Nelson Textbook of Pediatrics 20Th Edition Robert M Kliegman Full Chapter
Nelson Textbook of Pediatrics 20Th Edition Robert M Kliegman Full Chapter
Nelson Textbook of Pediatrics 20Th Edition Robert M Kliegman Full Chapter
Robert M. Kliegman, MD
Professor and Chair Emeritus
Department of Pediatrics
Medical College of Wisconsin
Milwaukee, Wisconsin
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vi
Contributors vii
Marilyn C. Augustyn, MD
Professor of Pediatrics
Boston University School of Medicine
Boston Medical Center
Boston, Massachusetts
Impact of Violence on Children
viii Contributors
Mark L. Batshaw, MD Susanne M. Benseler, MD, PhD Joshua A. Blatter, MD, MPH
Professor and Chairman Associate Professor, Faculty of Medicine Instructor in Pediatrics
Department of Pediatrics University of Calgary Division of Pediatric Allergy, Immunology, and
Associate Dean, Academic Affairs Pediatric Rheumatologist, Section Chief Pulmonary Medicine
George Washington University Rheumatology Associate Director, Pediatric Lung Transplantation
School of Medicine and Health Sciences Alberta Children’s Hospital Center
Executive Vice-President Calgary, Alberta, Canada Washington University School of Medicine in
Chief Academic Officer and Physician-in-Chief Central Nervous System Vasculitis St. Louis
Children’s National Medical Center St. Louis, Missouri
Washington, DC Daniel Bernstein, MD Congenital Disorders of the Lung
Intellectual Disability Alfred Woodley Salter and Mabel Smith Salter
Endowed Professor in Pediatrics Archie Bleyer, MD, FRCP (Glasg)
Nerissa S. Bauer, MD, MPH Stanford University School of Medicine Clinical Research Professor
Assistant Professor Director, Division of Pediatric Cardiology Knight Cancer Center
Department of General and Community Pediatrics Lucile Packard Children’s Hospital Oregon Health & Science University
Section of Children’s Health Services Research Palo Alto, California Chair, Institutional Review Board for St. Charles
Indiana University School of Medicine Cardiac Development Health System
Indianapolis, Indiana The Fetal to Neonatal Circulatory Transition Portland, Oregon;
Developmental-Behavioral Screening and History and Physical Examination Professor of Pediatrics
Surveillance Laboratory Evaluation University of Texas MD Anderson Cancer Center
Epidemiology and Genetic Basis of Congenital Heart Houston, Texas
Michelle L. Bayer, MD Disease Principles of Treatment (Cancer and Benign Tumors)
Resident Physician Evaluation and Screening of the Infant or Child with The Leukemias
Department of Dermatology Congenital Heart Disease Congenital Disorders of the Lung
Medical College of Wisconsin Acyanotic Congenital Heart Disease: Left-to-Right
Children’s Hospital of Wisconsin Shunt Lesions Steven R. Boas, MD, FAAP, FACSM
Milwaukee, Wisconsin Acyanotic Congenital Heart Disease: Obstructive Director, The Cystic Fibrosis Center of Chicago
Disorders of the Mucous Membranes Lesions President and CEO, The Cystic Fibrosis Institute
Acyanotic Congenital Heart Disease: Regurgitant Glenview, Illinois;
Richard E. Behrman, MD Lesions Clinical Associate Professor of Pediatrics
Nonprofit Healthcare and Educational Consultants Cyanotic Congenital Heart Disease: Evaluation of Northwestern University Feinberg School of
to Medical Institutions the Critically Ill Neonate with Cyanosis and Medicine
Santa Barbara, California Respiratory Distress Chicago, Illinois
Overview of Pediatrics Cyanotic Congenital Heart Lesions: Lesions Emphysema and Overinflation
Associated with Decreased Pulmonary Blood α1-Antitrypsin Deficiency and Emphysema
Michael J. Bell, MD Flow Other Distal Airway Diseases
Professor Cyanotic Congenital Heart Disease: Lesions Skeletal Diseases Influencing Pulmonary Function
Departments of Critical Care Medicine, Neurologic Associated with Increased Pulmonary Blood Flow
Surgery, and Pediatrics Other Congenital Heart and Vascular Walter O. Bockting, PhD
University of Pittsburgh School of Medicine Malformations Professor of Medical Psychology (in Psychiatry and
Director, Pediatric Neurocritical Care Pulmonary Hypertension Nursing)
Director, Pediatric Neurotrauma Center General Principles of Treatment of Congenital Heart Research Scientist, New York State Psychiatric
University of Pittsburgh Medical Center Disease Institute
Pittsburgh, Pennsylvania Infective Endocarditis Division of Gender, Sexuality, and Health
Neurologic Emergencies and Stabilization Rheumatic Heart Disease Department of Psychiatry
Heart Failure Columbia University College of Physicians and
John W. Belmont, MD, PhD Pediatric Heart and Heart-Lung Transplantation Surgeons
Professor Diseases of the Blood Vessels (Aneurysms and New York, New York
Departments of Molecular and Human Genetics, Fistulas) Sexual Identity Development
Pediatrics, and Pathology and Immunology
Baylor College of Medicine Zulfiqar Ahmed Bhutta, MBBS, PhD, Neal F. Boerkoel, MD, PhD
Houston, Texas FRCPCH, FAAP National Human Genome Research Institute
Genetics of Common Disorders Professor of Paediatrics, Nutritional Sciences, and National Institutes of Health
Public Health Bethesda, Maryland
Daniel K. Benjamin Jr., MD, PhD, University of Toronto Faculty of Medicine Genetic Approaches to Rare and Undiagnosed
MPH Robert Harding Chair in Global Child Health and Diseases
Professor of Pediatrics Policy
Division of Pediatric Infectious Diseases Co-Director, SickKids Centre for Global Child Natalija Bogdanovic, MD
Faculty Associate Director, Duke Clinical Research Health Department of Psychiatry
Institute The Hospital for Sick Children Boston Medical Center
Duke University Medical Center Toronto, Ontario, Canada; Boston, Massachusetts
Durham, North Carolina Founding Director, Centre of Excellence in Women Mood Disorders
Principles of Antifungal Therapy and Child Health
Candida The Aga Khan University, South Central Asia and Mark Boguniewicz, MD
East Africa Professor of Pediatrics
Michael J. Bennett, PhD, FRCPath, Karachi, Pakistan Division of Pediatric Allergy-Immunology
FACB Innovations in Addressing Child Health and University of Colorado School of Medicine
Professor of Pathology and Laboratory Medicine Survival in Low-Income Settings National Jewish Health
University of Pennsylvania Perelman School of Salmonella Denver, Colorado
Medicine Acute Gastroenteritis in Children Ocular Allergies
Director, Michael J. Palmieri Metabolic Disease Adverse Reactions to Drugs
Laboratory Samra S. Blanchard, MD
Children’s Hospital of Philadelphia Associate Professor of Pediatrics Daniel J. Bonthius, MD, PhD
Philadelphia, Pennsylvania University of Maryland School of Medicine Professor of Pediatrics and Neurology
Disorders of Mitochondrial Fatty Acid ß-Oxidation Baltimore, Maryland University of Iowa School of Medicine
Peptic Ulcer Disease in Children Iowa City, Iowa
Lymphocytic Choriomeningitis Virus
Contributors ix
Brett J. Bordini Cynthia Etzler Budek, MS, APN/NP, Miguel M. Cabada, MD, MSc
Assistant Professor CPNP-AC/PC Research Associate, Tropical Medicine Institute
Department of Pediatrics Pediatric Nurse Practitioner Universidad Peruana Cayetano Heredia
Medical College of Wisconsin Department of Pulmonary and Critical Care Lima, Peru;
Pediatric Hospitalist Medicine Adjunct Instructor of Medicine
Children’s Hospital of Wisconsin Transitional Care/Pulmonary Habilitation Unit Division of Infectious Diseases
Milwaukee, Wisconsin Ann & Robert H. Lurie Children’s Hospital of University of Texas Medical Branch at Galveston
Plastic Bronchitis Chicago Galveston, Texas
Chicago, Illinois Echinococcosis (Echinococcus granulosus and
Kenneth M. Boyer, MD Chronic Severe Respiratory Insufficiency Echinococcus multilocularis)
Woman’s Board Professor and Chairman
Rush Medical College of Rush University E. Stephen Buescher, MD Derya Caglar, MD
Chicago, Illinois Professor of Pediatrics Assistant Professor
