Nelson Textbook of Pediatrics

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Author(s): Robert M. Kliegman, Joseph W. St Geme III, Nathan J. Blum, Samir S. Shah, Robert C. Tasker, Karen M. Wilson
Edition: 21
Publisher: Elsevier
Year: 2019

Language: English
Pages: 3827

Cover
Front Matter
Copyright Page
Dedication
Contributors
Preface
Videos
Part I: The Field of Pediatrics
Chapter 1: Overview of Pediatrics
Keywords
Vital Statistics About Children’s Health Globally
The Changing Pediatric World
The New Morbidities
The “New” New Morbidities
Adverse Childhood Experiences
Toxic Stress and Allostatic Load
Ecobiodevelopmental Framework
Chronic Illness and Children With Special Health Care Needs
Systems of Care
Population Health Approach
Medical Home
Medical and Health Neighborhood
Bibliography
Chapter 2: Child Health Disparities
Keywords
Determinants of Health and Health Disparities
Psychosocial Stress and Allostatic Load
The Hispanic Paradox
Disparities in Child Health and Healthcare:
Child Health Disparities
Asthma
Obesity
Infant Mortality
Prematurity and Low Birthweight
Oral Health
Hearing Care
Vision Problems
Immunization
Adolescent Suicide
Child Maltreatment
Behavioral Health Disparities
Attention-Deficit/Hyperactivity Disorder (ADHD)
Depression and Anxiety Disorders
Autism Spectrum Disorder (ASD)
Behavioral or Conduct Problems
Developmental Delay
Disparities in Healthcare
Approaches to Eradicating Disparities: Interventions
2.1 Racism and Child Health
Keywords
Racism as Social Determinant
Explaining Racial Disparities: A Taxonomy of Racism
Defining Racism
Internalized Racism
Interpersonal Racism
Institutional Racism
Structural Racism
Opportunities to Address Racism
Clinical Settings
Institutional Settings
Pediatricians as Advocates for Antiracist Practices and Systems
Bibliography
Bibliography
Chapter 3: Global Child Health
Keywords
Global Burden and Trends in Child Health
Social Determinants of Child Health
Socioeconomic and Political Roots of Disparities in Global Health
Sustainable Development Goals
Evidence-Based Interventions and Innovations to Address Child Health Inequities
Vaccine-Preventable Diseases
Reaching Every Child, Everywhere
Effective Delivery Strategies: Integrated Management of Childhood Illness
Social Protection Programs: Conditional and Unconditional Cash Transfers
Challenges in Global Health
Adolescent Health
Global Climate Change
Conflict, Emergency Situations, and Migration
Information and Communications Technology
Bibliography
Chapter 4: Quality and Value in Healthcare for Children
Keywords
The Need for Improvement in Quality and Value
What Is Quality?
Framework for Quality
Developing Guidelines to Establish the Standard for Quality
Improving Quality
Model for Improvement
Six Sigma
Lean Methodology
Management Sciences
Tools for Organizing Quality Improvement Theory and Execution
Measuring Quality
Analyzing Quality Data
Comparing and Reporting Quality
Implications of the U.S. Healthcare Reform for Quality
Information Technology and Quality Improvement
Expanding Individual Quality Improvement Initiatives to Scale
Bibliography
Chapter 5: Safety in Healthcare for Children
Keywords
Error vs Harm
Safety Frameworks
Identifying and Analyzing Harm, Errors, and Latent Threats
Incident Reporting Systems
Simulation
Event Analysis
Safety Culture
Reliability Science and High-Reliability Organizations
Serious Harm Events and Healthcare-Associated Conditions
Safety Opportunities and Gaps
Clinical Deterioration
Handoffs/I-PASS
Surgical Safety
Ambulatory Safety
Occupational Safety
Emerging Areas of Safety Research and Improvement
Bibliography
Chapter 6: Ethics in Pediatric Care
Keywords
Assent and Parental Permission
Treatment of Critically Ill Children
Transitioning the Goals of Care
Withholding and Withdrawing Life-Sustaining Treatment
Advance Directives
Artificial Hydration and Nutrition
The Doctrine of Double Effect
Neonatal Ethics
Declaring Death and Organ Donation
Death by Neurologic Criteria
Circulatory Death
Religious or Cultural Objections to Treatment
Pediatric Ethics Committees and Ethics Consultation
Newborn Screening
Genetics, Genomics, and Precision Medicine
Adolescent Healthcare
Adolescent Assent and Consent
Chronic Illness
Decisions in Terminally Ill Adolescents
Research
Balancing Maternal and Fetal Interests
Justice and Pediatric Ethics
Emerging Issues
Bibliography
Chapter 7: Pediatric Palliative Care
Keywords
Care Settings
Communication, Advance Care Planning, and Anticipatory Guidance
The Parents
The Child
The Siblings
The Staff
Goals of Care and Decision-Making
Resuscitation Status
Symptom Management
Intensive Symptom Management
Approaching End of Life
The Pediatrician
Bibliography
Chapter 8: Domestic and International Adoption
Keywords
Domestic Adoption
Intercountry Adoption
Role of Pediatricians
Preadoption Medical Record Reviews
Postadoption Medical Care
Arrival Visit–International Adoption
Growth Delays
Developmental Delays
Language Development
Eating Concerns
Sleep Concerns
Social and Emotional Development
Racial Identity Development
Toxic Stress
Family Support
Adoption Narrative
Bibliography
Chapter 9: Foster and Kinship Care
Keywords
Epidemiology
Legislation in the United States
Early Childhood Trauma Leads to Poor Health Outcomes
Health Issues
Healthcare for Children and Adolescents in Foster Care
Bibliography
Chapter 10: Medical Evaluation of the Foreign-Born Child
Keywords
Commonly Encountered Infections
Hepatitis B
Hepatitis A
Hepatitis C
Intestinal Pathogens
Tuberculosis
Congenital Syphilis
HIV Infection
Immunizations
Bibliography
Chapter 11: Cultural Issues in Pediatric Care
Keywords
What Is Culture?
