439t Cefotetan, 55t, 220t Cefoxitin, 55t, 56t, 220t Cefpodoxime, 220t Cefprozil, 220t Ceftazidime, 323t, 439t Ceftibuten, 220t Ceftizoxime, 220t Ceftriaxone, 220t for acute otitis media, 126t for chancroid, 54t for gonorrhea, 53t for infective endocarditis prophylaxis, 80t for neonatal infections, 440t for pelvic inflammatory disease, 56t Cefuroxime, 220t Cellulitis, 114t, 233t, 234 Central core disease, 453t Central diabetes insipidus, 150–151t Central nervous system tumors, 296–297t Central shunt, 81 Central venous line (CVL) access, 25–27, 26t, 27f infections complications, 249 management, 247, 248f, 249t types, 247t Cephalexin, 80t, 220t Cephalosporins, 220–221t, 439–440t Cephapirin, 220t Cephradine, 220t Cerebral edema, 99, 101 Cerebral salt wasting (CSW), 150t Cerebrospinal fluid analysis, 244t, 442t Cerumen, 5–6, 6f Ceruminolytic preparations, 6t Cervical adenitis, 231t Cervical dysplasia, screening recommendations, 212t Chancroid, 54t CHARGE syndrome, 187 Chemical conjunctivitis, in neonates, 443–444t Chemistry studies SI laboratory value conversion table, 8t tube colors, 6, 7t Chemotherapy agents antitumor spectrum, 298–299t emetogenic potential, 291t hypersensitivity reactions to, 290–291t toxicities, 298–299t

Index Chest pain differential diagnosis, 78–79 green flags, 78 red flags, 78 workup, 79 Chest tube placement, 22–23, 22f, 22t Chest x-ray in congenital heart disease, 379, 382f, 382t in persistent pulmonary hypertension, 463t in respiratory distress syndrome, 458t in transient tachypnea of the newborn, 458t Chicken broth, 157t Chickenpox, 116t Child abuse, 141–142 Childhood weakness, 286–287t Chlamydia trachomatis infection, 54t, 212t Chlamydial conjunctivitis, in neonates, 443–444t Chloral hydrate, 18, 18t Chloride conversion, 43t daily requirement, 43t in mechanical ventilation, 89 Chlorothiazide, for bronchopulmonary dysplasia, 467t Cholelithiasis, in cystic fibrosis, 319t Cholestasis, in total parenteral nutrition, 44 Cholinergic toxidrome, 344t Chorionic villus sampling (CVS), 358–359 Chronic arthritis, 327t Chronic kidney disease, 265–267 Churg-Strauss syndrome, 333t Ciprofloxacin, 224t adverse effects, 322t for chancroid, 54t for P. aeruginosa, 322t Circumcision, 350 Cisplatin, 298t Citrullinemia, 193t, 194f Clarithromycin, 80t, 222t Clavicular fracture, 303t Cleft lip and palate, 128–129 Clindamycin, 223t adverse effects, 323t for bacterial vaginosis, 53t for infective endocarditis prophylaxis, 80t for neonatal infections, 439t for pelvic inflammatory disease, 56t for S. aureus, 323t Clinical Respiratory Score (CRS), 90–91t, 138t Clobetasol propionate, 123t Clotrimazole, 53t CMV-seronegative blood components, 207 Coagulation studies, tube colors for, 6, 7t Coagulopathies. See Bleeding disorders Coca Cola Classic, 157t Cocaine, 341t, 342t, 346t Coccidioidomycosis, 227 Codeine, 13t, 14t Colistimethate sodium, 323t Colitis, indeterminate, 180. See also Crohn disease; Ulcerative colitis Collection tubes, 7t

483

Coma, 141 Comminuted fracture, 302 Common warts, 117t Compensated (warm) shock, 93, 97f, 98. See also Shock Complement deficiency, 63–64, 64t Complex partial seizure, 275t. See also Seizures Computed tomographic angiography (CTA), 274t Computed tomography (CT) brain, 274t pre-procedural diet, 5t signal density, 274t Condyloma acuminata (genital warts), 56, 117t Congenital fiber type disproportion myopathy, 453t Congenital heart disease acyanotic, 66–67 arterial blood gases in, 382 chest x-ray findings in, 382f, 382t cyanotic, 67–70, 381t differential diagnosis, 383–384t in Down syndrome, 184 EKG findings in, 381–382t empiric approach to evaluation, 380t four-extremity blood pressures in, 381 history, 381 hyperoxia test in, 383, 383t infective endocarditis prophylaxis in, 80, 80t initial stabilization, 384 management, 384–385 oximetry in, 381 pathophysiology and age at presentation, 383–384t prenatal testing, 381 surgical procedures, 81–82, 81f, 82f symptoms, 381t, 383–384t Congenital hypoventilation syndrome, 469 Congenital melanocytic nevus, 387t Congenital muscular dystrophy, 453t Conjugated (direct) hyperbilirubinemia, 175 Conjunctivitis, in neonates, 443–444 Constipation, 39t, 179–180 Contact dermatitis, 111t Continuous positive airway pressure (CPAP), 85t Contraception, emergency, 59 Contraction stress test, 360 Controlled mechanical ventilation (CMV), 86t Cooked cabbage odor, 189t Coordination testing, 269 Copper, for direct hyperbilirubinemia, 420t Cordocentesis, 359 Corneal opacifications, 192 Corticosteroids adrenal insufficiency and, 392t as chemotherapy agents, 299t immunizations in patients receiving, 217t inhaled, 316t intranasal, 61t systemic, 9–10t, 137f topical, 110t, 123–124t Cortisone, equivalencies, 9t Cow’s milk, 156t Cow’s milk–based formulas, 46

484

Index

Coxsackie A16, 116t CPS (carbamoyl-phosphatase synthetase) deficiency, 193t, 194f Cradle cap, 111t Cranial nerves, examination methods, 268t Creatinine, serum, 251 Creatinine clearance, 250–251 Cremasteric reflex, 269t Crigler-Najjar syndrome, 412t Crohn disease clinical features, 180t definition, 179 endoscopic features, 181t epidemiology, 180t radiographic features, 181t treatment, 181–182, 182t Cromolyn sodium, 63t Cross-match, 205 Croup, 136–137t CRS (Clinical Respiratory Score), 90–91t, 138t Crural muscles, actions and nerve root supply, 273t Crust, 109 Cryoglobulinemia, 332t Cryoprecipitate, 207, 208 Cryptococcosis, 227 Crystals, in urine, 256t CSW (cerebral salt wasting), 150t CT. See Computed tomography (CT) CTA (computed tomographic angiography), 274t Cutaneous innervation, 270–271f CVL. See Central venous line (CVL) CVS (chorionic villus sampling), 358–359 Cyanosis, 353t, 380t, 381t Cyclophosphamide, 298t Cyproheptadine, 282t Cystic adenomatoid malformation, congenital, 475–477t Cystic fibrosis complications, 319–320, 320t diagnosis, 317 epidemiology, 317 genetics, 317 management airway clearance therapies, 321 antibiotic options, 321, 322–323t nutrition, 321 pharmacologic, 321–322t manifestations, 318–319t pneumonia in, 235t, 318t, 319 symptoms and signs, 318t treatment, 318–319t Cytarabine, 298t Cytokine release syndrome, 290–291t D D5 1/2 NS, 158t D5 1/4 NS, 158t D5W, 158t Dacryostenosis, 443–444t Dactinomycin, 298t Dactylitis (hand-foot syndrome), 199t Damus-Kaye-Stansel procedure, 81

Daptomycin, 226t De Quervain thyroiditis, 149 Decompensated (cold) shock, 93, 97f, 98. See also Shock Deep tendon reflexes, 269 Deferoxamine, 345t Dehydration classification, 55 intravenous rehydration, 157, 158t oral rehydration, 155–157 symptom-based determination, 154t weight-based determination, 154 Dejerine–Sottas disease, 453t Delayed puberty, 151 Demyelinating diseases, 284–286 Dennie’s lines, 62 Dental caries, 106–107 Dental procedures, infective endocarditis prophylaxis for, 80t Dermal melanocytosis, 387t Dermatomyositis, 329t Desonide, 124t Desoximetasone, 123t Developmental observations, 209–211t Dexamethasone adverse effects, 316t as antiemetic, 292t for asthma, 316t as chemotherapy agent, 299t equivalencies, 10t for spinal cord compression, 290 Dexmedetomidine, 18, 19t Diabetes insipidus, 150–151t Diabetes mellitus blood glucose goals, 146t cystic fibrosis and, 319t diagnosis, 143t HbA1c goals, 146t hyperglycemic emergencies in, 98–99, 99t, 100f hypoglycemia management in, 147, 148f inpatient management of, 145 insulin for, 145–147 in NPO patients, 146 in pregnancy, 355, 357t, 399t secondary causes, 144 secondary immunodeficiency in, 64t type 1, 143, 143t type 2, 143–144, 143t Diabetic ketoacidosis (DKA), 98–99, 99t, 100f Dialysis, indications for, 265 Diaper dermatitis, 119t, 390, 390t Diaphragmatic hernia, congenital, 475–477t Diarrhea, with enteral feeding, 39t Diazepam, 17t Dicloxacillin, 219t Diet, pre-procedural guidelines, 4–5t Diflorasone diacetate, 123t Digoxin as antiarrhythmic, 77t antidote, 345t for dilated cardiomyopathy, 73 poisoning/overdose, 346t