Toxoplasmosis (Toxoplasma gondii) Eastern Virginia Medical School Department of Pediatrics
Medical Director, Infection Control University of Washington School of Medicine
Amanda M. Brandow, DO, MS Medical Director, Clinical Microbiology Attending Physician
Associate Professor of Pediatrics Laboratory Division of Emergency Medicine
Division of Pediatric Hematology/Oncology Children’s Hospital of the King’s Daughters Seattle Children’s Hospital
Medical College of Wisconsin Norfolk, Virginia Seattle, Washington
Milwaukee, Wisconsin Diphtheria (Corynebacterium diphtheriae) Drowning and Submersion Injury
Polycythemia
Non-Clonal Polycythemia Supinda Bunyavanich, MD, MPH Mitchell S. Cairo, MD
Anatomy and Function of the Spleen Assistant Professor Professor
Splenomegaly Departments of Pediatrics and Genetics and Departments of Pediatrics, Medicine, Pathology,
Hyposplenism, Splenic Trauma, and Splenectomy Genomic Sciences Microbiology and Immunology and Cell
Jaffe Food Allergy Institute Biology and Anatomy
†David Branski, MD Mindich Child Heath and Development Institute New York Medical College
Professor Emeritus Ichan School of Medicine at Mount Sinai Chief, Division of Pediatric Hematology, Oncology,
The Hebrew University–Hadassah School of New York, New York and Stem Cell Transplantation
Medicine Diagnosis of Allergic Disease Maria Fareri Children’s Hospital at Westchester
Jerusalem, Israel Principles of Treatment of Allergic Disease Medical Center
Disorders of Malabsorption New York Medical College
Chronic Diarrhea Carey-Ann D. Burnham, PhD Valhalla, New York
Assistant Professor of Pathology and Immunology Lymphoma
David T. Breault, MD, PhD Assistant Professor of Pediatrics
Assistant Professor of Pediatrics Washington University School of Medicine in St. Lauren E. Camarda, MD
Harvard Medical School Louis Instructor in Pediatrics
Division of Endocrinology Medical Director, Clinical Microbiology Northwestern University Feinberg School of
Boston Children’s Hospital Barnes-Jewish Hospital Medicine
Boston, Massachusetts St. Louis, Missouri Division of Pediatric Pulmonary Medicine
Diabetes Insipidus Diagnostic Microbiology Ann & Robert H. Lurie Children’s Hospital of
Other Abnormalities of Arginine Vasopressin Chicago
Metabolism and Action Gale R. Burstein, MD, MPH Chicago, Illinois
Clinical Professor Wheezing, Bronchiolitis, and Bronchitis
William J. Britt, MD Department of Pediatrics
Charles A. Alford Professor of Pediatric Infectious State University of New York at Buffalo Bruce M. Camitta, MD
Diseases School of Medicine and Biomedical Sciences Rebecca Jean Slye Professor of Pediatrics
Professor of Pediatrics and Microbiology and Commissioner Division of Pediatric Hematology/Oncology
Neurobiology Erie County Department of Health Medical College of Wisconsin
University of Alabama Birmingham School of Buffalo, New York Midwest Children’s Cancer Center
Medicine Adolescent Physical and Social Development Milwaukee, Wisconsin
Birmingham, Alabama The Epidemiology of Adolescent Health Problems Polycythemia
Cytomegalovirus Delivery of Healthcare to Adolescents Non-Clonal Polycythemia
The Breast Anatomy and Function of the Spleen
Angela R. Bryan, MD Menstrual Problems Splenomegaly
Fellow in Pediatric Rheumatology Contraception Hyposplenism, Splenic Trauma, and Splenectomy
Duke University Health System Sexually Transmitted Infections Anatomy and Function of the Lymphatic System
Durham, North Carolina Abnormalities of Lymphatic Vessels
Juvenile Idiopathic Arthritis Amaya L. Bustinduy, MD, MPH, FAAP, Lymphadenopathy
FRCPCH
Rebecca H. Buckley, MD Paediatric Infectious Diseases Research Group Angela J.P. Campbell, MD, MPH
J. Buren Sidbury Professor of Pediatrics (PIDRG) Medical Officer
Professor of Immunology St. George’s University of London Epidemiology and Prevention Branch, Influenza
Duke University School of Medicine London, United Kingdom Division
Durham, North Carolina Schistosomiasis (Schistosoma) National Center for Immunization and Respiratory
Evaluation of Suspected Immunodeficiency Flukes (Liver, Lung, and Intestinal) Diseases
The T-, B-, and NK-Cell Systems Centers for Disease Control and Prevention
T Lymphocytes, B Lymphocytes, and Natural Killer Atlanta, Georgia
Cells Influenza Viruses
Primary Defects of Antibody Production Parainfluenza Viruses
Primary Defects of Cellular Immunity
Primary Combined Antibody and Cellular
Immunodeficiencies
†Deceased
x Contributors
David R. DeMaso, MD André A.S. Dick, MD, MPH, FACS Kelly A. Dougherty, PhD
George P. Gardner and Olga E. Monks Professor of Assistant Professor of Surgery Assistant Professor of Pediatrics
Child Psychiatry Division of Transplantation University of Pennsylvania Perelman School of
Professor of Pediatrics University of Washington School of Medicine Medicine
Harvard Medical School Division of Transplant Surgery Division of Gastroenterology, Hepatology, and
Psychiatrist-in-Chief and Chairman of Psychiatry Seattle Children’s Hospital Nutrition
The Leon Eisenberg Chair in Psychiatry Seattle, Washington Children’s Hospital of Philadelphia
Boston Children’s Hospital Intestinal Transplantation in Children with Philadelphia, Pennsylvania
Boston, Massachusetts Intestinal Failure Nutritional Requirements
Assessment and Interviewing
Psychological Treatment of Children and Adolescents Brianne Z. Dickey, MD Alexander Doyle, MBBS
Psychopharmacology Resident Physician HHMI Postdoctoral Research Fellow
Psychotherapy Department of Dermatology Institute of Genetic Medicine
Psychiatric Hospitalization Medical College of Wisconsin Johns Hopkins University School of Medicine
Somatic Symptom and Related Disorders Milwaukee, Wisconsin Baltimore, Maryland
Rumination and Pica Morphology of the Skin Marfan Syndrome
Motor Disorders and Habits Evaluation of the Patient
Mood Disorders Eczematous Disorders Daniel A. Doyle, MD
Suicide and Attempted Suicide Photosensitivity Chief, Division of Endocrinology
Disruptive, Impulse-Control, and Conduct Disorders Diseases of the Epidermis Alfred I. duPont Hospital for Children
Autism Spectrum Disorder Nemours Children’s Health System
Childhood Psychoses Harry C. Dietz III, MD Wilmington, Delaware
Victor A. McKusick Professor of Medicine and Hormones and Peptides of Calcium Homeostasis
Mark R. Denison, MD Genetics and Bone Metabolism
Craig-Weaver Professor of Pediatrics Departments of Pediatrics, Medicine, and Hypoparathyroidism
Division of Pediatric Infectious Disease Molecular Biology and Genetics Pseudohypoparathyroidism (Albright Hereditary
Vanderbilt University Medical Center Investigator, Howard Hughes Medical Institute Osteodystrophy)
Nashville, Tennessee Director, William S. Smilow Center for Marfan Hyperparathyroidism
Coronaviruses Syndrome Research
Institute of Genetic Medicine Jefferson J. Doyle, MBBChir, MHS,
Arlene E. Dent, MD, PhD Johns Hopkins University School of Medicine MA
Assistant Professor of Pediatrics Baltimore, Maryland Postdoctoral Research Fellow
Division of Infectious Diseases Marfan Syndrome Institute of Genetic Medicine
Case Western Reserve University School of Johns Hopkins University School of Medicine
Medicine Lydia J. Donoghue, MD Baltimore, Maryland
Cleveland, Ohio Director, Trauma Center Marfan Syndrome
Ascariasis (Ascaris lumbricoides) Children’s Hospital of Michigan
Trichuriasis (Trichuris trichiura) Detroit, Michigan Patrick C. Drayna, MD
Enterobiasis (Enterobius vermicularis) Tumors of the Digestive Tract Assistant Professor of Pediatrics
Strongyloidiasis (Strongyloides stercoralis) Division of Pediatric Emergency Medicine
Lymphatic Filariasis (Brugia malayi, Brugia timori, Patricia A. Donohoue, MD Medical College of Wisconsin
and Wuchereria bancrofti) Professor of Pediatrics Children’s Hospital of Wisconsin
Other Tissue Nematodes Chief, Section of Endocrinology and Diabetes Milwaukee, Wisconsin