Culture and Identity
Intracultural Variability
Culturally Informed Care
Understanding Culture in the Context of Healthcare
Awareness-Assessment-Negotiation Model
11.1 Culture-Specific Beliefs
Bibliography
Chapter 12: Maximizing Children’s Health
Keywords
Periodicity Schedule and Guidelines
Tasks of Well-Child Care
Infancy and Early Childhood
Middle Childhood and Adolescence
Office Intervention for Behavioral and Mental Health Issues
Strength-Based Approaches and Framework
Office System Change for Quality Improvement
Evidence
Caring for the Child and Youth in the Context of the Family and Community
Bibliography
Chapter 13: Injury Control
Keywords
Scope of the Problem
Mortality
Nonfatal Injuries
Global Child Injuries
Principles of Injury Control
Risk Factors for Childhood Injuries
Age
Gender
Race and Ethnicity
Socioeconomic Status
Rural-Urban Location
Environment
Mechanisms of Injury
Motor Vehicle Injuries
Occupants
Teenage Drivers
All-Terrain Vehicles
Bicycle Injuries
Pedestrian Injuries
Ski- and Snowboard-Related Head Injuries
Fire- and Burn-Related Injuries
Poisoning
Drowning
Traumatic Brain Injury
Firearm Injuries
Falls
Violent Behavior and Aggression
Psychosocial Consequences of Injuries
Bibliography
Chapter 14: Impact of Violence on Children
Keywords
Impacts of Violence
Diagnosis and Follow-Up
14.1 Bullying, Cyberbullying, and School Violence
Keywords
Bullying and Cyberbullying
Bullying Roles and Nomenclature
Epidemiology
Risk Factors
Consequences of Bullying
School Violence
Epidemiology
Risk Factors
Treatment and Prevention of Bullying and School Violence
Signs and Symptoms
Screening for Bullying
Prevention
Bibliography
Resources
14.2 Media Violence
Keywords
Screening
Recommendations (see Table 14.1)
Bibliography
Resources
14.3 Effects of War on Children
Keywords
Susceptibility of Children in Times of War
Psychological Impact of War
Efforts to Protect Children From the Effects of War
International Conventions
Humanitarian Efforts
Role of Pediatricians and Allied Health Professionals
Bibliography
Bibliography
Chapter 15: Child Trafficking for Sex and Labor
Keywords
Clinical Presentation
Approach to the Potentially Trafficked Child
Examination and Diagnostic Testing
Referrals and Resources
Bibliography
Chapter 16: Abused and Neglected Children
Keywords
Definitions
Incidence and Prevalence
Global
United States
Etiology
Clinical Manifestations
Neglect
General Principles for Assessing Possible Abuse and Neglect
General Principles for Addressing Child Maltreatment
Outcomes of Child Maltreatment
Prevention of Child Abuse and Neglect
Advocacy
16.1 Sexual Abuse
Keywords
Definition
Presentation of Sexual Abuse
Role of General Pediatrician in Assessment and Management of Possible Sexual Abuse
Physical Examination of the Child With Suspected Sexual Abuse
Additional Management
Sexual Abuse Prevention
Bibliography
16.2 Medical Child Abuse (Factitious Disorder by Proxy, Munchausen Syndrome by Proxy)
Keywords
Clinical Manifestations
Diagnosis
Treatment
Bibliography
Bibliography
General References
Physical Abuse
Neglect
Prevention
Professional Issues
Outcomes
American Academy of Pediatrics Policy Statements
Chapter 17: Strategies for Health Behavior Change
Keywords
Unified Theory of Behavior Change
Transtheoretical Model of Health Behavior Change
Common Factors Approach
Interpersonal Skills: HEL2P3
Elicit Concerns: Set the Agenda and Agree on the Problem
Establish a Plan
Respond to Anger and Demoralization and Emphasize Hope
Motivational Interviewing
Shared Decision-Making
Bibliography
Part II: Growth, Development, and Behavior
Chapter 18: Developmental and Behavioral Theories
Keywords
Biopsychosocial Model and Ecobiodevelopmental Framework: Models of Development
Biologic Influences
Psychologic Influences: Attachment and Contingency
Social Factors: Family Systems and the Ecologic Model
Unifying Concepts: The Transactional Model, Risk, and Resilience
Developmental Domains and Theories of Emotion and Cognition
Psychoanalytic Theories
Cognitive Theories
Behavioral Theory
Theories Used in Behavioral Interventions
Statistics Used in Describing Growth and Development
Bibliography
Chapter 19: Positive Parenting and Support
Keywords
The Importance of Parenting
The Role of the Family
Parenting Styles
Child Temperament
Child Behavioral Problems
Defining Positive Parenting
Parenting as an Intervention
The Role of the Pediatrician
Bibliography
Chapter 20: Assessment of Fetal Growth and Development
Keywords
Somatic Development
Embryonic Period
Fetal Period
Neurologic Development
Behavioral Development
Psychologic Changes in Parents
Threats to Fetal Development
Bibliography
Chapter 21: The Newborn
Keywords
Parental Role in Mother–Infant Attachment
Prenatal Factors
Peripartum and Postpartum Influences
The Infant’s Role in Mother–Infant Attachment
Physical Examination
Interactional Abilities
Modulation of Arousal
Behavioral States
Mutual Regulation
Implications for the Pediatrician
Optimal Practices
Assessing Parent–Infant Interactions
Teaching About Individual Competencies
Bibliography
Chapter 22: The First Year
Keywords
Keywords
Age 0-2 Months
Physical Development
Cognitive Development
Emotional Development
Implications for Parents and Pediatricians
Age 2-6 Months
Physical Development
Cognitive Development
Emotional Development and Communication
Implications for Parents and Pediatricians
Age 6-12 Months
Physical Development
Cognitive Development
Emotional Development
Communication
Implications for Parents and Pediatricians
22.1 Infant Crying and Colic
Bibliography
Infant Crying and Colic
Chapter 23: The Second Year
Keywords
Age 12-18 Months
Physical Development
Cognitive Development
Emotional Development
Linguistic Development
Implications for Parents and Pediatricians