Index Dilated cardiomyopathy, 72–73, 72t Dimercaprol, 345t Diphenhydramine, 61t Diphtheria, tetanus, pertussis vaccine catch-up schedule, 214t, 215t contraindications, 216t regular schedule, 213t Direct Coombs test, 206 Discharge criteria neonates, 376 post-sedation, 20t Discharge orders, 1 Discharge summary, 2 Disseminated intravascular coagulation (DIC), 427t Dissociative shock, 95t Distal intestinal obstruction syndrome (DIOS), 320t Distributive shock, 94t, 95t Diuretics for bronchopulmonary dysplasia, 467t for dilated cardiomyopathy, 73 in mechanical ventilation, 89 DMSA scan, 242t Dobutamine, 96, 96t Dog bites, 135t, 233t Dopamine in neonatal resuscitation, 365t for shock, 96, 96t Down syndrome (trisomy 21), 64t, 183, 184–185t, 422–423t Doxorubicin, 298t Doxycycline, 223t adverse effects, 322t for chlamydia, 54t for lymphogranuloma venereum, 54t for pelvic inflammatory disease, 55t, 56t for S. aureus, 322t Dronabinol, 292t Drug hypersensitivity syndrome (DHS), 122t Drug monitoring collection tube colors, 7t SI laboratory value conversion table, 8t urine testing for substances of abuse, 342t Drug reaction with eosinophilia and systemic symptoms (DRESS), 122t Drug reactions, 120–123t Drugs of abuse. See Substance abuse Duodenal atresia and stenosis, 473–475t Dyslipidemia, screening recommendations, 212t E Ear wax (cerumen), 5–6, 6f Eating disorders, 56–59, 57–58t ECF (extracellular fluid), 158t Echocardiography, pre-procedural diet, 5t Eclampsia, 356 ECMO (extracorporeal membrane oxygenation), 463t, 470 Ecstasy, 341t Ectodermal dysplasias, 389t Eczema, 110–111

485

Eczematous/papulosquamous eruptions, 111–113t Edema, cerebral, 99, 101 ELA-Max, 11t Elbow fractures, 304t Electrocardiogram (ECG; EKG) in congenital heart disease, 381–382t drugs and changes in, 77 electrolytes and changes in, 76–77 in hypertrophy, 74–75 intervals, 74 in ischemia and infarction, 75 low voltage, 75 normal values, 73t in pericarditis, 71, 71f QRS depolarization axis, 74, 74f rate, 74 rhythm, 74 Electrolytes, 43t, 153 EM (erythema multiforme), 122t Embryology, 186t, 187 Emergency contraception, 59 EMLA, 11t Encephalopathy, neonatal, 450–452 End-stage liver disease (ESLD) ascites in, 174 bacterial peritonitis in, 174, 175t clinical presentation, 172 diagnosis, 172 etiology, 172 hepatic encephalopathy in, 173, 173t hepatorenal syndrome in, 173, 174–175t portal hypertension in, 172 treatment, 172 varices in, 173 End-stage renal disease (ESRD), 266 Endotracheal tubes, 16t, 365t Energy, DRI by age, 40t Enfalyte, 156t Enfamil HMF, 48t Enfamil Lipil, 156t Ensure, 156t Enteral feeding bolus or intermittent, 38t complications, 38t, 39t continuous, 38t delivery routes, 38t in direct hyperbilirubinemia, 420t indications, 37, 38t preterm infants, 39t principles, 36, 37f Enterovirus 71, 116t Eosinophilia, 202t Eosinophils, 201t Ependymoma, 296–297t Epidermolysis bullosa (EB), 389t Epidermolytic hyperkeratosis, 388t Epididymitis, 131–132t Epiglottitis, 136–137t Epilepsy classification scheme, 277–278t syndromes, 278–279t treatment, 279–280t

486

Index

Epinephrine for anaphylaxis, 61t, 97f in neonatal resuscitation, 264t, 365t for shock, 96, 96t warnings, 11, 12t Equianalgesic doses, 14, 14t Erosion, 109 Erysipelas, 114t Erythema infectiosum (fifth disease), 116t Erythema multiforme (EM), 122t Erythema toxicum, 386t Erythromycin, 222t for chancroid, 54t for lymphogranuloma venereum, 54t ESLD. See End-stage liver disease (ESLD) ESRD (end-stage renal disease), 266 Ethanol, prenatal exposure effects, 423t Ethosuximide, 279t Ethyl chloride topical spray, 12t Ethylene glycol, antidote, 345t Etomidate adverse effects, 19t dosing, 19t onset and duration, 19t pharmacologic properties, 18 in septic shock, 98 Etoposide, 299t Exanthem subitum (roseola), 116t Exanthematous drug reaction, 120t Exchange transfusion complications, 431 for hyperbilirubinemia, 414f, 414t, 430t indications, 430t partial, 433t for polycythemia, 430t preparation, 430t procedure, 431 typing and cross-matching for, 430–431t Excoriation, 109 Extracellular fluid (ECF), 158t Extracorporeal membrane oxygenation (ECMO), 463t, 470 Extraocular muscles, innervation, 269 Extremely low birth weight infants, 369. See also Preterm infants Extremity laceration, 31t Eyelid laceration, 31t F Face masks, for O2 delivery, 84–85t FACES pain scale, 11f Facial laceration, 31t Facial nerve, 268t Factor 8 replacement, 204t Factor 9 replacement, 204t Factor H deficiency, 64t Failure to thrive, 182–183, 191t Famciclovir, 55t Fanconi syndrome, 152t, 428 Fatty acid oxidation disorders, 191t Febrile seizures, 276 Feeding hypoxemia, 469

Felbamate, 279t Femoral vein, 26, 27f Femur fractures, 308t Fentanyl dosing, 13t, 14t, 457 in neonates, 457 onset and duration, 13t in patient-controlled analgesia, 14t weaning from, 20 Fetal heart rate (FHR) monitoring, 361–362, 361t Fetal hydrops, nonimmune, 421–422t Fetus antepartum surveillance biophysical profile, 360–361t contraction stress test, 360 indications, 359–360t non-stress test, 360 complications of maternal medical conditions, 354, 355, 357t complications of maternal substance abuse, 357t genetic diagnosis techniques, 358–359 intrapartum heart rate monitoring, 361–362, 361t lung maturity evaluation, 355, 355t serum screening for chromosomal abnormalities, 358t sonographic assessment, 357, 358 Fever, as oncologic emergency, 289 Fever without localizing signs in patients 2 months and younger, 130f in patients 2 months to 36 months old, 131f FFP (fresh frozen plasma), 435t Fifth disease (erythema infectiosum), 116t Finger fractures, 307t Fissure, 109 Fixed drug eruption, 121t FLM-TDx, 355t Fluconazole, 53t, 119t Fludrocortisone, 10t Fluid requirements, 153–154 Flumazenil, 17, 345t Fluocinolone acetonide, 124t Fluocinonide, 123t Fluoride consumption, 106 Fluoroquinolones, 224–225t Fluoroscopy, pre-procedural diet, 5t Fluorosis, 106 Flurandrenolide, 123t Fluticasone acetonide, 124t Fluticasone propionate, 123t, 124t FOC. See Head circumference (FOC) Focal segmental glomerulosclerosis, 260t Folliculitis, 113t, 233t Fomepizole, 345t Fontan procedure, 81, 81f Fontanelles, 4 Foot fractures, 310–311t laceration, 31t Formulas, infant, 46–47t Fosphenytoin, 104f