Toxocariasis (Visceral and Ocular Larva Migrans) Medical College of Wisconsin Evaluation of the Sick Child in the Office and Clinic
Trichinosis (Trichinella spiralis) Program Director, Endocrine and Diabetes
Children’s Hospital of Wisconsin Stephen C. Dreskin, MD, PhD
Robert J. Desnick, MD, PhD Milwaukee, Wisconsin Professor of Medicine and Immunology
Dean for Genetics and Genomic Medicine Development and Function of the Gonads Division of Allergy and Clinical Immunology
Professor and Chair Emeritus, Genetics and Hypofunction of the Testes Department of Medicine
Genomic Sciences Pseudoprecocity Resulting from Tumors of the Testes University of Colorado School of Medicine
Professor, Departments of Pediatrics, Oncological Gynecomastia Aurora, Colorado
Sciences, and Obstetrics, Gynecology, and Hypofunction of the Ovaries Urticaria (Hives) and Angioedema
Reproductive Science Pseudoprecocity Resulting from Lesions of the Ovary
Icahn School of Medicine at Mount Sinai Disorders of Sex Development Beth A. Drolet, MD
New York, New York Professor of Dermatology
Lipidoses (Lysosomal Storage Disorders) Mary K. Donovan, RN, CS, PNP Medical College of Wisconsin
Mucolipidoses Pediatric Nurse Practitioner and Care Coordinator Children’s Hospital of Wisconsin
Disorders of Glycoprotein Degradation and Shriners Hospital for Children Milwaukee, Wisconsin
Structure Shriners Burns Hospital Principles of Therapy (Skin)
The Porphyrias Boston, Massachusetts Hyperpigmented Lesions
Burn Injuries Diseases of Subcutaneous Tissue
Gabrielle A. deVeber, MD, MHSc Cold Injuries Disorders of the Mucous Membranes
Professor of Paediatrics Cutaneous Bacterial Infections
Director, Children’s Stroke Program John P. Dormans, MD Cutaneous Fungal Infections
University of Toronto Faculty of Medicine Professor and The Richard M. Armstrong Jr. Cutaneous Viral Infections
Staff Neurologist Endowed Chair Arthropod Bites and Infestations
Senior Scientist, Child Health Evaluative Sciences Department of Orthopaedic Surgery
The Hospital for Sick Children University of Pennsylvania Perelman School of
Toronto, Ontario, Canada Medicine
Pediatric Stroke Chief, Division of Orthopaedic Surgery
Children’s Hospital of Philadelphia
Anil Dhawan, MD Philadelphia, Pennsylvania
Consultant Paediatric Hepatologist Growth and Development
Pediatric Liver Centre Evaluation of the Child
King’s College London School of Medicine The Hip
King’s College Hospital NSH Foundation Trust Common Fractures
London, United Kingdom
Liver and Biliary Disorders Causing Malabsorption
Contributors xiii
Yigal Dror, MD, FRCP(C) Elizabeth A. Edgerton, MD, MPH Susan Feigelman, MD
Professor of Paediatrics Assistant Professor Professor, Department of Pediatrics
University of Toronto Faculty of Medicine Departments of Pediatrics and Preventive and University of Maryland School of Medicine
Head, Hematology Section Director, Marrow Community Health Baltimore, Maryland
Failure and Myelodysplasia Program George Washington University School of Medicine Overview and Assessment of Variability
The Hospital for Sick Children Division of Emergency Medicine Assessment of Fetal Growth and Development
Toronto, Ontario, Canada Children’s National Medical Center The First Year
The Inherited Pancytopenias Washington, DC The Second Year
Interfacility Transport of the Seriously Ill or Injured The Preschool Years
Howard Dubowitz, MD, MS, FAAP Pediatric Patient Middle Childhood
Professor of Pediatrics
Chief, Division of Child Protection Marie E. Egan, MD Marianne E. Felice, MD
Director, Center for Families Associate Professor of Pediatrics (Respiratory) and Professor
Department of Pediatrics of Cellular and Molecular Physiology Departments of Pediatrics and Obstetrics and
University of Maryland School of Medicine Director, Cystic Fibrosis Center Gynecology
Baltimore, Maryland Yale School of Medicine University of Massachusetts Medical School
Abused and Neglected Children New Haven, Connecticut Principle Investigator, National Children’s Study
Cystic Fibrosis UMass Study Center
J. Stephen Dumler, MD Worcester, Massachusetts
Professor of Pathology and Microbiology and Jack S. Elder, MD, FACS Adolescent Pregnancy
Immunology Chief of Pediatric Urology Adolescent Rape
University of Maryland School of Medicine Massachusetts General Hospital
Baltimore, Maryland Boston, Massachusetts Eric I. Felner, MD, MSCR
Spotted Fever Group Rickettsioses Congenital Anomalies and Dysgenesis of the Kidneys Associate Professor of Pediatrics
Scrub Typhus (Orientia tsutsugamushi) Urinary Tract Infections Division of Pediatric Endocrinology
Typhus Group Rickettsioses Vesicoureteral Reflux Director, Pediatric Endocrinology Fellowship
Ehrlichioses and Anaplasmosis Obstruction of the Urinary Tract Program
Q Fever (Coxiella burnetii) Anomalies of the Bladder Director, Pediatric Clerkships
Neuropathic Bladder Emory University School of Medicine
Aubrey N. Duncan, MD Enuresis and Voiding Dysfunction Atlanta, Georgia
Resident Physician Anomalies of the Penis and Urethra Hormones of the Hypothalamus and Pituitary
Department of Pediatrics Disorders and Anomalies of the Scrotal Contents Hypopituitarism
University of Rochester Medical Center Trauma to the Genitourinary Tract
Rochester, New York Urinary Lithiasis Edward C. Fels, MD
Deformational Plagiocephaly Pediatric and Adult Rheumatology
Dianne S. Elfenbein, MD Rheumatology Associates, PA
Janet Duncan, MSN, CPNP Professor of Pediatrics Portland, Maine
Department of Psychosocial Oncology and Director, Division of Adolescent Medicine Vasculitis Syndromes
Palliative Care St. Louis University School of Medicine
Boston Children’s Hospital St. Louis, Missouri Kora N. Felsch, MD
Dana-Farber Cancer Institute Adolescent Pregnancy Hospitalist
Boston, Massachusetts Cardinal Glennon Children’s Medical Center
Pediatric Palliative Care Stephen C. Eppes, MD, FAAP St. Louis, Missouri
Professor of Pediatrics Breast Concerns
Paula M. Duncan, MD Jefferson Medical College of Thomas Jefferson
Professor University Thomas W. Ferkol Jr., MD
Department of Pediatrics Philadelphia, Pennsylvania; Alexis Hartmann Professor of Pediatrics
University of Vermont College of Medicine Director, Pediatric Infectious Diseases Director, Division of Pediatric Allergy,
Burlington, Vermont Christiana Care Health System Immunology, and Pulmonary Medicine
Maximizing Children’s Health: Screening, Wilmington, Delaware Washington University School of Medicine in
Anticipatory Guidance, and Counseling Lyme Disease (Borrelia burgdorferi) St. Louis
St. Louis, Missouri
Jeffrey A. Dvergsten, MD Jessica Ericson, MD Primary Ciliary Dyskinesia (Immotile Cilia
Assistant Professor of Pediatrics Pediatric Infectious Diseases Fellow Syndrome, Kartagener Syndrome)
Duke University School of Medicine Duke University Medical Center
Division of Pediatric Rheumatology Durham, North Carolina Can H. Ficicioglu, MD, PhD
Duke University Health System Candida Associate Professor
Durham, North Carolina Department of Pediatrics
Treatment of Rheumatic Diseases Alessio Fasano, MD University of Pennsylvania Perelman School of
Visiting Professor of Pediatrics Medicine
Michael G. Earing, MD Harvard Medical School Director, Newborn Metabolic Screening Program
Professor of Internal Medicine and Pediatrics Chief, Division of Pediatric Gastroenterology and Children’s Hospital of Philadelphia
Division of Adult Cardiovascular Medicine and Nutrition Philadelphia, Pennsylvania
Division of Pediatric Cardiology Associate Chief, Department of Pediatrics, Basic, Phenylalanine
Medical College of Wisconsin Clinical, and Translational Research
Director, Wisconsin Adult Congenital Heart Director, Center for Celiac Research Jonathan D. Finder, MD
Disease Program (WAtCH) MassGeneral Hospital for Children Professor of Pediatrics
Children’s Hospital of Wisconsin Boston, Massachusetts University of Pittsburgh School of Medicine