Age 18-24 Months
Physical Development
Cognitive Development
Emotional Development
Linguistic Development
Implications for Parents and Pediatricians
Bibliography
Chapter 24: The Preschool Years
Keywords
Structural Development of the Brain
Physical Development
Implications for Parents and Pediatricians
Language, Cognition, and Play
Language
Cognition
Play
Implications for Parents and Pediatricians
Emotional and Moral Development
Implications for Parents and Pediatricians
Bibliography
Chapter 25: Middle Childhood
Keywords
Physical Development
Implications for Parents and Pediatricians
Cognitive Development
Implications for Parents and Pediatricians
Social, Emotional, and Moral Development
Social and Emotional Development
Moral Development
Implications for Parents and Pediatricians
Bibliography
Chapter 26: Adolescence
Chapter 27: Assessment of Growth
Keywords
Techniques to Measure Growth
Growth Curves
Normal Growth
Abnormal Growth
Evaluation of Abnormal Growth
Other Growth Considerations
Obesity
Dental Development
Bibliography
Chapter 28: Developmental and Behavioral Surveillance and Screening
Keywords
Developmental and Behavioral Surveillance
The Developmental and Behavioral Histories
Observation
Developmental and Behavioral Screening
Evidence-Based Tools
Screening Test Properties
Beyond Surveillance and Screening
Comprehensive Evaluation
Referral and Intervention
Ongoing Management
Implementation
Bibliography
Resources
Chapter 29: Childcare
Keywords
Provision, Regulation, and Use of Childcare in America
Childcare Settings
Childcare Licensing, Regulation, and Accreditation
Childcare’s Role in Child Health and Development
Characteristics of Childcare and Associations With Child Developmental Outcomes
Sick Children
Childcare and Child Health
Childcare and Children With Special Needs
Role of Pediatric Providers in Childcare
Advising Parents on Childcare Selection
Advising Parents on Childcare Health Issues
Helping Children With Special Needs
Consulting and Partnering With Childcare Providers
Bibliography
Chapter 30: Loss, Separation, and Bereavement
Keywords
Separation and Loss
Divorce
Move/Family Relocation
Separation Because of Hospitalization
Military Families
Parental/Sibling Death
Grief and Bereavement
Developmental Perspective
Role of the Pediatrician in Grief
Treatment
Spiritual Issues
Bibliography
Chapter 31: Sleep Medicine
Keywords
Basics of Sleep and Chronobiology
Developmental Changes in Sleep
Common Sleep Disorders
Insomnia of Childhood
Obstructive Sleep Apnea Syndrome
Etiology
Epidemiology
Pathogenesis
Clinical Manifestations
Diagnosis
Treatment
Parasomnias
Etiology
Epidemiology
Clinical Manifestations
Treatment
Sleep-Related Movement Disorders: Restless Legs Syndrome/Periodic Limb Movement Disorder and Rhythmic Movements
Etiology
Epidemiology
Clinical Manifestations
Treatment
Narcolepsy
Etiology
Epidemiology
Clinical Manifestations and Diagnosis
Treatment
Delayed Sleep–Wake Phase Disorder
Etiology
Epidemiology
Clinical Manifestations
Treatment
Health Supervision
Evaluation of Pediatric Sleep Problems
Bibliography
Part III: Behavioral and Psychiatric Disorders
Chapter 32: Psychosocial Assessment and Interviewing
Keywords
Aims of Assessment
Presenting Problems
General Principles of the Psychosocial Interview
Indications for Referral
Psychiatric Diagnostic Evaluation
Special Considerations in the Diagnostic Evaluation of Infants and Young Children
Bibliography
Chapter 33: Psychopharmacology
Keywords
Stimulants and Other ADHD Medications
Antidepressants
Antipsychotics
Mood Stabilizers
Medication Use in Physical Illness
Hepatic Disease
Gastrointestinal Disease
Renal Disease
Cardiac Disease
Respiratory Disease
Neurologic Disease
Principles for Psychotropic Prescribing in Primary Care
Bibliography
Chapter 34: Psychotherapy and Psychiatric Hospitalization
Keywords
Psychotherapy
Behavior Therapy
Cognitive-Behavioral Therapy
Interpersonal Psychotherapy
Psychodynamic Psychotherapy
Supportive Psychotherapy
Family Therapy
Parenting Interventions
Common Elements of Evidence-Based Psychotherapies
Modular Therapy Packages
Common Elements of Treatment Engagement Interventions
Psychotherapy in the Medical Home
Psychiatric Hospitalization
Bibliography
Chapter 35: Somatic Symptom and Related Disorders
Keywords
Epidemiology
Risk Factors
Individual
Temperament/Coping Styles
Learned Behavior
Psychiatric Comorbidity
Childhood Physical Illness
Family and Environmental
Symptom Modeling
Parental Responses
School and Family Stressors
Trauma
Genetic and Biologic Vulnerabilities
Assessment
Medical
Psychosocial
Differential Diagnoses
Management
Treatment
Treatment Setting
Bibliography
Chapter 36: Rumination and Pica
Keywords
36.1 Rumination Disorder
Epidemiology
Etiology and Differential Diagnosis
Treatment
Bibliography
36.2 Pica
Epidemiology
Etiology and Differential Diagnosis
Treatment
Bibliography
Chapter 37: Motor Disorders and Habits
Keywords
37.1 Tic Disorders
Description
Clinical Course
Epidemiology
Differential Diagnosis
Comorbidities
Etiology
Sequelae
Screening
Assessment
Treatment
37.2 Stereotypic Movement Disorder
Description
Clinical Course
Epidemiology
Comorbidity
Differential Diagnosis
Etiology
Treatment
Habits
Thumb Sucking
Bruxism
Bibliography
Chapter 38: Anxiety Disorders
Keywords
Anxiety Associated With Medical Conditions
Safety and Efficacy Concerns About SSRIs
Bibliography
Chapter 39: Mood Disorders
Keywords
39.1 Major and Other Depressive Disorders
Description
Epidemiology
Clinical Course
Differential Diagnosis
Comorbidity
Sequelae
Etiology and Risk Factors
Prevention
Screening/Case Finding
Early Intervention
Treatment
Level of Care
Bibliography
39.2 Bipolar and Related Disorders
Description
Epidemiology
Clinical Course
Differential Diagnosis