Index Fractures ankle, 310t in child abuse, 141 elbow, 304t evaluation, 302 femur, 308t foot, 310–311t hand, 306–307t hip, 308t knee, 308–309t management principles, 303 radius, 305t shoulder, 303t tibia and fibula, 309t types, 302, 302f ulna, 306t Free amino acid formulas, 46 Free water deficit, 161 Freiberg’s infarction, 311t Fresh frozen plasma (FFP), 435t Fresh hay odor, 341t Fruity odor, 341t FSI, 355t Fungal infections, 118–119t, 227 Furosemide, for bronchopulmonary dysplasia, 467t Furuncle, 233t G Gabapentin, 279t GABHS (group A β-hemolytic streptococcus), 114t, 125 Galeazzi fracture, 305t Gallstones, 140 Gamekeepers thumb, 307t γ-hydroxybutyrate (GHB), 341t Garlic odor, 341t Gastric aspirates, large or increased, 414–415 Gastric emptying study, pre-procedural diet, 5t Gastroesophageal reflux, 169–170 Gastroesophageal reflux disease (GERD), 169–170, 410–411 Gastrojejunal feeding, 38t. See also Enteral feeding Gastroschisis, 471–472t Gastrostomy, 38t. See also Enteral feeding Gatifloxacin, 225t Gatorade, 157t GBS. See Group B streptococcus (GBS) infections Gell and Coombs classification, hypersensitivity immune responses, 60t Generalized seizures, 276t. See also Seizures Genital warts, 56, 117t Gentamicin, 56t, 224t, 438t Gentamicin/tobramycin, 323t German measles (rubella), 116t Gestational age assessment, 354, 354f Gestational hypertension, 356 GHB (γ-hydroxybutyrate), 341t Gianotti-Crosti syndrome, 117t Giant hemangioma, 427t Gilbert syndrome, 412t Glabellar reflex, 270t Glenn anastomosis, 81, 81f

487

Glioma, brainstem, 296–297t Glomerular filtration rate (GFR), 250, 251t Glossopharyngeal nerve, 268t Glucagon, 245t Glucose for neonatal hypoglycemia, 405–406 requirements, 153 in urine, 255t Glycogen storage diseases, 191t Glycopeptides, 222t Glycosylation disorders, 191t Gonococcal conjunctivitis, in neonates, 443–444t Gonorrhea, 53t, 212t Goodpasture syndrome, 332t Gram-negative organisms algorithm for identification, 230f empiric antibiotic choices, 228f Gram-positive organisms algorithm for identification, 229f empiric antibiotic choices, 228f Granulocyte transfusion, 436t Graves disease, 149 Greenstick fracture, 302 Griseofulvin, 118t Group A β-hemolytic streptococcus (GABHS), 114t, 125 Group B streptococcus (GBS) infections classification, 442–443t intrapartum prophylaxis, 367f, 368f treatment, 443t Growth charts, 49, 50f Gynecologic examination, 51–52, 51t H H2R antagonists, 169 Haemophilus ducreyi, 54t Haemophilus influenza in acute otitis media, 125 in cystic fibrosis, 318t, 322t immunization schedule, 213t, 214t in sinusitis, 128 Hair, abnormal, 189 Halcinonide, 123t Halobetasol propionate, 123t Hand fractures, 306–307t laceration, 31t Hand-foot-mouth disease, 116t Harlequin ichthyosis, 389t Hashimoto’s thyroiditis, 147 HbA1c, 146t Head circumference (FOC) by age for boys, 2t, 3 by age for girls, 3t by gestational age, 50f rate of gain, 3, 4 Headaches chronic daily, 283 classification scheme, 281t migraine. See Migraine headaches neuroimaging in, 281 tension type, 283

488

Index

Health supervision, 209–211t Hearing screening, 212t Heart disease arrhythmias, 75t, 76f, 77t athletic restrictions in, 80–81 congenital. See Congenital heart disease dilated cardiomyopathy, 72–73, 72t infective endocarditis prophylaxis in, 80, 80t myocarditis, 70–71, 70t pacemakers for, 77 pericardial, 71–72, 71f Heavy metal poisoning, antidote, 345t Height/length by age for boys, 2t by age for girls, 3t by gestational age, 50f rate of gain, 3 Heliox for respiratory failure, 85t for status asthmaticus, 92t Hematology studies SI laboratory value conversion table, 8t tube colors, 6, 7t Hematuria, differential diagnosis, 252–254, 253f, 255t Hemoglobin, 196t Hemolysis effect on lab values, 7t hyperbilirubinemia and, 412t Hemolytic disease of the newborn, 430t Hemophagocytic-lymphohistiocytosis (HLH), 295 Hemophilia, 204, 204t Hemoptysis, in cystic fibrosis, 320t Henoch Schönlein purpura, 333, 335 Hepatic encephalopathy, 173, 173t Hepatitis infectious, 170 noninfectious, 171 Hepatitis A, 170t Hepatitis A immunization, 212t, 215t Hepatitis B, 170t Hepatitis B immunization in preterm infants, 444 schedule, 213t, 214t, 215t Hepatitis C, 170t Hepatomegaly, 192 Hepatorenal syndrome, 173–174t Hepatosplenomegaly, 192 Hereditary angioedema, 62 Hereditary hemochromatosis (HH), 171t Hereditary hypophosphatemic rickets with hypercalciuria (HHRN), 152t Hereditary motor and sensory neuropathy type III, 453t Hernia congenital diaphragmatic, 475–477t incarcerated, 140 Heroin, 341t Herpes simplex virus (HSV) diagnosis, 54t genital infection, 54t, 55t, 117t

Herpes simplex virus (HSV) (cont.) oral infection, 117t superinfection in eczema, 110t treatment, 55t, 117t Herpes zoster (shingles), 117t HHV-6, 116t HIDA scan, pre-procedural diet, 5t HIE (hypoxic-ischemic encephalopathy), 448t, 451–452t High-frequency oscillatory ventilation (HFOV), 87t, 375t Hip fracture, 308t Hirschsprung’s disease, 140, 409t Histoplasmosis, 227 HIV immunizations in, 217t screening recommendations, 212t HLHS (hypoplastic left heart syndrome), 69, 69f, 81, 81f Hoffman sign, 269t HSV. See Herpes simplex virus (HSV) Human bites, 135t, 233t Human milk characteristics, 45 composition, 45, 156t contraindications, 45 fortifiers, 48t with infant formula powder, 47t storage durations, 36t vitamin and mineral supplementation, 35t Human papillomavirus (HPV), 56, 117t, 212t, 215t Hybrid Norwood procedure, 81–82 Hydrocephalus, 453–454 Hydrocodone, 13t, 14t Hydrocortisone, 124t for anaphylaxis, 61t equivalencies, 9t Hydrocortisone butyrate, 124t Hydrocortisone valerate, 124t Hydromorphone, 13t, 14t 21-Hydroxylase deficiency, 392t 3-β-Hydroxysteroid dehydrogenase deficiency, 392t Hyperammonemia, 194–195, 195f Hyperbilirubinemia direct (conjugated) definition, 175, 418 diagnosis, 175, 419, 419t etiologies, 419t management, 420t in inborn errors of metabolism, 189 indirect (unconjugated), 175–176, 411 nonphysiologic causes, 412t evaluation, 412 management, 412–414, 413f, 414f physiologic, 411 Hypercalcemia ECG changes in, 76 neonatal, 397–398, 397f Hyperekplexia, 449t

Index Hyperglycemia with enteral feeding, 39t neonatal, 403–404 in total parenteral nutrition, 44 Hyperglycemic hyperosmolar state (HHS), 98–99, 99t, 100f Hyperkalemia causes, 158–159 ECG changes in, 76 exchange transfusion for, 430t neonatal, 395–396 signs and symptoms, 158 treatment, 159 workup, 159 Hyperleukocytosis, 291 Hypermagnesemia, ECG changes in, 77 Hypernatremia dehydration in, 155t neonatal, 406–407 signs and symptoms, 160 treatment, 161–162 workup, 161, 161f Hyperoxia test, 383, 383t Hypersensitivity immune responses, 60t, 290–291t Hypersplenism, 427t Hypertension definition, 251 initial workup, 252f in neonates, 446–447 in pregnancy, 356, 356t, 357t Hyperthyroidism, 149 Hypertonic saline, 158t Hypertriglyceridemia, in total parenteral nutrition, 44 Hyperventilation, 165t Hypocalcemia early neonatal, 398–399, 400t, 401t ECG changes in, 76 late neonatal, 399–401, 400t, 401t Hypoglossal nerve, 268t Hypoglycemia in inborn errors of metabolism, 188, 188f, 191t, 192, 404t neonatal, 188f, 404–406 in type 1 diabetes mellitus, 147, 148f Hypokalemia causes, 160 ECG changes in, 76 neonatal, 396–397 signs and symptoms, 159–160 treatment, 160 workup, 160 Hypomagnesemia ECG changes in, 77 neonatal, 399t Hyponatremia causes, 163f dehydration in, 155t neonatal, 407–408 signs and symptoms, 162 treatment, 162–164 workup, 163f