Milwaukee, Wisconsin Celiac Disease (Gluten-Sensitive Enteropathy) Attending Pediatric Pulmonologist
Congenital Heart Disease in Adults Division of Pediatric Pulmonology
Children’s Hospital of Pittsburgh of UPMC
Matthew D. Eberly, MD Pittsburgh, Pennsylvania
Assistant Professor of Pediatrics Bronchomalacia and Tracheomalacia
Uniformed Services University of the Health Congenital Disorders of the Lung
Sciences
Bethesda, Maryland
Primary Amebic Meningoencephalitis
xiv Contributors
Walter S. Gilliam, MSEd, PhD Leslie B. Gordon, MD, PhD Larry A. Greenbaum, MD, PhD
Associate Professor Associate Professor of Pediatrics Research Marcus Professor of Pediatrics
Department of Psychology Warren Alpert Medical School of Brown University Director, Division of Pediatric Nephrology
Child Study Center Providence, Rhode Island; Emory University School of Medicine
Director, The Edward Zigler Center in Child Department of Anesthesia Children’s Healthcare of Atlanta
Development and Social Policy Boston Children’s Hospital Atlanta, Georgia
Yale School of Medicine Harvard Medical School Rickets and Hypervitaminosis D
New Haven, Connecticut Boston, Massachusetts; Vitamin E Deficiency
Childcare: How Pediatricians Can Support Children Medical Director, The Progeria Research Vitamin K Deficiency
and Families Foundation Micronutrient Mineral Deficiencies
Peabody, Massachusetts Electrolyte and Acid-Base Disorders
Salil Ginde, MD, MPH Hutchinson-Gilford Progeria Syndrome Maintenance and Replacement Therapy
Assistant Professor of Pediatrics Deficit Therapy
Division of Pediatric Cardiology Marc H. Gorelick, MD, MSCE Fluid and Electrolyte Treatment of Specific Disorders
Medical College of Wisconsin Professor of Pediatrics
Milwaukee, Wisconsin Medical College of Wisconsin Anne G. Griffiths, MD
Congenital Heart Disease in Adults Children’s Hospital of Wisconsin Instructor in Pediatrics
Milwaukee, Wisconsin Northwestern University Feinberg School of
Charles M. Ginsburg, MD Evaluation of the Sick Child in the Office and Clinic Medicine
Senior Associate Dean for Academic Hospitalist, Neonatal Intensive Care Unit
Administration Jane M. Gould, MD, FAAP Ann & Robert H. Lurie Children’s Hospital of
Professor of Pediatrics Associate Professor of Pediatrics Chicago
Marilyn R. Corrigan Distinguished Chair in Drexel University College of Medicine Chicago, Illinois
Pediatric Research Attending Physician, Infectious Diseases Chronic or Recurrent Respiratory Symptoms
University of Texas Southwestern Medical Center St. Christopher’s Hospital for Children
Houston, Texas Philadelphia, Pennsylvania Allison Grimes, MD
Animal and Human Bites Cryptococcus neoformans Fellow in Pediatric Hematology/Oncology
Histoplasmosis (Histoplasma capsulatum) University of Texas Health Sciences Center
John A. Girotto, MD, MMA, FAAP, Paracoccidioides brasiliensis San Antonio, Texas
FACS Zygomycosis (Mucormycosis) Abnormal Hemoglobins Causing Cyanosis
Associate Professor of Pediatrics, Neurosurgery, Hereditary Methemoglobinemia
and Plastic and Reconstructive Surgery Olivier Goulet, MD Hereditary Methemoglobinemia with Deficiency of
Director, Cleft and Craniofacial Anomalies Center Professor of Pediatrics NADH Cytochrome B5 Reductase
Golisano Children’s Hospital at Strong University of Paris V—René Descartes
University of Rochester Medical Center Head, Division of Pediatric Gastroenterology- Natalia M. Grindler, MD
Rochester, New York Hepatology and Nutrition Resident Physician
Deformational Plagiocephaly Hôpital Necker-Enfants Malades/AP-HP Department of Obstetrics and Gynecology
Paris, France Washington University School of Medicine in St.
Lisa Giulino-Roth, MD Other Malabsorptive Syndromes Louis
Assistant Professor of Pediatrics St. Louis, Missouri
Division of Pediatric Hematology/Oncology Deanna M. Green, MD, MHS Vulvovaginal and Müllerian Anomalies
Weill Cornell Medical College Assistant Professor of Pediatrics
New York, New York Division of Pediatric Pulmonary and Sleep Kenneth L. Grizzle, PhD
Lymphoma Medicine Associate Professor
Duke University School of Medicine Child Development Center—Brookfield
Frances Page Glascoe, PhD Durham, North Carolina Medical College of Wisconsin
Professor Cystic Fibrosis Brookfield, Wisconsin
Department of Pediatrics Childhood-Onset Fluency Disorder: Dysfluency
Vanderbilt University School of Medicine Michael Green, MD, MPH (Stuttering, Stammering)
Nashville, Tennessee Professor of Pediatrics and Surgery
Developmental-Behavioral Screening and University of Pittsburgh School of Medicine Veronique Groleau, MD
Surveillance Division of Pediatric Infectious Diseases Fellow
Children’s Hospital of Pittsburgh of UPMC Division of Gastroenterology, Hepatology, and
Denise M. Goodman, MD, MS Pittsburgh, Pennsylvania Nutrition
Professor Infections in Immunocompromised Persons Children’s Hospital of Philadelphia
Department of Pediatrics Philadelphia, Pennsylvania
Northwestern University Feinberg School of Thomas P. Green, MD Nutritional Requirements
Medicine Founders’ Board Centennial Professor and Chair Feeding Healthy Infants, Children, and Adolescents
Ann & Robert H. Lurie Children’s Hospital of Department of Pediatrics
Chicago Northwestern University Feinberg School of Andrew B. Grossman, MD
Chicago, Illinois Medicine Assistant Professor of Clinical Pediatrics
Wheezing, Bronchiolitis, and Bronchitis Physician-in-Chief University of Pennsylvania Perelman School of
Ann & Robert H. Lurie Children’s Hospital of Medicine
Alison Gopnik, PhD Chicago Co-Director, Center for Pediatric Inflammatory
Professor of Psychology and Affiliate Professor of Chicago, Illinois Bowel Disease
Philosophy Diagnostic Approach to Respiratory Disease The Children’s Hospital of Philadelphia
University of California at Berkeley Chronic or Recurrent Respiratory Symptoms Philadelphia, Pennsylvania
Berkeley, California Pulmonary Edema Inflammatory Bowel Disease
Cognitive Development: Domains and Theories
xvi Contributors
David C. Grossman, MD, MPH Scott B. Halstead, MD Lindsay A. Hatzenbuehler, MD, MPH
Senior Investigator Group Health Research Professor of Pediatrics and Medical Genetics Pediatric Infectious Diseases Fellow
Institute University of British Columbia Faculty of Medicine Baylor College of Medicine
Professor of Health Services British Columbia’s Children’s Hospital Texas Children’s Hospital
University of Washington School of Public Health Vancouver, British Columbia, Canada Houston, Texas
Adjunct Professor of Pediatrics Arboviral Infections in North America Tuberculosis (Mycobacterium tuberculosis)
University of Washington School of Medicine Arboviral Infections Outside North America
Seattle, Washington Dengue Fever and Dengue Hemorrhagic Fever Fern R. Hauck, MD, MS
Injury Control Yellow Fever Spencer P. Bass MD Twenty-First Century
Ebola and Other Viral Hemorrhagic Fevers Professor of Family Medicine
Alfredo Guarino, MD Hantavirus Pulmonary Syndrome Departments of Family Medicine and Public
Professor Health Sciences
Department of Pediatrics Margaret R. Hammerschlag, MD Director, International Family Medicine Clinic
University of Naples Federico II Professor of Pediatrics and Medicine University of Virginia School of Medicine
Napoli, Italy Director, Division of Pediatric Infectious Diseases Charlottesville, Virginia
Chronic Diarrhea SUNY Down State Medical Center Sudden Infant Death Syndrome
Brooklyn, New York
Reut Gurion, DO Chlamydia (Chlamydophila) pneumoniae Fiona P. Havers, MD, MHS
Pediatric Rheumatology Fellow Chlamydia trachomatis Epidemic Intelligence Service Office
Division of Rheumatology Psittacosis (Chlamydia psittaci) Epidemiology and Prevention Branch, Influenza
University Hospitals Case Medical Center Division
Rainbow Babies & Children’s Hospital Aaron Hamvas, MD National Center for Immunization and Respiratory
Cleveland, Ohio Raymond and Hazel Speck Barry Professor of Diseases