Comorbidity
Sequelae
Etiology and Risk Factors
Prevention
Case Finding
Treatment
Level of Care
Bibliography
Chapter 40: Suicide and Attempted Suicide
Keywords
Epidemiology
Suicidal Ideation and Attempts
Suicide Completions
Risk Factors
Preexisting Mental Disorder
Cognitive Distortions
Biologic Factors
Social, Environmental, and Cultural Factors
Protective Factors
Assessment and Intervention
Prevention
Bibliography
Chapter 41: Eating Disorders
Keywords
Definitions
Epidemiology
Pathology and Pathogenesis
Clinical Manifestations
Differential Diagnosis
Laboratory Findings
Complications
Treatment
Principles Guiding Primary Care Treatment
Nutrition and Physical Activity
Primary Care Treatment
Referral to Mental Health Services
Referral to an Interdisciplinary Eating Disorder Team
Supportive Care
Prognosis
Prevention
Bibliography
Chapter 42: Disruptive, Impulse-Control, and Conduct Disorders
Keywords
Description
Epidemiology
Clinical Course
Differential Diagnosis
Comorbidity
Sequelae
Etiology and Risk Factors
Prevention
Screening/Case Finding
Early Intervention
Treatment
Level of Care
Bibliography
Chapter 43: Tantrums and Breath-Holding Spells
Keywords
Bibliography
Chapter 44: Lying, Stealing, and Truancy
Keywords
Lying
Stealing
Truancy
Bibliography
Chapter 45: Aggression
Keywords
Bibliography
Chapter 46: Self-Injurious Behavior
Keywords
Bibliography
Chapter 47: Childhood Psychoses
Keywords
47.1 Schizophrenia Spectrum and Other Psychotic Disorders
Description
Epidemiology
Clinical Course
Differential Diagnosis
Comorbidity
Sequelae
Etiology and Risk Factors
Genetic Factors
Environmental Factors
Neuroanatomic Abnormalities
Prevention
Screening/Case Finding
Assessment
Treatment
Pharmacotherapy
Bibliography
47.2 Psychosis Associated With Epilepsy
Bibliography
47.3 Catatonia in Children and Adolescents
Diagnosis and Treatment
Bibliography
47.4 Acute Phobic Hallucinations of Childhood
Clinical Manifestations
Diagnosis and Differential Diagnosis
Treatment
Bibliography
Part IV: Learning and Developmental Disorders
Chapter 48: Neurodevelopmental and Executive Function and Dysfunction
Keywords
Terminology and Epidemiology
Etiology and Pathogenesis
Core Neurodevelopmental Functions
Sensory and Motor Function
Language
Visual-Spatial/Visual-Perceptual Function
Intellectual Function
Memory
Social Cognition
Executive Function
Clinical Manifestations
Academic Problems
Nonacademic Problems
Assessment and Diagnosis
Treatment
Demystification
Bypass Strategies (Accommodations)
Treatment of Neurodevelopmental Dysfunctions
Treatment of Executive Dysfunction
Developmental Therapy
Curriculum Modifications
Strengthening of Strengths
Individual and Family Counseling
Nonstandard Therapies
Medication
Bibliography
Chapter 49: Attention-Deficit/Hyperactivity Disorder
Keywords
Etiology
Epidemiology
Pathogenesis
Clinical Manifestations
Diagnosis and Differential Diagnosis
Clinical Interview and History
Behavior Rating Scales
Physical Examination and Laboratory Findings
Differential Diagnosis
Treatment
Psychosocial Treatments
Behaviorally Oriented Treatments
Medications
Prognosis
Prevention
Bibliography
Chapter 50: Dyslexia
Keywords
Etiology
Epidemiology
Pathogenesis
Clinical Manifestations
Diagnosis
Management
Prognosis
Bibliography
Chapter 51: Math and Writing Disabilities
51.1 Math Disabilities
Keywords
Math Learning Disability Defined
Terminology
Epidemiology
Prevalence
Risk Factors
Genetics
Medical/Genetic Conditions
Comorbidities
Causes of Math Learning Disability
Broad Cognitive Processes
Intelligence
Working Memory
Processing Speed
Math-Specific Processes
Procedural Errors
Memory for Math Facts
Treatment and Interventions
Bibliography
51.2 Writing Disabilities
Keywords
Epidemiology
Skill Deficits Associated With Impaired Writing
Transcription
Oral Language
Executive Functions
Working Memory
Treatment
Educational Resources
Bibliography
Chapter 52: Language Development and Communication Disorders
Keywords
Normal Language Development
Receptive Language Development
Expressive Language Development
Variations of Normal
Language and Communication Disorders
Epidemiology
Etiology
Pathogenesis
Clinical Manifestations
Classification
Language Disorder or Specific Language Impairment
Higher-Level Language Disorder
Intellectual Disability
Autism Spectrum Disorder
Asperger Syndrome
Selective Mutism
Isolated Expressive Language Disorder
Motor Speech Disorders
Dysarthria
Childhood Apraxia of Speech
Speech Sound Disorder
Hearing Impairment
Hydrocephalus
Rare Causes of Language Impairment
Hyperlexia
Landau-Kleffner Syndrome (Verbal Auditory Agnosia)
Metabolic and Neurodegenerative Disorders
Screening
Noncauses of Language Delay
Diagnostic Evaluation
Psychologic Evaluation
Cognitive Assessment
Evaluation of Social Behaviors
Relationship of Language and Social Behaviors to Mental Age
Speech and Language Evaluation
Medical Evaluation
Treatment
Prognosis
Academic Disorders
Comorbid Disorders
Emotional and Behavioral Difficulty
Motor and Coordination Delays
52.1 Childhood-Onset Fluency Disorder
Keywords
Epidemiology
Genetics
Etiology
Comorbidities
Developmental Progression
Treatment
Bibliography
Bibliography
Chapter 53: Developmental Delay and Intellectual Disability
Keywords
Definition
Etiology
Epidemiology
Pathology and Pathogenesis
Clinical Manifestations
Diagnostic Evaluation
Differential Diagnosis
Diagnostic Psychologic Testing
Complications
Prevention
Treatment
Supportive Care and Management
Primary Care
Interdisciplinary Management
Periodic Reevaluation
Federal and Education Services
Leisure and Recreational Activities
Family Counseling
Transition to Adulthood
Prognosis
53.1 Intellectual Disability With Regression
Bibliography
Chapter 54: Autism Spectrum Disorder
Keywords
Definition