489

Hypoparathyroidism, neonatal, 399t Hypophosphatemia, 152t Hypopituitarism, 393t Hypoplastic left heart syndrome (HLHS), 69, 69f, 81, 81f Hyposplenism, secondary immunodeficiency in, 64t Hypotension, 94, 357t. See also Shock Hypothermia, induced, 102 Hypothyroidism, 147, 149 Hypothyroxinemia, in preterm infants, 394–395, 394f Hypotonia in inborn errors of metabolism, 188 neonatal, 452 Hypovolemic shock, 94t, 95t Hypoxic-ischemic encephalopathy (HIE), 448t, 451–452t I Ibuprofen in cystic fibrosis, 322t dosing, 9t, 12t for migraine headache, 282t ICP (increased intracranial pressure), 101–102 Idiopathic thrombocytopenic purpura (ITP), 202 Ifosfamide, 298t IgA (Berger) nephropathy, 259t Ileal atresia, 409t Ileus, 410t Imipenem, 221t Immediate hypersensitivity skin test (IHST), 60 Immunizations after bone marrow transplantation, 299–300 catch-up schedule, 214–215t contraindications, 216t in preterm infants, 444, 444t recommended schedule, 213t for special populations, 217t Immunodeficiency disorders immunizations in, 217t primary, 63, 63t secondary, 64–65t Immunotherapy, for allergic rhinitis, 63t Imperforate anus, 409t Impetigo, 113t, 233t IMV (intermittent mandatory ventilation), 87t Inborn errors of metabolism acute and recurrent attacks, 192 chronic and progressive symptoms, 192 classes, 188t clinical and laboratory findings, 191t hypoglycemia in, 188f, 404t laboratory tests, 192–193 management, 194–195 metabolic acidosis in, 189f signs and symptoms, 188–189, 188f, 189f urine and body odors in, 189–190t urine discoloration in, 190t Incision and drainage (I&D), abscess, 32–33, 33t Increased intracranial pressure (ICP), 101–102

490

Index

Indirect Coombs test, 205 Indomethacin, 12t, 380, 380t Infants bacterial meningitis in, 245t feeding bottle weaning, 106 enteral. See Enteral feeding formula mixing, 46–47t formula selection, 45–46 vitamin and mineral supplementation, 35t fever without localizing signs in, 130f, 131f preterm. See Preterm infants reflexes, 271–272t skin and soft tissue infections in, 234 total parenteral nutrition. See Total parental nutrition (TPN) Infections central venous catheter-related, 247–249, 247t, 248f, 249t fungal, 118–119t, 227 intravascular catheter-related, 246–247, 246t in neonates, 437–440t odontogenic, 108 pneumonia. See Pneumonia skin, 233–234 urinary tract. See Urinary tract infections Infectious rhinitis, 127 Infective endocarditis, prophylaxis, 80, 80t Infertility, in cystic fibrosis, 319t Inflammatory bowel disease, 180, 180t, 181t. See also Crohn disease; Ulcerative colitis Influenza vaccine, 212t Infuvite, 44t Inhaled nitric oxide (iNO) for persistent pulmonary hypertension, 463t for preterm infants, 373 Insulin for diabetes mellitus dose adjustment, 147 regimens, 145–146, 146t sliding scale, 146–147 types, 145t for neonatal hyperglycemia, 403 Insulin aspart, 145t Insulin detemir, 145t Insulin glargine, 145t Insulin glulisine, 145t Insulin lispro, 145t Insulin NPH, 145t Insulin pump, 146 Insulin regular, 145t Intermittent mandatory ventilation (IMV), 87t Internal jugular vein (IJ), 26 Interventional radiology, pre-procedural diet, 5t Intestinal lymphangiectasia, secondary immunodeficiency in, 65t Intracranial hemorrhage intraventricular, 450, 450t MRI evaluation, 275t neonatal seizures and, 448t Intraosseous (IO) access, 24–25, 25t Intraventricular hemorrhage, 450, 450t

Intussusception, 140 Ionizing radiation, secondary immunodeficiency and, 65t Ipratropium bromide, 92t, 137f Iron-deficiency anemia, 197t, 198–199 Iron overload, 171t Iron poisoning, antidote, 345t Irradiated blood, 208 Irritant contact dermatitis, 111t Isolyte M, 158t Isolyte P, 158t Isonatremic dehydration, 155t ITP (idiopathic thrombocytopenic purpura), 202 Itraconazole, 322t Ivermectin, 120t J Jejunoileal atresia and stenosis, 473–475t Jejunostomy, 38t. See also Enteral feeding Jevity, 156t Jock itch (tinea cruris), 118t Joint pain, non-traumatic causes, 132–133t Juvenile idiopathic arthritis (JIA), 329–330 Juvenile myoclonic epilepsy, 278t K Kasabach-Merritt syndrome, 427t Kawasaki disease clinical findings, 332t, 336–337t definition, 335 diagnosis, 335, 336t, 338f differential diagnosis, 336t epidemiology, 335 incomplete, 337t treatment, 337 Keppra, 103t Keratosis pilaris, 112t Ketamine adverse effects, 18t dosing, 18t onset and duration, 18t pharmacologic properties, 18 for status asthmaticus, 92t Ketoconazole for seborrheic dermatitis, 111t for tinea versicolor, 120t Ketogenesis disorders, 191t Ketorolac, 12t Kleihauer-Betke preparation, 433 Knee fractures, 308–309t Kohler’s disease, 310t Konno-Rastan procedure, 81 L Laceration repair, 30–31t, 30–32 Lactated Ringer’s solution (LR), 158t Lactic acidosis, in inborn errors of metabolism, 191t Lactose-free formulas, 46 Lamellar body count, 355t Lamellar ichthyosis, 388t Lamotrigine, 280t Landau-Kleffner syndrome, 279t

Index Langerhans cell histiocytosis, 391t Laryngomalacia, 477–478 Laryngoscope tube, 16t Lead poisoning antidote, 345t screening recommendations, 212t Left ventricular hypertrophy (LVH), 74 Length. See Height/length Lennox-Gastaut syndrome, 279t LET (lidocaine, epinephrine, tetracaine) solution, 11t Leukocyte esterase, in urine, 255t Leukocyte-reduced blood, 207 Leukocytes, total, 201t Leukocytosis, 202, 202t Leukotriene receptor antagonist, 63t, 316t Levalbuterol, 91t, 316t Levetiracetam, 280t, 282t Levofloxacin, 224t Levonorgestrel, 59 Levothyroxine, 147 Lice, 120t Lichenification, 109 Lidocaine, 12t Limp, 132–134 Lincosamides, 223t Linezolid, 223t, 322t, 440t Lip laceration, 31t Lipids, in total parenteral nutrition, 42 Lipopeptides, 226t Lisfranc fracture, 310t Lithium, antidote, 345t Little league elbow, 304t Little league shoulder, 303t Liver failure. See also End-stage liver disease (ESLD) in cystic fibrosis, 319t fulminant, 171–172, 172f in inborn errors of metabolism, 189, 191t, 192 in secondary immunodeficiency, 64t L.M.X. 4%, 11t Local anesthetics injectable, 12t for laceration repair, 30–31t topical, 11–12t Lorazepam adverse effects, 17t dosing, 17t onset and duration, 17t for seizures, 280t for status epilepticus, 104f Low birth weight infants, 369. See also Preterm infants Low-carbohydrate formulas, 46 Low-molecular-weight heparin (LMWH), 205t Low T3 syndrome, 149 Lower motor neuron lesions, 272t, 452t L:S ratio, 355t LSD, 341t Lucey-Driscoll syndrome, 412t Lumbar puncture, 23–24, 23t, 24f Lyme disease, 115t

491

Lymphadenitis clinical presentation, 231t definition, 231 differential diagnosis and management, 232f infectious causes, 228t, 231 noninfectious causes, 228t Lymphadenopathy definition, 231 differential diagnosis and management, 232f infectious causes, 228t noninfectious causes, 228t, 231 Lymphocytes, 201t Lymphocytosis, 202t Lymphogranuloma venereum, 54t Lysosomal metabolism disorders, 191t M Macrocephaly, 191t, 192 Macrocytic anemia, 197 Macrolides, 222t, 440t Macular stain, 387t Macule, 109 Magnesium, 43t, 77 Magnesium sulfate, 92t Magnetic resonance angiography (MRA), 274t Magnetic resonance imaging (MRI) brain, 274t fetal, 359 in intracranial hemorrhage, 275t sedated, pre-procedural diet, 5t sequences and potential applications, 275t signal intensity, 274t Magnetic resonance spectroscopy (MRS), 274t Magnetic resonance venogram (MRV), 274t Malabsorptive syndromes, 179 Malassezia furfur, 119t Mallampati classification, 15, 16t Mallet finger, 307t Malnutrition in cystic fibrosis, 319t, 320 definition, 49 secondary immunodeficiency in, 65t severity determination, 183t Waterlow criteria, 49 Malrotation clinical presentation, 140, 473–474t diagnosis, 409t epidemiology, 473t management, 409t, 474–475t pathophysiology, 140, 473t Manganese, for direct hyperbilirubinemia, 420t Mannitol, 101, 102 Maple syrup odor, 189t Marijuana, 341t, 342t Mast cell stabilizer, 63t Mastoiditis, 126–127 May-Hegglin anomaly, 427t McBurney’s point, 139 Mead Johnson powder formulas, 46–47t Mean cell hemoglobin (MCH), 196t Mean cell volume (MCV), 196t Measles (rubeola), 116t