Miscellaneous Conditions Associated with Arthritis Neonatology Centers for Disease Control and Prevention
Head, Division of Neonatology Atlanta, Georgia
Lisa R. Hackney, MD Ann & Robert H. Lurie Children’s Hospital of Influenza Viruses
Assistant Professor of Pediatrics Chicago
Division of Pediatric Hematology/Oncology Northwestern University Feinberg School of Jacqueline T. Hecht, PhD
Cleveland Clinic Foundation Medicine Professor and Division Head
Cleveland, Ohio Chicago, Illinois Leah L. Lewis Distinguished Chair
Hereditary Stomatocytosis Diffuse Lung Diseases in Childhood Pediatric Research Center
Glucose-6-Phosphate Dehydrogenase Deficiency and Vice-Chair for Research
Related Deficiencies Abeer J. Hani, MD Department of Pediatrics
Resident Physician UT Health Medical School of Houston
Gabriel G. Haddad, MD Division of Pediatric Neurology Associate Dean for Research
Distinguished Professor and Chair Duke University Medical Center UT Health School of Dentistry
Department of Pediatrics Durham, North Carolina Houston, Texas
University of California, San Diego Seizures in Childhood General Considerations (Bone and Joint Disorders)
Physician-in-Chief Disorders Involving Cartilage Matrix Proteins
Chief Scientific Officer James C. Harris, MD Disorders Involving Transmembrane Receptors
Rady Children’s Hospital Professor of Pediatrics, Psychiatry and Behavioral Disorders Involving Ion Transporters
San Diego, California Sciences, Mental Health, and History of Disorders Involving Transcription Factors
Diagnostic Approach to Respiratory Disease Medicine Disorders Involving Defective Bone Resorption
Division of Child and Adolescent Psychiatry Disorders for Which Defects Are Poorly Understood
Joseph Haddad Jr., MD Director, Developmental Neuropsychiatry or Unknown
Howard W. Smith Professor and Interim Chair Johns Hopkins University School of Medicine
Lawrence Savetsky Professor Baltimore, Maryland Sabrina M. Heidemann, MD
Department of Otolaryngology—Head and Neck Disorders of Purine and Pyrimidine Metabolism Professor
Surgery Department of Pediatrics
Columbia University College of Physicians and Mary E. Hartman, MD, MPH Wayne State University School of Medicine
Surgeons Assistant Professor of Pediatrics Director, Intensive Care Unit
Director, Pediatric Otolaryngology—Head and Washington University in St. Louis Co-Director of Transport
Neck Surgery Pediatric Critical Care Medicine Children’s Hospital of Michigan
New York-Presbyterian Morgan Stanley Children’s St. Louis Children’s Hospital Detroit, Michigan
Hospital St. Louis, Missouri Respiratory Pathophysiology and Regulation
New York, New York Pediatric Emergencies and Resuscitation
Congenital Disorders of the Nose J. Owen Hendley, MD
Acquired Disorders of the Nose David B. Haslam, MD Professor
Nasal Polyps Associate Professor of Pediatrics Department of Pediatrics
General Considerations and Evaluation (Ear) University of Cincinnati College of Medicine University of Virginia School of Medicine
Hearing Loss Director, Antimicrobial Stewardship Program Charlottesville, Virginia
Congenital Malformations Clinical Director, Division of Infectious Diseases Sinusitis
External Otitis (Otitis Externa) Cincinnati Children’s Hospital Medical Center Retropharyngeal Abscess, Lateral Pharyngeal
The Inner Ear and Diseases of the Bony Labyrinth Cincinnati, Ohio (Parapharyngeal) Abscess, and Peritonsillar
Traumatic Injuries of the Ear and Temporal Bone Non–Group A or B Streptococci Cellulitis/Abscess
Tumors of the Ear and Temporal Bone Enterococcus
Frederick M. Henretig, MD
Joseph F. Hagan Jr., MD H. Hesham Abdel-Kader Hassan, MD Division of Emergency Medicine
Clinical Professor Professor of Pediatrics Children’s Hospital of Philadelphia
Department of Pediatrics Chief, Division of Pediatric Gastroenterology and Professor Emeritus of Pediatrics
University of Vermont College of Medicine Nutrition Perelman School of Medicine
Hagan, Rinehart, and Connolly Pediatricians, The University of Arizona College of Medicine University of Pennsylvania
PLLC Tucson, Arizona Philadelphia, Pennsylvania
Burlington, Vermont Cholestasis Biologic and Chemical Terrorism
Maximizing Children’s Health: Screening,
Anticipatory Guidance, and Counseling
Contributors xvii
C. Egla Rabinovich, MD, MPH Harold L. Rekate, MD, FACS, FAAP Elizabeth V. Robilotti, MD, MPH
Associate Professor of Pediatrics Professor of Neurosurgery Associate Director, Infection Control
Duke University School of Medicine Hofstra Northshore School of Medicine Memorial Hospital Division of Infectious Diseases
Co-Chief, Division of Pediatric Rheumatology Director, The Chiari Institute Memorial Sloan Kettering Cancer Center
Duke University Health System Harvey Cushing Neurosciences Institute New York, New York
Durham, North Carolina Great Neck, New York Legionella
Evaluation of Suspected Rheumatic Disease Spinal Cord Disorders
Treatment of Rheumatic Diseases Angela Byun Robinson, MD, MPH
Juvenile Idiopathic Arthritis Megan E. Reller, MD, PhD, MPH Assistant Professor
Scleroderma and Raynaud Phenomenon Assistant Professor of Pathology, Medicine, and Department of Pediatrics
Sjögren Syndrome International Health Case Western Reserve University School of
Miscellaneous Conditions Associated with Arthritis Johns Hopkins University School of Medicine Medicine
Baltimore, Maryland Program Director, Pediatric Rheumatology
Leslie J. Raffini, MD Spotted Fever Group Rickettsioses University Hospitals Case Medical Center
Associate Professor of Pediatrics Scrub Typhus (Orientia tsutsugamushi) Cleveland, Ohio
University of Pennsylvania Perelman School of Typhus Group Rickettsioses Juvenile Dermatomyositis
Medicine Ehrlichioses and Anaplasmosis Miscellaneous Conditions Associated with Arthritis
Division of Hematology Q Fever (Coxiella burnetii)
Children’s Hospital of Philadelphia Genie E. Roosevelt, MD, MPH
Philadelphia, Pennsylvania Jorges D. Reyes, MD Associate Professor of Pediatrics
Hemostasis Assistant Professor of Surgery Department of Emergency Medicine
Hereditary Predisposition to Thrombosis Division of Transplantation University of Colorado School of Medicine
Thrombotic Disorders in Children University of Washington School of Medicine Denver Health Medical Center
Disseminated Intravascular Coagulation Chief, Division of Transplant Surgery Denver, Colorado
Seattle Children’s Hospital Acute Inflammatory Upper Airway Obstruction
Octavio Ramilo, MD Seattle, Washington (Croup, Epiglottitis, Laryngitis, and Bacterial
Henry G. Cramblett Chair in Medicine Intestinal Transplantation in Children with Tracheitis)
Professor of Pediatrics Intestinal Failure
The Ohio State University College of Medicine Liver Transplantation David R. Rosenberg, MD
Chief, Section of Infectious Diseases and Professor and Chair, Department of Psychiatry
Immunology Geoffrey A. Rezvani, MD Miriam L. Hamburger Endowed Chair of Child
Nationwide Children’s Hospital Assistant Professor Psychiatry
Columbus, Ohio Department of Pediatrics Psychiatrist-in-Chief
Mycoplasma pneumoniae Drexel University College of Medicine Wayne State University and the Detroit Medical
Section of Endocrinology, Diabetes, and Center
Denia Ramirez-Montealegre, MD Metabolism Detroit, Michigan
Assistant Professor of Neurology St. Christopher’s Hospital for Children Anxiety Disorders
University of Virginia School of Medicine Philadelphia, Pennsylvania;
Division of Pediatric Neurology Novo Nordisk, Inc. David S. Rosenblatt, MD
UVA Children’s Hospital Princeton, New Jersey Holder, Dodd Q. Chu and Family Chair in Medical
Charlottesville, Virginia An Approach to Inborn Errors of Metabolism Genetics
Ataxias Professor, Departments of Human Genetics,
Iraj Rezvani, MD Medicine, Pediatrics, and Biology
Asma Rashid, MD, MPH Professor of Pediatrics (Emeritus) Faculties of Medicine and Science
Department of Psychiatry Temple University School of Medicine McGill University