Diagnostic Criteria and Symptoms
Social Communication and Social Interaction
Social-Emotional Reciprocity
Nonverbal Communicative Behavior
Developing, Maintaining, and Understanding Relationships
Restrictive and Repetitive Behavior
Stereotyped Motor Movements or Speech
Insistence on Sameness
Restricted Interests
Hypo- or Hyperreactivity to Sensory Input
Severity Levels Defined in DSM-5
Specifiers Defined in DSM-5
Epidemiology
Etiology
Differential Diagnosis
Comorbid Conditions
Screening
Assessment
First Tier
Second Tier
Treatment and Management
Educational
Co-occurring Conditions
Pharmacology
Complementary and Alternative Medicine
Transition
Outcome
Bibliography
Part V: Nutrition
Chapter 55: Nutritional Requirements
Keywords
Dietary Reference Intakes
Energy
Fat
Protein
Carbohydrates
Fiber
Micronutrients
Iron
Vitamin D
Calcium
Vitamin K
Electrolytes
Water
Measuring Nutritional Adequacy
Bibliography
Chapter 56: Feeding Healthy Infants, Children, and Adolescents
Keywords
Feeding During the First Year of Life
Breastfeeding
Nipple Pain
Engorgement
Mastitis
Inadequate Milk Intake
Jaundice
Breast Milk Collection
Growth of the Breastfed Infant
Formula Feeding (Fig. 56.1)
Cow’s Milk Protein–Based Formulas
Soy Formulas
Protein Hydrolysate Formulas
Amino Acid Formulas
Milk and Other Fluids in Infants and Toddlers
Complementary Feeding
Feeding Toddlers and Preschool-Age Children
Feeding Practices
Eating in the Daycare Setting
Feeding School-Age Children and Adolescents
MyPlate
Eating at Home
Eating at School
Eating Out
Nutrition Issues of Importance Across Pediatric Ages
Food Environment
Using Food as Reward
Cultural Considerations in Nutrition and Feeding
Vegetarianism
Organic Foods
Nutrition as Part of Complementary and Alternative Medicine, Functional Foods, Dietary Supplements, Vitamin Supplements, and Botanical and Herbal Products
Food Safety
Preventive Nutrition Counseling in Pediatric Primary Care
U.S. Food Assistance Programs
Bibliography
Chapter 57: Nutrition, Food Security, and Health
Keywords
Malnutrition as the Intersection of Food Insecurity and Health Insecurity
Food Security
Measuring Food Insecurity
Nutrition, Food Security, and Poverty
Food Security and Nutrition Targets
Future Food Security
Undernutrition
Measurement of Undernutrition
Prevalence of Undernutrition
Consequences of Undernutrition
Key Interventions
Clinical Manifestations and Treatment of Undernutrition
Severe Acute Malnutrition
Clinical Manifestations of Severe Acute Malnutrition (Table 57.6)
Pathophysiology
Principles of Treatment
Emergency Treatment
Stabilization
Rehabilitation
Community-Based Treatment
Bibliography
Chapter 58: Refeeding Syndrome
Keywords
Bibliography
Chapter 59: Malnutrition
Keywords
Clinical Manifestations
Etiology and Diagnosis
Treatment
Prognosis
Bibliography
Chapter 60: Overweight and Obesity
Keywords
Epidemiology
Body Mass Index
Etiology
Environmental Changes
Genetics
Microbiome
Endocrine and Neural Physiology
Comorbidities
Identification
Evaluation
Intervention
Prevention
60.1 Rapid-Onset Obesity With Hypothalamic Dysfunction, Hypoventilation, and Autonomic Dysregulation (ROHHAD)
Keywords
Clinical Manifestations
Diagnosis
Management
Etiology: Studies and Hypotheses
Genetic Studies
Paraneoplastic/Autoimmune Hypothesis
Neurocristopathy
Differential Diagnosis
Bibliography
Bibliography
Chapter 61: Vitamin A Deficiencies and Excess
Keywords
Overview of Vitamin A
Metabolism of Vitamin A
Vitamin A Status in Neonates
Inflammation Causing Low Plasma Retinol
Functions of Vitamin A and Mechanisms of Action
Vitamin A Deficiency
Clinical Manifestations of Vitamin A Deficiency
Diagnosis
Epidemiology and Public Health Issues
Dietary Reference Intakes for the Healthy Population
Vitamin A for Treatment of Deficiency
Hypervitaminosis A
Bibliography
Chapter 62: Vitamin B Complex Deficiencies and Excess
62.1 Thiamine (Vitamin B1)
Keywords
Thiamine Deficiency
Clinical Manifestations
Diagnosis
Prevention
Treatment
Thiamine Toxicity
Bibliography
62.2 Riboflavin (Vitamin B2)
Keywords
Riboflavin Deficiency
Clinical Manifestations
Diagnosis
Prevention
Treatment
Riboflavin Toxicity
Bibliography
62.3 Niacin (Vitamin B3)
Keywords
Niacin Deficiency
Clinical Manifestations
Diagnosis
Prevention
Treatment
Niacin Toxicity
Bibliography
62.4 Vitamin B6 (Pyridoxine)
Keywords
Vitamin B6 Deficiency
Clinical Manifestations
Diagnosis
Prevention
Treatment
Vitamin B6 Toxicity
Bibliography
62.5 Biotin
Keywords
Bibliography
62.6 Folate
Keywords
Folate Deficiency
Clinical Manifestations
Diagnosis
Prevention
Treatment
Folate Toxicity
Bibliography
62.7 Vitamin B12 (Cobalamin)
Keywords
Vitamin B12 Deficiency
Clinical Manifestations
Diagnosis
Treatment
Prevention
Bibliography
Chapter 63: Vitamin C (Ascorbic Acid) Deficiency and Excess
Keywords
Dietary Needs and Sources of Vitamin C
Vitamin C Deficiency
Clinical Features
Laboratory Findings and Diagnosis
Differential Diagnosis
Treatment
Prevention
Vitamin C Toxicity
Bibliography
Chapter 64: Vitamin D Deficiency (Rickets) and Excess
Keywords
Rickets
Etiology
Clinical Manifestations
Radiology
Diagnosis
Clinical Evaluation
Vitamin D Disorders
Vitamin D Physiology
Nutritional Vitamin D Deficiency
Etiology
Clinical Manifestations
Laboratory Findings
Diagnosis and Differential Diagnosis
Treatment
Prognosis
Prevention
Congenital Vitamin D Deficiency
Secondary Vitamin D Deficiency
Etiology
Treatment
Vitamin D–Dependent Rickets, Type 1
Treatment
Vitamin D–Dependent Rickets, Type 2
Treatment
Chronic Kidney Disease
Treatment
Calcium Deficiency
Pathophysiology
Clinical Manifestations
Diagnosis
Treatment
Phosphorus Deficiency
Inadequate Intake
Fibroblast Growth Factor-23
X-Linked Hypophosphatemic Rickets
Pathophysiology
Clinical Manifestations
Laboratory Findings