492

Index

Measles, mumps, rubella vaccine, 212t, 215t Mechanical ventilation adjustments affecting oxygenation and ventilation, 88t blood gases and clinical parameters in, 89 for bronchopulmonary dysplasia, 466t complications, 89 diuresis and metabolic alkalosis in, 89 fluids in, 88 modalities, 86–87t, 87–88, 88t in neonates, 374–375t for respiratory distress syndrome, 459t sedation and analgesia in, 88 stepwise approach, 86–90 weaning and extubation, 89–90, 90t Meckel’s diverticulum, 140 Meconium, delayed passage of, 409–410 Meconium aspiration, 366–367, 366f, 464–465t Meconium ileus, 320t, 409t Meconium plug, 409t Medial epicondyle traction apophysitis, 304t Medulloblastoma, 296–297t Melanocytic nevus, congenital, 387t Membranoproliferative glomerulonephritis, 260t Menarche, 151 Meningitis aseptic, 243, 245 definitions, 242 diagnosis, 243–244, 244t etiology, 243t follow-up, 246 isolation and prophylaxis, 246 management, 244–245, 245t neonatal, 441–442 prognosis, 246 risk factors, 243t Meningococcal vaccine, 212t Meningococcemia, 115t Mercaptopurine, 298t Mercury poisoning, antidote, 345t Meropenem, 221t, 323t, 440t Metabolic acidosis additional testing, 164–165t differential diagnosis, 164–165t general approach, 166f, 167–168 in inborn errors of metabolism, 189f, 191t neonatal, 189, 189f with partial compensation, 167–168t Metabolic alkalosis differential diagnosis, 165t general approach, 166f, 167–168 in mechanical ventilation, 89 with partial compensation, 167–168t Metacarpal fracture, 307t Metatarsal fracture, 310t Methadone, 13t, 14t Methanol, antidote, 345t Methemoglobinemia, 346t Methimazole, 149 Methotrexate, 298t, 423t

Methylprednisolone adverse effects, 316t for anaphylaxis, 61t for asthma, 316t equivalencies, 9t for status asthmaticus, 91t Metoclopramide, 292t Metronidazole, 226t for bacterial vaginosis, 53t for pelvic inflammatory disease, 56t for T. vaginalis infection, 53t Miconazole, 53t Microcephaly, 191t, 192 Microcytic anemia, 197f, 197t Midazolam adverse effects, 17t dosing, 17t onset and duration, 17t for status epilepticus, 103t weaning from, 20 Midgut volvulus, 140, 473–475t Migraine headaches complications, 282 diagnostic criteria, 281t prophylaxis, 282t status migrainosus, 283 treatment, 282t Milia, 386t Miliaria crystalline, 386t Miliaria rubra, 386t Milk of Magnesia, 179 Milk scan, pre-procedural diet, 5t Milrinone in neonatal resuscitation, 365t for shock, 96, 96t Minerals, in total parenteral nutrition, 44t Minimal change disease, 259t Minocycline, 223t Mite infestations, 119–120t Mitochondrial disorders, 191t Mixed connective tissue disease (MCTD), 329t Molluscum contagiosum, 118t Mometasone furoate, 123t, 124t Mongolian spot, 387t Monobactams, 221t Monocytes, 201t Monocytosis, 202t Monteggia fracture, 306t Montelukast sodium, 63t, 316t Moraxella, 125, 128 Morbilliform drug reaction, 120t Moro reflex, 271t Morphine dosing, 13t, 14t, 457 in neonates, 457 in patient-controlled analgesia, 14t Mosteller’s formula, body surface area, 4 Mothball odor, 341t Motor testing, 269t Mousy odor, 189t Moxifloxacin, 225t MRA (magnetic resonance angiography), 274t

Index MRI. See Magnetic resonance imaging (MRI) MRS (magnetic resonance spectroscopy), 274t MRV (magnetic resonance venogram), 274t Mucolytics, 321t Mucositis, 292 Multiple sclerosis (MS), 285t Murmur, asymptomatic, 380t Muscles, principal, 272–273t Muscular dystrophy, congenital, 453t Musculoskeletal complaints, differential diagnosis, 37 Mustard procedure, 82, 82f Myocarditis, 70–71, 70t Myoclonus benign neonatal sleep, 449t stimulus-evoked, 449t N N-acetylcysteine, 322t Nafcillin, 219t, 437t Nailbed laceration, 31t Nalidixic acid, 224t Naloxone, 13, 346t, 365t Naproxen, 12t Nasal cannula, for O2 delivery, 84t Nasal continuous positive airway pressure (NCPAP) for neonatal apnea, 470 for respiratory distress syndrome, 459t Nasal decongestants, 63t Nasoduodenal feeding, 38t. See also Enteral feeding Nasogastric feeding, 38t. See also Enteral feeding Nasogastric tube insertion, pre-procedural diet, 5t Nasojejunal feeding, 38t. See also Enteral feeding Nasojejunal tube insertion, pre-procedural diet, 5t Natal tooth, 108 Nausea. See Vomiting/nausea Neck laceration, 31t Necrotizing enterocolitis (NEC) clinical presentation, 416t diagnosis, 417 differential diagnosis, 417 management, 418 platelet consumption in, 427t prognosis, 418 risk factors, 415–416 staging, 416–417t Needle aspiration/decompression of pneumopericardium, 461t of pneumothorax, 21, 21t, 460t Neisseria gonorrhea, 53t Neisseria meningitidis, 115t Nemaline rod myopathy, 453t Neocate Infant, 157t Neocate Jr., 157t Neonatal cephalic pustulosis (neonatal acne), 386t Neonatal lupus erythematosus, 388t Neonatal resuscitation algorithm, 364f endotracheal tube sizes, 365t medications for, 364–365t preparation, 363

493

Neonatal resuscitation (cont.) risk factors, 363 triage, 366t umbilical catheter placement, 365–366 Neonatal tooth, 108 Neonates abnormal rashes in, 388–391t acute kidney injury in, 445–446 anemia in, 431–433 antibiotic use in, 437–440t Apgar score, 349t apnea in. See Apnea, neonatal bacterial meningitis in, 245t benign rashes in, 386–387t blood product transfusion in, 433t, 434–436t bronchopulmonary dysplasia in, 465–468t circumcision of, 350 complications of maternal medical conditions, 354, 355, 357t complications of maternal substance abuse, 357t congenital cystic adenomatoid malformation in, 475–477t congenital diaphragmatic hernia in, 475–477t conjunctivitis in, 443–444 cyanosis in, 353t delayed passage of meconium in, 409–410 discharge criteria, 376 discharge planning considerations, 376–377t duodenal atresia and stenosis in, 473–475t early discharge criteria, 349–350 encephalopathy in, 450–452 extracranial bleeding sites in, 352f gastric aspirates in, 414–415 gastroesophageal reflux disease in, 410–411 gastroschisis in, 471–472t group B streptococcal disease in. See Group B streptococcus (GBS) infections hydrocephalus in, 453–454 hyperbilirubinemia in. See Hyperbilirubinemia hypercalcemia in, 397–398, 397f hyperglycemia in, 403–404 hyperkalemia in, 395–396 hypernatremia in, 406–407 hypertension in, 446–447 hypocalcemia in, 398–401 hypoglycemia in, 188f, 404–406 hyponatremia in, 407–408 hypotonia in, 452 intraventricular hemorrhage in, 450, 450t jejunoileal atresia and stenosis in, 473–475t laryngomalacia in, 477–478 malrotation in. See Malrotation meconium aspiration in, 366–367, 366f, 464–465t meningitis in, 441–442 necrotizing enterocolitis in. See Necrotizing enterocolitis neuromuscular disorders in, 453 neuromuscular maturity assessment, 348 nonsterile delivery, 353 omphalocele in, 471–472t pain management in, 456–457 persistent pulmonary hypertension in, 462–464t

494

Index

Neonates (cont.) physical exam, 350–351t physical maturity assessment, 348 pulmonary air leak syndromes in, 459–462 respiratory distress in, 353t respiratory distress syndrome in, 458–459t resuscitation. See Neonatal resuscitation routine care, 348–349 seizures in, 448–449 sepsis evaluation and empiric treatment, 369f skin and soft tissue infections in, 234 stridor in, 352t thrombocytopenia in, 425–428 transient tachypnea in, 458, 458t vomiting in, 352t Nephritic syndromes management, 261 overview, 258, 259t primary, 260t prognosis, 261 secondary, 260t workup, 260 Nephrogenic diabetes insipidus, 150–151t Nephrotic syndromes complications, 261–262 management, 261 overview, 258, 259–260t primary, 260t prognosis, 261 secondary, 260t secondary immunodeficiency in, 65t workup, 260–261 Nepro, 157t Nesiritide, 73 Nestle powder formulas, 46–47t Netherton syndrome, 389t Neuroblastoma, 295–296 Neuroimaging modalities, 274t Neuroleptics, antidote, 346t Neurologic exam, 268–270 Neuromuscular blockade, in traumatic brain injury, 102 Neuromuscular disorders, 453 Neuromyelitis optica (NMO), 285t, 286 Neutropenia in inborn errors of metabolism, 189 as oncologic emergency, 289 Neutrophilia, 202t Neutrophils, 201t Nevus sebaceous, 387t Nevus simplex, 387t Newborns. See Neonates Nitric oxide, inhaled (iNO) for persistent pulmonary hypertension, 463t for preterm infants, 373 Nitrite, in urine, 256t Nitroimidazoles, 226t Nodule, 109 Non-stress test, 360 Nonbullous congenital ichthyosiform erythroderma, 388t Nonimmune fetal hydrops, 421–422t