Boston Medical Center Adjunct Professor Montreal, Quebec, Canada
Boston, Massachusetts Department of Pediatrics Methionine
Disruptive, Impulse-Control, and Conduct Disorders Drexel University College of Medicine Valine, Leucine, Isoleucine, and Related Organic
Section of Endocrinology, Diabetes, and Acidemias
Giuseppe J. Raviola, MD Metabolism
Assistant Professor of Psychiatry and Global St. Christopher’s Hospital for Children Cindy Ganis Roskind, MD
Health and Social Medicine Philadelphia, Pennsylvania Assistant Clinical Professor
Harvard Medical School An Approach to Inborn Errors of Metabolism Division of Pediatric Emergency Medicine
Director, Psychiatry Quality Program Defects in Metabolism of Amino Acids Columbia University College of Physicians and
Boston Children’s Hospital Surgeons
Boston, Massachusetts A. Kim Ritchey, MD New York, New York
Autism Spectrum Disorder Professor of Pediatrics Acute Care of the Victim of Multiple Trauma
Childhood Psychoses Vice-Chair for Clinical Affairs
Department of Pediatrics A. Catharine Ross, PhD
Gerald V. Raymond, MD University of Pittsburgh School of Medicine Professor and Dorothy Foehr Huck Chair
Professor of Neurology Division of Hematology/Oncology Department of Nutritional Sciences
University of Minnesota School of Medicine Children’s Hospital of Pittsburgh of UPMC The Pennsylvania State University
Chief of Pediatric Neurology Pittsburgh, Pennsylvania University Park, Pennsylvania
University of Minnesota Medical Center, Fairview Principles of Diagnosis (Cancer) Vitamin A Deficiencies and Excess
Minneapolis, Minnesota Principles of Treatment
Disorders of Very Long Chain Fatty Acids The Leukemias Mary M. Rotar, RN, BSN, CIC
Infection Prevention and Control Coordinator
Ann M. Reed, MD Frederick P. Rivara, MD, MPH Children’s Hospital of Wisconsin
Professor of Pediatrics Seattle Children’s Guild Endowed Chair in Milwaukee, Wisconsin
Chair, Department of Pediatrics Pediatrics Infection Prevention and Control
Physician-in-Chief, Duke Children’s Professor and Vice-Chair, Department of Pediatrics
Duke University University of Washington School of Medicine
Durham, North Carolina Seattle, Washington
Juvenile Dermatomyositis Injury Control
xxviii Contributors
xxxvi
lxviii Contents
VIDEOS
Video 304-1 Live Echinococcus granulosus protoscole,
Video 598-1 Severely limited level of consciousness and
movement disorder in a patient with anti-
NMDAR encephalitis after herpes simplex
encephalitis
Video 598-2 Improved level of consciousness in patient
shown in Video 598-1 following
immunotherapy
Video 598-3 Intact cognition in patient shown in Videos
598-1 and 598-2 after immunotherapy and
prolonged follow-up
Nelson
TEXTBOOK of
PEDIATRICS
PART
The Field of Pediatrics I
stage of industrialization and urbanization; (7) the gene frequencies
for some disorders; (8) the health and social welfare infrastructure
Chapter 1 available within these countries; and (9) political focus and stability.
The state of health of any community is defined by the incidence of
Overview of Pediatrics illness and by data from studies that show the changes that occur with
time and in response to programs of prevention, case finding, therapy,
Bonita F. Stanton and Richard E. Behrman and surveillance. To ensure that the needs of children and adults across
the globe were not obscured by local needs, in 2000 the international
community established 8 Millennium Development Goals (MDGs) to
be achieved by 2015 (http://www.countdown2015mnch.org). Although
Pediatrics is the only discipline dedicated to all aspects of the well- all 8 MDGs impact child well-being, MDG 4 (“Reduce by two-thirds,
being of infants, children, and adolescents, including their health; their between 1990 and 2015, the under-five mortality rate”) is exclusively
physical, mental, and psychologic growth and development; and their focused on children.
opportunity to achieve full potential as adults. Pediatricians must be Great strides have been made toward achieving the MDGs. Globally,
concerned not only with particular organ systems and biologic pro- there has been a reduction in under-5 mortality since 1990 from 90 to
cesses, but also with environmental, social, and political influences, 48 deaths per 1,000 live births, with a reduction from 15 to 6 deaths in
which have a major impact on the health and well-being of children developed countries and from 99 to 53 deaths in developing countries.
and their families. With the exception of sub-Saharan Africa and Oceania, all global
Children cannot advocate for themselves. As the professionals whose regions reduced their under-5 mortality rate by more than half from
entire purpose is to advance the well-being of children, pediatricians 1990 to 2012. There were nearly 13 million under-5 deaths in 1990;
must be advocates for the individual child and for all children, irrespec- 2006 marked the first year that there were fewer than 10 million deaths
tive of culture, religion, gender, race, or ethnicity or of local, state, or (9.7 million), which further decreased to 9.0 million in 2007, 8.8
national boundaries. The more politically, economically, or socially million in 2008, 7.6 million in 2010, and 6.6 million in 2012. Despite
disenfranchised a population or a nation is, the greater the need for these substantial successes, the annual rate of reduction in the global
advocacy for children. The young are often among the most vulnerable under-5 mortality rate of 3.9% remains below the MDG targeted rate
or disadvantaged in society and thus their needs require special atten- of 4.4%, necessary to achieve the goal of a 2 3 reduction in the 1990 rate
tion. As divides between nations blur through modern transportation, by 2015 (Fig. 1-1).
communication and economics, through global climate change, The infant mortality rate (deaths of children <1 yr) accounts for 83%
through contemporary means of warfare, and through uneven develop- of the under-5 mortality rate in industrialized countries, but only 64%
ment within and across countries, a global, rather than a national, of the rate in the least-developed nations. Neonatal (<1 mo) death
perspective for the field of pediatrics becomes both a reality and a contributes substantially to the under-5 mortality rate, growing in
necessity. The interrelation of health issues across the globe has achieved proportion as the under-5 death rate decreases. The neonatal mortality
widespread recognition in the wake of the SARS (severe acute respira- rate has been slower to decline. Globally, the neonatal mortality rate of
tory syndrome) and AIDS epidemics, expansions in the pandemics of 23 per 1,000 live births represents 57% of the infant mortality rate of
cholera and West Nile virus, war and bioterrorism, the tsunami of 2004, 40 per 1,000 live births and 40% of the under-5 death rate (up from
the global recession beginning in 2008, the “Arab Spring” beginning in 37% in 1990). The neonatal mortality rate is responsible for 50% of the
2010, and the growing severity of hurricanes and cyclones. under-5 mortality rate in industrialized nations, 40% of the rate in
More than a century ago, pediatrics emerged as a medical specialty developing countries, but only 33% in the least-developed countries.
in response to increasing awareness that the health problems of chil- Most of the decline in infant mortality in the United States and
dren differ from those of adults and that a child’s response to illness other industrialized countries since 1970 is attributable to a decrease
and stress varies with age. In 1959, the United Nations issued the in the birthweight-specific infant mortality rate related to neonatal
Declaration of the Rights of the Child, articulating the universal pre- intensive care, not to the prevention of low-birthweight births (see
sumption that children everywhere have fundamental needs and rights. Chapter 93).