Treatment
Prognosis
Autosomal Dominant Hypophosphatemic Rickets
Autosomal Recessive Hypophosphatemic Rickets
Hereditary Hypophosphatemic Rickets With Hypercalciuria
Pathophysiology
Clinical Manifestations
Laboratory Findings
Treatment
Overproduction of FGF-23
Fanconi Syndrome
Dent Disease
Rickets of Prematurity
Pathogenesis
Clinical Manifestations
Laboratory Findings
Diagnosis
Prevention
Treatment
Distal Renal Tubular Acidosis
Hypervitaminosis D
Etiology
Pathogenesis
Clinical Manifestations
Laboratory Findings
Diagnosis and Differential Diagnosis
Treatment
Prognosis
Bibliography
Chapter 65: Vitamin E Deficiency
Keywords
Pathogenesis
Clinical Manifestations
Laboratory Findings
Diagnosis and Differential Diagnosis
Treatment
Prognosis
Prevention
Bibliography
Chapter 66: Vitamin K Deficiency
Keywords
Pathogenesis
Clinical Manifestations
Laboratory Findings
Diagnosis and Differential Diagnosis
Treatment
Prevention
Bibliography
Chapter 67: Micronutrient Mineral Deficiencies
Keywords
Bibliography
Part VI: Fluid and Electrolyte Disorders
Chapter 68: Electrolyte and Acid-Base Disorders
68.1 Composition of Body Fluids
Keywords
Total Body Water
Fluid Compartments
Electrolyte Composition
Osmolality
Point-of-Care Testing
Bibliography
Composition of Body Fluids
68.2 Regulation of Osmolality and Volume
Keywords
Regulation of Osmolality
Regulation of Volume
Bibliography
Regulation of Osmolality and Volume
68.3 Sodium
Keywords
Sodium Metabolism
Body Content and Physiologic Function
Sodium Intake
Sodium Excretion
Hypernatremia
Etiology and Pathophysiology
Clinical Manifestations
Diagnosis
Treatment
Hyponatremia
Etiology and Pathophysiology
Clinical Manifestations
Diagnosis
Treatment
Bibliography
Sodium
68.4 Potassium
Keywords
Potassium Metabolism
Body Content and Physiologic Function
Potassium Intake
Potassium Excretion
Hyperkalemia
Etiology and Pathophysiology
Clinical Manifestations
Diagnosis
Treatment
Hypokalemia
Etiology and Pathophysiology
Clinical Manifestations
Diagnosis
Treatment
Bibliography
Potassium
68.5 Magnesium
Keywords
Magnesium Metabolism
Body Content and Physiologic Function
Magnesium Intake
Magnesium Excretion
Hypomagnesemia
Etiology and Pathophysiology
Clinical Manifestations
Diagnosis
Treatment
Hypermagnesemia
Etiology and Pathophysiology
Clinical Manifestations
Diagnosis
Treatment
Bibliography
Magnesium
68.6 Phosphorus
Keywords
Phosphorus Metabolism
Body Content and Physiologic Function
Phosphorus Intake
Phosphorus Excretion
Hypophosphatemia
Etiology and Pathophysiology
Clinical Manifestations
Diagnosis
Treatment
Hyperphosphatemia
Etiology and Pathophysiology
Clinical Manifestations
Diagnosis
Treatment
Bibliography
Phosphorus
68.7 Acid-Base Balance
Keywords
Acid-Base Physiology
Terminology
Physiologic Buffers
Normal Acid-Base Balance
Renal Mechanisms
Clinical Assessment of Acid-Base Disorders
Terminology
Diagnosis
Metabolic Acidosis
Etiology and Pathophysiology
Clinical Manifestations
Diagnosis
Treatment
Metabolic Alkalosis
Etiology and Pathophysiology
Clinical Manifestations
Diagnosis
Treatment
Respiratory Acidosis
Etiology and Pathophysiology
Clinical Manifestations
Diagnosis
Treatment
Respiratory Alkalosis
Etiology and Pathophysiology
Clinical Manifestations
Diagnosis
Treatment
Bibliography
Acid-Base Balance
Chapter 69: Maintenance and Replacement Therapy
Keywords
Maintenance Therapy
Maintenance Water
Intravenous Solutions
Glucose
Selection of Maintenance Fluids
Variations in Maintenance Water and Electrolytes
Replacement Fluids
Bibliography
Chapter 70: Deficit Therapy
Keywords
Clinical Manifestations
Laboratory Findings
Calculation of the Fluid Deficit
Approach to Severe Dehydration
Monitoring and Adjusting Therapy
Hyponatremic Dehydration
Hypernatremic Dehydration
Bibliography
Chapter 71: Fluid and Electrolyte Treatment of Specific Disorders
Acute Diarrhea
Pyloric Stenosis
Perioperative Fluids
Part VII: Pediatric Drug Therapy
Chapter 72: Pediatric Pharmacogenetics, Pharmacogenomics, and Pharmacoproteomics
Keywords
Definition of Terms
Developmental or Pediatric Pharmacogenetics and Pharmacogenomics
Pharmacoproteomic and Metabolomic Tools
Drug Biotransformation: Applications to Pediatric Therapy
CYP2D6
CYP2C9
CYP2C19
CYP3A4, CYP3A5, and CYP3A7
Glucuronosyl Transferases (UGTs)
Thiopurine S-Methyltransferase
Pharmacogenetics of Drug Transporters
Adenosine Triphosphate–Binding Cassette Superfamily
Organic Anion–Transporting Polypeptides
Organic Cation Transporters
Polymorphisms in Drug Receptors
Current and Future Applications in Pediatrics
Bibliography
Chapter 73: Principles of Drug Therapy
Keywords
General Pharmacokinetic and Pharmacodynamic Principles
Impact of Ontogeny on Drug Disposition
Drug Absorption
Peroral Absorption
Extravascular Drug Absorption
Drug Distribution
Drug Metabolism
Renal Drug Elimination
Impact of Ontogeny on Pharmacodynamics
Surrogate Endpoints
Additional Considerations in Pediatric Therapeutics
Pediatric Dose and Regimen Selection
Therapeutic Drug Monitoring
Drug Formulation and Administration
Peroral Drug Administration
Parenteral Drug Administration
Other Routes for Drug Administration
Adherence and Compliance
Drug-Drug Interactions
Adverse Drug Reactions
Personalized Medicine
Bibliography
Chapter 74: Anesthesia and Perioperative Care
Keywords
Preanesthetic Evaluation
Respiratory System
Airway Evaluation
Cardiovascular System
Hematologic System
Neurologic System
Psychological Assessment
Genetic Evaluation
Preoperative Preparation
Preoperative Fasting
The Full Stomach
Parental Presence During Induction of Anesthesia
General Anesthesia
Analgesia
Hypnosis and Amnesia
Akinesia (Immobility or Muscular Relaxation)
Monitoring
Specific Medications
Inhalational Anesthetics
Respiratory Effects.
Cardiovascular Effects.