Noninvasive positive pressure ventilation, 86t Nonopioid analgesics, 12, 12t. See also specific drugs Nonrebreather face mask, for O2 delivery, 85t Norepinephrine, 96t, 97f Normal saline (NS), 158t, 264t Norwood procedure, 81, 82f Notes discharge summary, 2 on-service, 1 progress, 1–2 Nursemaid’s elbow, 305t Nutramigen Lipil, 157t Nutren 1.0, 156t Nutren 2.0, 156t Nutren Jr., 156t Nystatin, 119t O Obstructive lung disease, 326t. See also Asthma; Cystic fibrosis Obstructive shock, 94t, 95t Obstructive sleep apnea, 128, 129f Obturator sign, 139 Oculomotor nerve, 268t Odontogenic infections, 108 Odors abnormal, 189–190t of agents of intoxication, 341t Ofloxacin, 224t Old fish odor, 189t Olecranon fracture, 306t Olfactory nerve, 268t Omalizumab, 316t Omphalocele, 471–472t On-service notes, 1 Oncologic emergencies fever and neutropenia, 289 hyperleukocytosis, 291 hypersensitivity reactions to infusions, 290–291t spinal cord compression, 289–290 tumor lysis syndrome, 289 Ondansetron, 292t Ophthalmia neonatorum, 443–444 Opioid analgesics. See also specific drugs antidote, 346t conversion between, 14, 14t dosing, 13t, 14t indications, 13 in patient-controlled analgesia, 13, 14t toxidrome, 345t urine testing, 342t weaning from, 14–15 Optic nerve, 268t Oral hygiene, 106, 212t Oral rehydration solutions and formulas, 156–157t strategies, 155–156 Orders admission, 1 discharge, 1 preoperative, 1

Index Organ systems, embryonic development from weeks 3 to 7, 186t Organic acidemias, 194f Organic acidurias, 191t Organophosphates, antidote, 346t Ornithine transcarbamylase (OTC) deficiency, 193t, 194f Orogastric feeding, 38t. See also Enteral feeding Osgood-Schlatter disease, 308t Osteochondritis dissecans, femur, 309t Osteopenia of prematurity, 401–403 Osteopetrosis, infantile, 400t Ostium primum, 66 Ostium secundum, 66 Otitis media, acute, 125–126 Otitis media with effusion (OME), 126 Oxacillin, 219t Oxazolidinones, 223t, 440t Oxcarbazepine, 280 Oxycodone, 13t, 14t Oxygen delivery modes, 84–85t P P. aeruginosa, in cystic fibrosis, 318t, 322–323t Pacemakers, 77 Packed cell volume, 196t Packed red blood cells (PRBCs), 206t, 433t, 434t Pain assessment, 11, 11f Pain management in neonates, 456–457 nonpharmacologic, 11 pharmacologic. See also specific drugs nonopioid analgesics, 12, 12t opioid analgesics. See Opioid analgesics patient-controlled analgesia, 13, 14t topical preparations, 11–12t Palivizumab, 378 Palmar grasp, 272t Palmomental reflex, 270t Pancreas, annular, 140 Pancreatic enzyme replacement therapy, 321t Pancreatic insufficiency, in cystic fibrosis, 318t Pancreatitis, 176–178t Pancrelipase, 321t Pancytopenia, 202t Papile’s grading, intraventricular hemorrhage, 450t Papular acrodermatitis, 117t Papule, 109 Paracentesis, in ascites, 174 Paracoccidioidomycosis, 227 Paramyxovirus, 116t Partial rebreathing face mask, for O2 delivery, 85t Parvovirus B19, 116t Patch, 109 Patellar fracture, 308t Patent ductus arteriosus (PDA), 67, 379–380 Patient-controlled analgesia (PCA), 13, 14t PCP, 341t Peak flow monitors, 313 Pear odor, 341t

495

Pedialyte, 156t PediaSure, 156t Pediatric Appendicitis Score (PAS), 139t Pediatric systemic lupus erythematosus (pSLE), 329t, 331–332 Pediculosis pubis, 56 Pediculus capitis, 120t PEG 3550, 179 PEG-electrolyte solution, 179 Pelvic examination, 51–52, 51t Pelvic fracture, 308t Pelvic inflammatory disease, 55–56t Penicillin G, 218t Penicillin G benzathine, 54t, 218t Penicillin GK, 437t Penicillin V, 218t Penicillins, 218–219t Penis laceration, 31t noncircumcised, care of, 350 Pentobarbital, 18, 18t, 103t Percutaneous umbilical blood sampling, 359 Perianal streptococcal dermatitis, 390t Pericardial disease, 71–72, 71f Pericardiocentesis, 462 Peripheral nerves, 270–271f Permethrin, 120t Peroxisomal metabolism disorders, 191t Persistent pulmonary hypertension, 462–464t PG, 355t pH, urine, 254t Phalanx fractures, 307t, 311t Phencyclidine, 342t Phenobarbital, 104f, 280t Phenotype matching, extended, 206 Phenytoin/fosphenytoin, 279t, 423t Phosphate deficiency, 152t Phosphate enema, 179 Phosphorus, daily requirement, 43t Photodrug reaction, 123t Phototherapy, for hyperbilirubinemia, 413f, 414t Physical abuse, 141–142 Pigtail catheter placement, 460t, 461–462 Pinna laceration, 31t Piperacillin, 219t, 437t Piperacillin–tazobactam, 219t Pityriasis alba, 112t Pityriasis rosea, 112t Plan B, 59 Plaque, 109 Plasma glucose, 143t Plasma transfusion, 207, 435t Plasma volume (PV), 208 Platelet disorders, 203f, 427t Platelet transfusion, 206t, 207, 435t Pleural effusions, 239t Pleural fluid analysis, 236t Pneumococcal vaccine, 213t, 214t Pneumococcus, 125, 127 Pneumomediastinum, 461t

496

Index

Pneumonia community-acquired complications, 237 definition, 234 diagnosis, 235–236, 236t etiologic agents, 234–235t inpatient management, 237–239 outpatient management, 237t risk factors, 235t in cystic fibrosis, 235t, 318t, 319 nosocomial or hospital-associated, 239 Pneumopericardium, 461t Pneumothorax in cystic fibrosis, 320t needle decompression, 21, 21t in neonates, 460t Poisoning antidotes, 345–346t commonly ingested substances, 340 history, 341 initial evaluation, 340f physical exam findings, 342–344t secondary survey, 341 substances detectable with conventional radiography, 342t substances with unique odors, 341t toxidromes, 344–345t urine color changes associated with, 342t urine testing, 342t Poliovirus vaccine, 212t, 215t Polyarteritis nodosa, 332t Polycythemia clinical presentation, 428, 429t definition, 428 differential diagnosis, 428t exchange transfusion for, 430t. See also Exchange transfusion management, 429, 429t in nonphysiologic hyperbilirubinemia, 412t Polymyositis, 329t Portal hypertension, 172 Potassium conversion, 43t daily requirement, 43t, 153 ECG changes and, 76 Potassium channel blockers, as antiarrhythmics, 77t Potts shunt, 81 Poxvirus, 118t PRBCs (packed red blood cells), 206t, 433t, 434t Precocious puberty, 151 Prednisolone adverse effects, 316t for asthma, 316t equivalencies, 9t for status asthmaticus, 91t Prednisone adverse effects, 316t for asthma, 316t as chemotherapy agent, 299t equivalencies, 9t for status asthmaticus, 91t