Across the globe, there are significant variations in infant mortality
VITAL STATISTICS ABOUT CHILD HEALTH rates by nation, by region, by economic status, and by level of industrial
(See Also Chapter 1.1) development, the categorizations employed by the World Bank and the
From 1990 to 2010, the world population grew at an annual rate United Nations (Table 1-1; see also Figs. 1-8 and 1-9). As of 2012 three
of 1.3% per yr, down from 1.8% annually during the prior 20 yr. nations in the world still have an under-5 mortality rate of ≥150 per
The annual growth rate from 2010 to 2030 is expected to further 1,000 live births (Sierra Leone, 182; Angola, 164, Chad, 150), with
decline to 0.9%. Worldwide, children younger than age 18 yr account an additional 13 nations having ≥100 deaths per 1,000 live births.
for 2.2 billion (30%) of the world’s 7.02 billion persons. In 2010, there Although these 3 nations are among the poorest in the world, many of
were an estimated 135 million births worldwide, 121 million (90%) of their economic matches have enjoyed greater improvements in child
which were in developing countries. India, with 27.2 million births survival in recent years, demonstrating that economics are important
annually, is home to the largest number, followed by China at 16.5 but that other factors, such as political will, are also important. Simi-
million. larly, in 2012, the United States, with one of the 10 highest gross
Despite global interconnectedness, the health problems of children national incomes in the world, had an under-5 mortality rate of 7 per
and youth vary widely between and within populations in the nations 1,000 live births; 39 nations had lower under-5 mortality rates, with 9
of the world depending on a number of often interrelated factors. These countries having a rate of 3 and 2 countries having a rate of 2 per 1,000
factors include (1) economic considerations (economic disparities); (2) live births.
educational, social, and cultural considerations; (3) the prevalence and Causes of under-5 mortality differ markedly between developed and
ecology of infectious agents and their hosts; (4) climate and geography; developing nations. In developing countries, 66% of all deaths resulted
(5) agricultural resources and practices (nutritional resources); (6) from infectious and parasitic diseases. Among the 42 countries having
1
2 Part I ◆ The Field of Pediatrics
Sub-Saharan Africa 90% of childhood deaths, diarrheal disease accounted for 22% of
177 deaths, pneumonia 21%, malaria 9%, AIDS 3%, and measles 1%. Neo-
98 natal causes contributed to 33%. The contribution for AIDS varies
Southern Asia greatly by country, being responsible for a substantial proportion of
126 deaths in some countries and negligible amounts in others. Likewise,
58
there is substantial co-occurrence of infections; a child may die with
Oceania
HIV, malaria, measles, and pneumonia. Infectious diseases are still
74
55 responsible for much of the mortality in developing countries. In the
Caucasus and Central Asia United States, pneumonia (and influenza) accounted for only 2% of
73 under-5 deaths, with only negligible contributions from diarrhea and
36 malaria. Unintentional injury is the most common cause of death
South-Eastern Asia among U.S. children ages 1-4 yr, accounting for approximately 33% of
71 deaths, followed by congenital anomalies (11%), homicides (9%), and
30 malignant neoplasms (8%). Other causes accounted for <5% of total
Western Asia mortality within this age group (Table 1-2). Although unintentional
65 injuries in developing countries are proportionately less important
25 causes of mortality than in developed countries, their absolute rates
Northern Africa and their contributions to morbidity are substantially greater.
73 Just as economic status of a country as a whole is closely correlated
22
with child survival, so too is relative wealth within a country. Poorer
Latin America and the Caribbean
children in nations worldwide have higher death rates than their
54
19 wealthier national counterparts (Fig. 1-2).
Eastern Asia Causes of death vary by developmental status of the nation. In the
53 United States, the 3 leading causes of death among infants were con-
14 genital anomalies, disorders related to gestation and low birthweight,
Developed regions and sudden infant death (see Table 1-2). By contrast, in developing
15 countries, the majority of infant deaths are caused by infectious dis-
6 eases; even in the neonatal period, 24% of deaths are caused by severe
Developing regions infections and 7% by tetanus. Although immunization rates remain
99 higher in industrialized nations compared to developing nations, this
53 gap is closing. In 2010, immunization percentage rates against diph-
0 50 100 150 200 theria, pertussis, tetanus, measles, and polio were in the mid-90s; com-
1990 2012 2015 target parable levels in developing countries were in the mid-80s, with rates
Figure 1-1 Despite substantial progress, the world is still falling short in the least-developed countries very close. In developing countries,
of the MDG child mortality target. Under-5 mortality rate per 1,000 29% of neonatal deaths are caused by birth asphyxia and 24% are
live births, 1990 and 2012 (deaths per 1,000 live births). (From Millen- caused by complications of prematurity.
nium Development Goals Report, 2014. New York, 2014, United A consistently robust predictor of infant mortality across the globe
Nations, p. 24.) is a poor level of maternal education (consequently, another of the
MDGs addresses the need for universal access to schooling for girls;
Fig. 1-3). Other maternal risk characteristics, such as unmarried status,
adolescence, and high parity, correlate with increased risk of postneo-
natal mortality and morbidity and low birthweight.
Table 1-2 Leading Causes of Death and Numbers of Deaths, According to Age: United States, 2010
AGE AND PERCENT OF
RANK ORDER CAUSE OF DEATH NUMBER TOTAL DEATHS
Under 1 yr All causes 24,586 100%
Congenital malformations, deformations, and chromosomal abnormalities 5,107 21%
Disorders related to short gestation and low birthweight, not elsewhere classified 4,148 17%
Sudden infant death syndrome 2,063 8%
Newborn affected by maternal complications of pregnancy 1,561 6%
Unintentional injuries 1,110 5%
Newborn affected by complications of placenta, cord, and membranes 1,030 4%
Bacterial sepsis of newborn 583 2%
Respiratory distress of newborn 514 2%
Diseases of the circulatory system 507 2%
Newborn affected by maternal complications of pregnancy 472 2%
1-4 yr All causes 4,316 100%
Unintentional injuries 1,394 32%
Congenital malformations, deformations, and chromosomal abnormalities 507 12%
Homicide 385 9%
Malignant neoplasms 346 8%
Diseases of heart 159 4%
Influenza and pneumonia 91 1%
Septicemia 62 1%
In situ neoplasms, benign neoplasms, and neoplasms of uncertain or unknown behavior 59 1%
Certain conditions originating in the perinatal period 52 1%
Chronic lower respiratory diseases 51 1%
5-14 yr All causes 5,279 100%
Unintentional injuries 1,643 31%
Malignant neoplasms 916 17%
Congenital malformations, deformations, and chromosomal abnormalities 298 6%
Suicide 274 5%
Homicide 261 5%
Diseases of heart 185 4%
Chronic lower respiratory diseases 133 3%
Cerebrovascular diseases 90 2%
In situ neoplasms, benign neoplasms, and neoplasms of uncertain or unknown behavior 82 2%
Influenza and pneumonia 71 1%
15-24 yr All causes 29,551 100%
Unintentional injuries 12,341 30%
Homicide 4,678 16%
Suicide 4,600 16%
Malignant neoplasms 1,604 5%
Diseases of heart 1,028 3%
Congenital malformations, deformations, and chromosomal abnormalities 412 1%
Cerebrovascular diseases 190 <1%
Influenza and pneumonia 181 <1%
Diabetes mellitus 165 <1%
Pregnancy, childbirth, and the puerperium 163 <1%
Adapted from National Center for Health Statistics: Health, United States, 2013: with special feature on prescription drugs. Hyattsville, MD, 2014, Department of
Health and Human Services, Table 23, p. 98.