Sevoflurane
Isoflurane
Desflurane
Nitrous Oxide
Intravenous Anesthetic Agents
Propofol
Etomidate
Ketamine
Opioids
Benzodiazepines
Dexmedetomidine
Barbiturates
Neuromuscular Blocking Agents
Induction of General Anesthesia
Complications During Induction
Maintenance of Anesthesia
Temperature Management
Fluid Management
Postanesthesia Care Unit
Postanesthetic Complications
Awareness During Anesthesia
Postoperative Nausea and Vomiting
Thermoregulation and Malignant Hyperthermia
Mediastinal Masses
Postoperative Apnea
Postoperative Pain Management
Regional Anesthesia
74.1 Anesthetic Neurotoxicity
Keywords
Bibliography
Bibliography
Chapter 75: Procedural Sedation
Keywords
Bibliography
Chapter 76: Pediatric Pain Management
Keywords
Definition and Categories of Pain
Assessment and Measurement of Pain in Children
Age-Specific and Developmentally Specific Measures
The Newborn and Infant
The Older Child
The Cognitively Impaired Child
Conceptual Framework for Treatment of Pediatric Pain
Pharmacologic Treatment of Pain
Acetaminophen, Aspirin, Nonsteroidal Antiinflammatory, and Coxib Drugs
Opioids
Local Anesthetics
Unconventional Medications in Pediatric Pain
Antidepressant Medications
Antiepileptic Drugs
Benzodiazepines
Antipsychotics and Major Sedatives
Other Pain Control Medications
Nonpharmacologic Treatment of Pain
Invasive Interventions for Treating Pain
Head and Neck Blocks
Upper-Extremity Blocks
Trunk and Abdominal Visceral Blocks
Lower-Extremity Blocks
Sympathetic Blocks
Epidural Anesthesia (Thoracic, Lumbar, and Caudal)
Intrathecal Analgesia
Nerve Ablation and Destruction
Considerations for Special Pediatric Populations
Pain Perception and Effects of Pain on Newborns and Infants
Children With Cancer Pain
Children With Pain Associated With Advanced Disease
Chronic and Recurrent Pain Syndromes
Neuropathic Pain Syndromes
Myofascial Pain Disorders and Fibromyalgia
Erythromelalgia
Other Chronic Pain Conditions in Children
Managing Complex Chronic Pain Problems
Bibliography
Chapter 77: Poisoning
Keywords
Prevention
Approach to the Poisoned Patient
History
Symptoms
Past Medical and Developmental History
Social History
Physical Examination
Laboratory Evaluation
Additional Diagnostic Testing
Principles of Management
Supportive Care
Decontamination
Syrup of Ipecac
Gastric Lavage
Single-Dose Activated Charcoal
Whole-Bowel Irrigation
Directed Therapy
Antidotal Therapy
Intralipid Emulsion Therapy
Enhanced Elimination
Urinary Alkalinization
Hemodialysis
Multidose Activated Charcoal
Select Compounds in Pediatric Poisoning
Pharmaceuticals
Analgesics
Acetaminophen.
Pathophysiology.
Clinical and Laboratory Manifestations.
Treatment.
1. Prophylactic.
2. Hepatic Injury.
3. Acute Liver Failure.
4. Repeated Supratherapeutic Ingestion.
Salicylates.
Pathophysiology.
Clinical and Laboratory Manifestations.
Treatment.
Ibuprofen and Other Nonsteroidal Antiinflammatory Drugs (NSAIDs).
Pathophysiology.
Clinical and Laboratory Manifestations.
Treatment.
Prescription Opioids.
Pathophysiology.
Clinical and Laboratory Manifestations.
Treatment.
Cardiovascular Medications
β-Adrenergic Receptor Blockers.
Pathophysiology.
Clinical and Laboratory Manifestations.
Treatment.
Calcium Channel Blockers.
Pathophysiology.
Clinical and Laboratory Manifestations.
Treatment.
Clonidine.
Pathophysiology.
Clinical and Laboratory Manifestations.
Treatment.
Digoxin.
Pathophysiology.
Clinical and Laboratory Manifestations.
Treatment.
Iron.
Pathophysiology.
Clinical and Laboratory Manifestations.
Treatment.
Oral Hypoglycemics
Pathophysiology.
Clinical and Laboratory Manifestations.
Treatment.
Psychiatric Medications: Antidepressants
Tricyclic Antidepressants.
Pathophysiology.
Clinical and Laboratory Manifestations.
Treatment.
1. Altered Mental State.
2. Widened QRS on ECG.
3. Hypotension.
4. Seizures.
Selective Serotonin Reuptake Inhibitors.
Pathophysiology.
Clinical and Laboratory Manifestations.
Treatment.
Atypical Antidepressants.
Clinical and Laboratory Manifestations.
Treatment.
Monoamine Oxidase Inhibitors.
Psychiatric Medications: Antipsychotics
Pathophysiology.
Clinical and Laboratory Manifestations.
Management.
Household Products
Caustics
Pathophysiology.
Clinical Manifestations.
Treatment.
Pesticides
Cholinesterase-Inhibiting Insecticides.
Pathophysiology.
Clinical and Laboratory Manifestations.
Treatment.
Pyrethrins and Pyrethroids.
Pathophysiology.
Clinical and Laboratory Manifestations.
Treatment.
Hydrocarbons
Pathophysiology.
Clinical and Laboratory Manifestations.
Treatment.
Toxic Alcohols
Methanol
Pathophysiology.
Clinical and Laboratory Manifestations.
Treatment.
Ethylene Glycol
Pathophysiology.
Clinical and Laboratory Manifestations.
Treatment.
Plants
Toxic Gases
Carbon Monoxide
Pathophysiology.
Clinical and Laboratory Manifestations.
Treatment.
Hydrogen Cyanide
Pathophysiology.
Clinical and Laboratory Manifestations.
Treatment.
Miscellaneous Toxic Agents Found in the Home
Nicotine-Containing Products
Pathophysiology.
Clinical and Laboratory Manifestations.
Treatment.
Single-Use Detergent Sacs
Electric Dishwasher Detergent
Magnets
Batteries
Bibliography
Chapter 78: Complementary Therapies and Integrative Medicine
Keywords
Dietary Supplements
Dietary Supplement Safety
Dietary Supplement Efficacy
Massage and Chiropractic
Mind-Body Therapies
Acupuncture
Cannabis
Internet Resources
Bibliography
Part VIII: Emergency Medicine and Critical Care
Chapter 79: Emergency Medical Services for Children
Keywords
Primary Care Physician and Office Preparedness
Staff Training and Continuing Education
Protocols
Resuscitation Equipment
Transport
Pediatric Prehospital Care
Access to the EMS System
Provider Capability
Destination Determination
The Emergency Department
Disaster Preparedness
79.1 Interfacility Transport of the Seriously Ill or Injured Pediatric Patient
Keywords
Communications and Dispatch Center
Medical Control Physician
Transport Team
Ground vs Air Ambulance
Transport Physiology
Safety
Family-Centered Care
Referring Hospital Responsibilities
Educational Outreach
Bibliography
79.2 Outcomes and Risk Adjustment of Pediatric Emergency Medical Services
Keywords
Outcome Measures in Emergency Medical Services for Children
Risk Adjustment
Risk Adjustment Tools in the ED
Pediatric Risk of Admission II
Revised Pediatric Emergency Assessment Tool
Bibliography
79.3 Principles Applicable to the Developing World
Keywords
Continuum-of-Care Model
Prevention
Infectious Diseases
Injuries
Out-of-Hospital Care
Access to Care
Prehospital Care
Methods of Transport
Hospital-Based Care
Triage
Pediatric-Specific Emergency Centers
Practitioners
Clinical Guidelines
Trauma
Equipment
Inpatient Services
Humanitarian Disasters
Exchange and Dissemination of Information
Bibliography
Bibliography
Chapter 80: Triage of the Acutely Ill Child
Keywords
Assessment of Vital Signs
History
Physical Examination
Management
Disposition
Bibliography
Chapter 81: Pediatric Emergencies and Resuscitation
Keywords
Approach to the Emergency Evaluation of a Child
General Assessment
Primary Assessment
Airway and Breathing
Circulation
Disability
Alert, Verbal, Pain, Unresponsive Pediatric Response Scale.