Preeclampsia, 356 Pregabalin, 280t Pregestimil, 157t Pregnancy diabetes mellitus during, 355, 357t, 399t fetal chromosomal abnormalities screening during, 354, 358, 358t fetal lung maturity evaluation during, 355, 355t genetic diagnosis during, 358–359 hypertension in, 356, 356t immunization during, 216t teratogenic agents in, 187, 423–424t ultrasound screening during, 354 Prehn’s sign, 132t Prenatal care. See Pregnancy Preoperative orders, 1 Prescriptions, essential components, 2 Pressure regulated volume control (PRVC, VC+), 87t Pressure support (PS) ventilation, 374t Preterm infants admission orders, 372–373 cardiovascular care, 370–371t catch-up times, 4 CSF profiles, 442t developmental follow-up, 378 discharge criteria, 376 discharge planning considerations, 376–377t feeding enteral, 39 formulas, 46 total parenteral nutrition. See Total parental nutrition (TPN) vitamin and mineral supplementation, 35t fluid/electrolyte management, 371t growth chart, 50f hypothyroxinemia in, 394–395, 394f infection control, 372t nitric oxide administration, 373 nutrition, 372t osteopenia in, 401–403 respiratory care, 370t retinopathy of prematurity in, 454–455, 454f RSV prophylaxis, 378 secondary immunodeficiency in, 64t sepsis evaluation and empiric treatment, 370f surfactant administration, 373–374t ventilation modes, 374–375t PRICEMMMS mnemonic, for treatment of acute musculoskeletal injury, 301–302 Primary immunodeficiency, 63, 63t Primitive reflexes, 270t, 271–272t Progress note (SOAP note), 1–2 Promethazine, 292t Propofol, 18, 19t, 103t Propranolol, 282t Propylthiouracil, 149 Prostaglandin E1, 384–385 Protein, DRI by age, 41–42t Protein hydrolysate formulas, 46 Proteinuria, 255t, 257–258 Proton pump inhibitors (PPIs), 169 Pseudohyperkalemia, 158

Index Pseudohypoaldosteronism, 392t Pseudohypokalemia, 160 Pseudomonas, 127 pSLE (pediatric systemic lupus erythematosus), 329t, 331–332 Psoriasis, 113t, 391t Pubarche, 151–152 Puberty, 151–152 Pulmocare, 157t Pulmonary air leak, in neonate complications, 460–461t management, 460–461t pathophysiology, 459–460 procedures, 461–462 risk factors, 459 syndromes, 460–461t Pulmonary artery banding, 82 Pulmonary atresia with intact ventricular septum (PA/IVS), 69–70, 69f Pulmonary blood flow, abnormal, 381 Pulmonary function testing (spirometry), 325–326 Pulmonary hypoplasia, 475–477t Pulmonary interstitial emphysema, 461t Pustule, 109 PV (plasma volume), 208 Pyloric stenosis, 140 R Rabies prophylaxis, 135 Radial head subluxation, 305 Radionuclide cystogram (RNC), 242t Radius fractures, 305t Ranitidine, 61t Rastelli operation, 82 Reactive arthritis, 327t Red blood cells (RBCs) normal values, 196t transfusion, 206, 206t, 208 in urine. See Hematuria Red urine, 252–254, 253f Refeeding syndrome, 59 Rehydralyte, 156t Renal flow study, pre-procedural diet, 5t Renal salt wasting (cerebral salt wasting), 150t Renal scan, pre-procedural diet, 5t Renal stones, 140 Renal ultrasound, 242t Respiratory acidosis, 165–168 Respiratory alkalosis, 165–168, 191t Respiratory distress, in neonates, 353t Respiratory distress syndrome (RDS), 458–459t Respiratory failure classification, 83 clinical predictors, 83 management mechanical ventilation. See Mechanical ventilation noninvasive ventilation modalities, 85–86t oxygen delivery modes, 84–85t pathophysiology, 84f workup algorithm, 83f

497

Respiratory syncytial virus (RSV), prophylaxis, 378 Restrictive lung disease, 326t Resuscitation, neonatal. See Neonatal resuscitation Retinitis pigmentosa, 192 Retinoic acid, 424t Retinopathy of prematurity (ROP), 454–455, 454f Retropharyngeal abscess, 136–137t rhDNase, 321t Rhinitis, 127 Rickets, 152t Rickettsia rickettsii, 115t Rifampin, 226t Rifamycins, 226t Right ventricular hypertrophy (RVH), 75 Riley–Day syndrome, 453t RNC (radionuclide cystogram), 242t Rocky Mountain spotted fever, 115t Romberg test, 269 Root reflex, 272t Roseola (exanthem subitum), 116t Ross procedure, 82 Rotavirus, 213t, 214t Rotten egg odor, 341t Rovsing’s sign, 139 Rubella (German measles), 116t Rubeola (measles), 116t Rumination, 169 S Salicylism, 345t Salter-Harris fracture classification, 302, 302f Sandifer syndrome, 169 Sarcoptes scabiei, 119–120t Sarnat stages of hypoxic-ischemic encephalopathy, 451t Scabies, 56, 119–120t Scale, 109 Scalp laceration, 30t Scapular fracture, 303t Scar, 109 Scarlet fever, 114t Schwartz formula, for GFR estimate, 250 Scleroderma, 329t Screening recommendations, 209–211t, 212t Scrotum laceration of, 31t pain and swelling in, 131–132t Seborrheic dermatitis, 111t, 391t Secondary immunodeficiency, 64–65t Sedation continuum of, 15 discharge criteria, 19, 20t emergency equipment, 16, 16t medications, 16–19, 17t, 18–19t monitoring following, 19 patient preparation, 15–16, 15t, 16t Sedative-hypnotics, toxidrome, 345t Segmental fracture, 302

498

Index

Seizures. See also Epilepsy breakthrough in known epilepsy, 277 classification, 275–276t evaluation of first nonfebrile, 276–277 neonatal, 188, 448–449 in poisonings, 343t practice parameters, 275 simple febrile, 276 Selenium sulfide shampoo, 118t Self-mutilation, 192 Senna, 179 Senning procedure, 82 Sensory testing, 269 Sepsis, 94t in preterm infant, evaluation and empiric treatment, 370f in term infant, evaluation and empiric treatment, 369f Septic arthritis, 134t, 327t Septic shock, 94t Serum sickness-like reaction, 121t Severe sepsis, 94t Sever’s disease, 310t Sexual development, 151–152 Sexually transmitted infections, 53–54t, 212t Shaken baby (shaken impact) syndrome, 142 Shingles (herpes zoster), 117t Shock classifications, 95–96t clinical findings, 94t clinical pearls, 96, 98 definition, 93 management, 95–96t, 97f in sepsis, 94 stages, 93 Shoulder dislocation, 303t Shoulder fractures, 303t SI units, laboratory value conversion table, 8t SIADH (syndrome of inappropriate antidiuretic hormone), 150t Sick euthyroid syndrome, 149 Sickle cell disease diagnosis, 199 health maintenance in, 200t immunizations in, 217t pneumonia in, 235t secondary immunodeficiency in, 64t signs and symptoms, 199–200t treatment, 199–200t Similac Advance, 156t Similac HMF, 48t Simple febrile seizure, 276 Simple partial seizure, 275t. See also Seizures SIMV (synchronized intermittent mandatory ventilation), 87t, 88t, 374t Sinding-Larsen-Johansson syndrome, 308 Sinus tachycardia, 75t Sinus venosus, 66 Sinusitis, 128, 319t SIRS (systemic inflammatory response syndrome), 94t Sjögren’s syndrome, 329t

SJS (Stevens-Johnson syndrome), 122t Skin infections, 233–234 Skin lesions, 109–110t Sleeve fracture, 308t Snoring, 129f Snout reflex, 270t SOAP equipment, for sedation, 16 SOAP note (progress note), 1–2 Sodium, 43t, 153 Sodium bicarbonate, in neonatal resuscitation, 365t Sodium channel blockers, 77t Soft tissue infections, 233–234 Soy-based formulas, 46 Specific gravity, urine, 254t Spinal accessory nerve, 268t Spinal cord compression, 289–290 Spinal muscular atrophy type 1, 453t Spirometry (pulmonary function testing), 325–326 Spironolactone, 174 Splenic sequestration crisis, 199t Spondylolisthesis, 301 Spondylolysis, 301 Sporotrichosis, 227 Sprains, 301–302 Staphylococcal scalded skin syndrome, 115t, 389t Staphylococcus aureus in cellulitis, 114t in cystic fibrosis, 318t, 322–323t in folliculitis, 113t in impetigo, 113t in mastoiditis, 127 in skin abscess, 114t superinfection in eczema, 110t StAR (acute steroid regulatory protein) deficiency, 392t Startle reflex, 271t STATS, for intubation preparation, 86 Status asthmaticus clinical respiratory score, 90–91t definition, 90 management advanced, 92–93 clinical pearls, 93 first-line, 91t second-line, 92t risk factors for ICU admission and sudden death, 90 symptoms, 90 Status epilepticus, 102–103 causes, 103 clinical pearls, 103 management algorithm, 104f medications, 103t types, 103 workup, 105f Status migrainosus, 283 Steatohepatitis, 171t Stevens-Johnson syndrome (SJS), 122t Stiff-man syndrome, 449t Stimulant laxatives, 179 Stimulus-evoked myoclonus, 449t Strains, 301–302