THE CHANGING PEDIATRIC WORLD Bank as among the poorest nations (per capita income <$750/yr) have
A profound improvement in child health within industrialized nations invested heavily in infectious disease control through the development
occurred in the 20th century with the introduction of antibacterial of internal vaccine production capability. From 2000 to 2010, globally
disinfectants, antibiotic agents, and vaccines. Efforts to control infec- there was a 74% decline (with sub-Saharan Africa witnessing an 85%
tious diseases were complemented by better understanding of nutri- decline) in deaths caused by measles as a result of increased vaccina-
tion. In the United States, Canada, and parts of Europe, new and tion. As diarrheal diseases continued through the mid-1970s to account
continuing discoveries in these areas led to establishment of public for ≈25% of infant and childhood deaths in developing countries
well-child clinics for low-income families. Although the timing of (~4 million deaths per year at that time), attention turned to the
control of infectious disease was uneven around the globe, this focus development and utilization of oral resuscitation fluids to sustain chil-
on control was accompanied by significant decreases in morbidity and dren through potentially life-threatening episodes of acute diarrheal
mortality in all countries. The smallpox eradication program of the diseases. Oral rehydration solutions are largely credited with the
1970s resulted in the global eradication of smallpox in 1977. The intro- current reduction of diarrheal deaths annually to 1.5 million. Substan-
duction in the 1970s of the Expanded Program of Immunizations tial improvements have been witnessed in malaria control (global
(universal vaccination against polio, diphtheria, measles, tuberculosis, decrease of incidence by 17% and mortality rate by 25% since 2000).
tetanus, and pertussis) by the World Health Organization (WHO) and There have been substantial increases in the percent of households
United Nations Children’s Fund (UNICEF) has resulted in an esti- having insecticide-treated bed nets and in treatment of children with
mated annual reduction of 1-2 million deaths per year globally. Rec- fever in endemic areas receiving antimalarial drugs.
ognizing the importance of prevention of infectious diseases to the In the later 20th century, with improved control of infectious dis-
health of children, several countries among the 50 ranked by the World eases (including the elimination of polio in the Western hemisphere)
4 Part I ◆ The Field of Pediatrics
Latin America and the Caribbean through both prevention and treatment, pediatric medicine in indus-
2.8 trialized nations increasingly turned its attention to a broad spectrum
Southern Asia of conditions. These included both potentially lethal conditions and
2.7 temporarily or permanently handicapping conditions; among these
disorders were leukemia, cystic fibrosis, diseases of the newborn infant,
Eastern Asia (excluding China) and South-Eastern Asia
congenital heart disease, mental retardation, genetic defects, rheumatic
2.3
diseases, renal diseases, and metabolic and endocrine disorders.
Northern Africa and Western Asia Increasing attention has also been given to behavioral and social
2.1 aspects of child health, ranging from reexamination of child-rearing
Caucasus and Central Asia practices to creation of major programs aimed at prevention and man-
1.7 agement of abuse and neglect of infants and children. Developmental
Sub-Saharan Africa
psychologists, child psychiatrists, neuroscientists, sociologists, anthro-
1.7 pologists, ethnologists, and others have brought us new insights into
human potential, including new views of the importance of the envi-
Developing regions ronmental circumstances during pregnancy, surrounding birth, and in
1.8 the early years of child rearing. The later 20th century witnessed the
0 1 3 beginning of nearly universal acceptance by pediatric professional
societies of attention to normal development, child rearing, and psy-
Higher mortality Equal Higher mortality among chosocial disorders across the continents. In the past decade, irrespec-
among the richest the poorest 20%
20%
tive of level of industrialization, nations have developed programs
addressing not only causes of mortality and physical morbidity (such
Note: Analysis is based on 73 developing countries with data on under- as infectious diseases and protein-calorie malnutrition), but also
five mortality rate by household’s wealth quintile, accounting for 71 factors leading to decreased cognition and thwarted psychosocial
percent of total births in developing countries in 2010.
development, including punitive child-rearing practices (whether at
Figure 1-2 Ratio of under-5 mortality rate for children from the home or in school) and wife abuse, child labor, undernutrition, war,
poorest 20% quintile of households to children from the richest 20% and poor-quality schooling. Obesity is recognized as a major health
of households, 2000/2010. (From Millennium Development Goals risk not only in industrialized nations, but increasingly in transitional
Report, 2012. New York, 2012, United Nations, p. 28.) countries. Progress at the turn of the 21st century in unraveling the
human genome offers for the first time the realization that significant
genetic screening, individualized pharmacotherapy, and genetic
manipulation will be a part of routine pediatric treatment and preven-
Northern Africa and Western Asia tion practices in the future. The prevention implications of the genome
3.3 project give rise to the possibility of reducing costs for the care of illness
1.9 but also increase concerns about privacy issues (see Chapter 3).
Although local famines and disasters, and regional and national
Latin America and the Caribbean
wars have periodically disrupted the general trend for global improve-
3.3
ment in child health indices, it was not until the advent of the AIDS
1.6
epidemic in the later 20th century that the first substantial global
Southern Asia erosion of progress in child health outcomes occurred. This erosion
2.3 resulted in ever-widening gaps between childhood health indices in
1.5 sub-Saharan Africa compared to the rest of the world. From 1990 to
Eastern Asia (excluding China) and South-Eastern Asia 2002, life expectancy in sub-Saharan Africa decreased from 50 yr to
2.1 46 yr. However, as of 2008, it had returned to 52 yr and in 2012 was
1.3 56 yr. Wide distribution of effective antiretroviral therapy (Fig. 1-4),
aggressive HIV prevention education, and increased access to antitu-
Sub-Saharan Africa bercular drugs have been important in these successes, but continued
2.0 successes will require sustained international support. Despite this
1.4 positive news, children with HIV remain the least-likely group to
Developing regions receive antiretroviral treatment. Despite these gains, diseases once con-
2.7 fined to limited geographic niches, including West Nile virus, and
1.5 diseases previously uncommon among humans, such as the avian flu
virus, increased awareness of the interconnectedness of health around
0 1 2 3 4 the world and the impact of global warming. Formerly perceived as a
Higher Equal Higher mortality among children of less problem of industrialized nations, motor vehicle crashes are now rec-
mortality among educated mothers ognized as a major cause of mortality in developing countries.
children of
educated mothers MORBIDITIES AMONG CHILDREN
Adequately addressing special healthcare needs is important in all
Children of mothers with no education compared to children of
countries, both to minimize loss of life and to maximize the potential
mothers with secondary or higher education
of each individual.
Children of mothers with no education compared to children of In the United States, ≈70% of all pediatric hospital bed days are for
mothers with primary education
chronic illnesses; 80% of pediatric health expenditures are for 20% of
Note: Analysis is based on 78 developing countries with data on under- children. Approximately 14% of U.S. children have special healthcare
five mortality rates by mother’s education, accounting for 75 needs, ranging from 10% to 19.8% across the 50 states and the District
percent of total births in developing countries in 2010. of Columbia. One in 5 households with children had ≥1 children with
Figure 1-3 Ratio of under-5 mortality rate of children of mothers with special healthcare needs (see Chapter 42). Significantly, more poor
no education compared to the rate of children of mothers with higher children and minority children have special healthcare needs.
education. (From Millennium Development Goals Report, 2012. New Although there are numerous chronic conditions and the prevalence
York, 2012, United Nations, p. 28.) of these disorders vary by population, 2 of these morbidities—asthma
Chapter 1 ◆ Overview of Pediatrics 5
Table 1-4 Infant, Neonatal, and Postnatal Deaths and Mortality Rates by Specified Race or Origin of Mother: United
States, 2009 and 2010
Mortality Rate Per 1,000 Live Births
RACE OF MOTHER YEAR(S) INFANT NEONATAL POSTNATAL
All races 2007 6.4 4.2 2.2
2008 6.1 4.0 2.1
White 2007 5.3 3.5 1.9
2008 5.2 3.5 1.8
Black or African American 2007 12.1 8.0 4.2
2008 11.2 7.3 3.9
American Indian or Alaska Native 2007 8.3 4.3 4.0
Asian or Pacific Islander 2007 4.3 3.0 1.3
Hispanic or Latino 2007 5.3 3.6 1.7
Mexican 2007 5.1 3.5 1.6
Puerto Rican 2007 7.1 4.8 2.3
Cuban 2007 3.8 2.9 2.1*
Central and South American 2007 4.4 3.0 1.4
Other and unknown 2007 6.1 4.0 2.1
Hispanic or Latino
Not Hispanic or Latino
White 2007 5.2 3.4 1.8
Black or African American 2007 11.5 7.5 4.0
Adapted from National Center for Health Statistics: Health, United States, 2013: with special feature on prescription drugs, Hyattsville, MD, 2014, Department of
Health and Human Services, Tables 11, p. 98, and 17, p. 71.
*2009.
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