Index Streptococcus pneumonia, 128 Streptococcus pyogenes, 113t Streptomycin, 224t Stress factors, energy expenditure, 41t Stridor, 135–137, 352t Stroke, in sickle cell disease, 200t Subclavian vein (SCV), 26 Substance abuse in pregnancy, 357t street names of drugs of abuse, 341t urine testing for, 342t Sucrose, oral, 12, 456 Sulfonamides, 225t Sulphur odor, 190t Sumatriptan, 282t Superior cavopulmonary anastomosis, 81, 81f Suplena, 157t Supracondylar fracture, elbow, 304t Supraventricular tachycardia (SVT), 75t Surfactant, for preterm infants, 373–374t Sutures cranial, 4 for laceration repair, 30–31t Swallow study, pre-procedural diet, 5t Sweat chloride test, 317, 317t Sweaty feet odor, 190t Synchronized intermittent mandatory ventilation (SIMV), 87t, 88t, 374t Syncope, 79 Syndrome of inappropriate antidiuretic hormone (SIADH), 150t Synovial fluid, evaluation, 327t Syphilis, 54t, 212t Systemic inflammatory response syndrome (SIRS), 94t Systemic lupus erythematosus, 329t, 331–332, 388t T Tachycardia, 75t, 76f Takayasu arteritis, 332t Tanner stages, 151–152t TAPVR (total anomalous pulmonary venous return), 68, 68f TAR (thrombocytopenia-absent radii) syndrome, 428 TBV (total blood volume), 208 Teeth, 107, 107f, 108 Teething, 107 Temperature, conversion formulas, 4 TEN (toxic epidermal necrolysis), 122t Tension type headache, 283 Teratogens, 187, 423–424t Terbutaline, 92t Terconazole, 53t Testicular torsion, 131–132t Tetanus prophylaxis, 32, 32t Tetracycline, 223t Tetracyclines, 223t Tetralogy of Fallot (TOF), 67–68, 67f, 81, 81f TGA (transposition of the great arteries), 68, 68f, 81, 82, 82f α-Thalassemia, 199t

499

β-Thalassemia major, 199t β-Thalassemia minor, 197t, 199t Thelarche, 151–152 Thiazides, for bronchopulmonary dysplasia, 467t Thioguanine, 298t Thrombocytopathy, 203f Thrombocytopenia alloimmune, 426–427t autoimmune, 426–427t congenital, 428 with decreased platelet survival, 427t differential diagnosis, 203f, 425f idiopathic, 202 in inborn errors of metabolism, 189 in neonates, 425–428 Thrombocytopenia-absent radii (TAR) syndrome, 428 Thromboembolism, 205t Thrombophilia, 205t Thumb fractures, 306–307t Thyroid storm, 149 Thyroiditis, 147, 149 Ticarcillin, 219t Ticarcillin/clavulanic acid, 323t Ticarcillin–clavulanate, 219t Tinea capitis, 118t Tinea corporis, 118t Tinea cruris, 118t Tinea pedis, 118t Tinea versicolor, 119t Tinidazole, 53t Tobramycin, 224t, 322t, 438t Toddler fracture, 309t TOF (tetralogy of Fallot), 67–68, 67f, 81, 81f Tonic neck reflex, 272t Topical anesthetics, 11–12t Topiramate, 280t, 282t Total anomalous pulmonary venous return (TAPVR), 68, 68f Total blood volume (TBV), 208 Total parental nutrition (TPN) carbohydrate/amino acid requirements and rate, 42, 43t complications, 44 electrolyte requirements, 43t energy requirement, 40–41, 40t, 41t final check, 44–45 intravenous fluid requirement, 39–40, 40t laboratory monitoring, 45t lipid requirements and rate, 42 protein requirement, 41–42t stress factors, 41t vitamins, minerals, and trace elements, 44t Toxic epidermal necrolysis (TEN), 122t Toxic shock syndrome, 115t Toxicology. See also Poisoning collection tube colors, 7t SI laboratory value conversion table, 8t Toxidromes, 344–345t Trace elements, in total parenteral nutrition, 44t Traction apophysitis of proximal humerus, 303t Transient neonatal pustular melanosis, 386t

500

Index

Transient synovitis, 134t Transient tachypnea of the newborn (TTN), 458, 458t Transposition of the great arteries (TGA), 68, 68f, 81, 82, 82f Trauma, nonaccidental, 141–142 Traumatic arthritis, 327t Traumatic brain injury, 101–102 Treponema pallidum, 54t Triamcinolone, 10t Triamcinolone acetonide, 123t, 124t Trichomonas vaginalis infection, 52–53t Tricuspid atresia, 69, 69f Tricyclic antidepressants antidote, 346t poisoning/overdose, 347t toxidrome, 345t Trigeminal nerve, 268t Trimethoprim/sulfamethoxazole, 225t, 322t Trisomy 13, 422–423t Trisomy 18, 422–423t Trisomy 21 (Down syndrome), 64t, 183, 184–185t, 422–423t Trochlear nerve, 268t Truncus arteriosus, 68–69, 68f, 82 Trunk laceration, 31t Tube colors, for lab samples, 6, 7t Tuberculosis screening, 212t Tuberculous arthritis, 327t Tumor, 109 Tumor lysis syndrome, 289 Turner syndrome, 64t Tympanostomy tube, 126 Type 1 diabetes mellitus. See Diabetes mellitus Type 2 diabetes mellitus. See Diabetes mellitus U Ulcer, 109 Ulcerative colitis clinical features, 180t definition, 179 endoscopic features, 181t epidemiology, 180t prognosis, 182 radiographic features, 181t treatment, 181–182, 182t Ulna fractures, 306t Ultrasonography cranial, 274t, 376t fetal, 354, 357, 358 pre-procedural diet, 5t Ultraviolet B exposure, secondary immunodeficiency and, 65t Umbilical artery catheterization (UAC), 27–29, 28f, 28t, 365 Umbilical blood sampling, percutaneous, 359 Umbilical vein catheterization (UVC), 28f, 29–30, 29t, 365–366 Unconjugated (indirect) hyperbilirubinemia, 175–176 Unfractionated heparin (UFH), 205t Upper extremity fracture, 303–306t

Upper GI series, pre-procedural diet, 5t Upper motor neuron lesions, 272t, 452t Urea cycle, 194f Urea cycle defects, 191t, 193–194t, 195f Uremia, secondary immunodeficiency in, 64t Urinalysis, 254–256t Urinary tract infections definition, 239 diagnosis, 240, 241t etiologic agents, 240 imaging modalities, 242t management, 241 prevention, 241 risk factors, 239 Urine discoloration, 190t, 342t organic acid analysis, 193 unusual odors, 189–190t Urine culture, 241t Ursodeoxycholic acid (UDCA), 420t Urticaria, 61–62, 120t V Vaccines/vaccinations. See Immunizations Vaginitis, 52–53t Vagus nerve, 268t Valacyclovir, 55t Valgus, 301 Valproic acid, 280t, 282t, 423t Valvotomy, 82 Valvuloplasty, 82 Vancomycin, 222t, 323t, 438t Varicella zoster virus, 116t, 117t Varicella zoster virus immunization, 212t, 215t Varices, 173 Varus, 301 Vasculitides, 332t, 334f Vasopressin, for shock, 96, 96t VATER/VACTERL association, 187 Vaughan-Williams classification, antiarrhythmics, 77t Ventricular septal defect (VSD), 66 Venturi mask, for O2 delivery, 84t Verruca vulgaris, 117t Very low birth weight infants, 369. See also Preterm infants Vesicle, 109 Vesicoureteral reflux (VUR), 262–263 Vinblastine, 299t Vincristine, 299t Vision screening, 212t Vitamin A, for prevention of bronchopulmonary dysplasia, 466t Vitamin D deficiency, 152t, 400t, 402t Vitamins, in total parenteral nutrition, 44t Voiding cystourethrography (VCUG), 5t, 242t, 262 Volume guarantee (VG) ventilation, 374t Volvulus, 410t Vomiting/nausea chemotherapy-related, 291–292 with enteral feeding, 39t in neonates, 352t von Willebrand disease, 204t

Index W Warfarin, 205t, 423t Waterlow criteria, malnutrition, 49 Waterston shunt, 81 Weakness, childhood, 286–287t Wegener granulomatosis, 329t, 332t Weight by age for boys, 2t by age for girls, 3t at birth, 3 conversion, 4 by gestational age, 50f rate of gain, 3 Werdnig–Hoffmann disease, 453t West syndrome, 278t Wheal, 109 Wheezing, 312 White blood cells (WBCs) normal values, 201t in urine, 256t Wilson’s disease, 171t

Wintergreen odor, 341t Wiskott-Aldrich syndrome, 427t Withdrawal, 342–344t World Health Organization (WHO), oral rehydration solution, 156t Wounds repair, 30–31t, 30–32 tetanus prophylaxis, 32, 32t Wrist fracture, 306t X X-linked hypophosphatemia, 152t Xanthines, for neonatal apnea, 469 Y Yeast vaginitis, 52–53t Z Zafirlukast, 316t Zinc, oral, 156 Zolmitriptan, 282t Zonisamide, 280t

501

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