Glasgow Coma Scale.
Exposure
Secondary Assessment
Tertiary Assessment
Recognition and Treatment of Respiratory Distress and Failure
Airway Obstruction
Airway Narrowing
Parenchymal Lung Disease
Advanced Airway Management Techniques
Bag-Valve-Mask Positive Pressure Ventilation
Endotracheal Intubation
Recognition and Management of Shock
Recognition of Bradyarrhythmias and Tachyarrhythmias
Bradyarrhythmias
Tachyarrhythmias
Recognition and Management of Cardiac Arrest
Vascular Access
Venous Access
Intraosseous Access
Arterial Access
Nonvascular Emergency Procedures
Thoracentesis and Chest Tube Placement
Pericardiocentesis
Postresuscitation Care
Bibliography
Chapter 82: Acute Care of Multiple Trauma
Keywords
Epidemiology
Regionalization and Trauma Teams
Primary Survey
Airway/Cervical Spine
Breathing
Circulation
Neurologic Deficit
Exposure and Environmental Control
Secondary Survey
Head Trauma
Cervical Spine Trauma
Thoracic Trauma
Abdominal Trauma
Pelvic Trauma
Lower Genitourinary Trauma
Extremity Trauma
Radiologic and Laboratory Evaluation
Psychological and Social Support
Bibliography
Chapter 83: Spinal Cord Injuries in Children
Keywords
Clinical Manifestations
Clearing the Cervical Spine in Children
Treatment
Prevention
Bibliography
Chapter 84: Care of Abrasions and Minor Lacerations
Keywords
Lacerations and Cuts
Epidemiology
Evaluation
Treatment
Abrasions
Treatment
Bibliography
Chapter 85: Neurologic Emergencies and Stabilization
Keywords
Neurocritical Care Principles
Traumatic Brain Injury
Etiology and Epidemiology
Pathology
Pathogenesis
Clinical Manifestations
Laboratory Findings
Diagnosis and Differential Diagnosis
Treatment
Supportive Care
Prognosis
Bibliography
Chapter 86: Brain Death
Keywords
Epidemiology
Clinical Manifestations and Diagnosis
Coma
Brainstem Reflexes
Apnea
Observation Periods
Ancillary Studies
Documentation
Supportive Care
Objections to the Idea of Brain Death
Bibliography
Chapter 87: Syncope
Keywords
Mechanisms
Evaluation
Treatment
87.1 Postural Tachycardia Syndrome
Keywords
Clinical Presentation
Diagnosis
Management
Bibliography
Bibliography
Chapter 88: Shock
Keywords
Epidemiology
Types of Shock
Pathophysiology
Clinical Manifestations
Diagnosis
Laboratory Findings
Treatment
Initial Management
Additional Early Considerations
Considerations for Continued Therapy
Prognosis
Bibliography
Chapter 89: Respiratory Distress and Failure
Keywords
Respiratory Distress
Respiratory Disease Manifesting as Respiratory Distress
Respiratory Distress Without Respiratory Disease
Cardiovascular Disease Manifesting as Respiratory Distress
Neurologic Disease Manifesting as Respiratory Distress
Toxic Metabolic States Manifesting as Respiratory Distress
Other Nonpulmonary Entities Manifesting as Respiratory Distress
Respiratory Failure
Pathophysiology
Ventilation-Perfusion Mismatch
Diffusion
Monitoring a Child in Respiratory Distress and Respiratory Failure
Clinical Examination
Blood Gas Abnormalities
Acid-Base Abnormalities
Metabolic Acidosis With Respiratory Compensation
Respiratory Acidosis With Metabolic Compensation
Assessment of Oxygenation and Ventilation Deficits
Management
Oxygen Administration
Airway Adjuncts
Inhaled Gases
Positive Pressure Respiratory Support
Endotracheal Intubation and Mechanical Ventilation
Transient Manual Ventilation in Immediate Preintubation and Postintubation Periods
89.1 Mechanical Ventilation
Keywords
Basic Concepts of Ventilator Management
Equation of Motion
Functional Residual Capacity
Time Constant
Critical Opening Pressure
Noninvasive Mechanical Ventilation
Invasive Mechanical Ventilation
Initiation of Inspiration and the Control Variable (Mode)
Control Modes
Intermittent Mandatory Ventilation Mode.
Assist-Control Mode.
Control Variable
Support Modes
Inspiratory Phase Characteristics
Termination of Inspiration (Cycle)
Expiratory Phase Maneuvers
Additional Ventilatory Modalities
Airway Pressure Release Ventilation
High-Frequency Ventilation
Conventional Ventilator Settings
Fraction of Inspired Oxygen
Mode
Tidal Volume and Rate
Inspiratory Time and Expiratory Time
Positive End-Expiratory Pressure
Patient-Ventilator Asynchrony
Triggering the Ventilator
Selection of Appropriate Inspiratory Time
Selection of Inspiratory Flow Pattern
Use of Support Modes
Use of Sedation and Pharmacologic Neuromuscular Blockade
Cardiopulmonary Interactions
Monitoring Respiratory Mechanics
Exhaled Tidal Volume
Peak Inspiratory Pressure
Respiratory System Dynamic Compliance and Static Compliance
Assessment of Auto-PEEP
Assessment of Dead Space Ventilation
Ventilator-Induced Lung Injury
Ventilator-Associated Pneumonia
Weaning
89.2 Long-Term Mechanical Ventilation
Bibliography
Bibliography
Chapter 90: Altitude-Associated Illness in Children (Acute Mountain Sickness)
Keywords
Etiology
General Effects of Hypobaric Hypoxia
Acclimatization
Acute Mountain Sickness
Epidemiology and Risk Factors
Pathophysiology
Prevention
Diagnosis
Periodic Breathing
Management
High-Altitude Cerebral Edema
High-Altitude Pulmonary Edema
Epidemiology and Risk Factors
Pathophysiology
Diagnosis
Management
Special Considerations
Reentry HAPE
Travel With Young Infants
Sickle Trait and Sickle Cell Disease
Bibliography
Chapter 91: Drowning and Submersion Injury
Keywords
Etiology
Epidemiology
Children <1 Yr Old
Children 1-4 Yr Old
School-Age Children
Adolescents
Underlying Conditions
Alcohol Use
Sports and Recreation
Global Impact of Drowning
Pathophysiology
Anoxic-Ischemic Injury
Pulmonary Injury
Cold Water Injury
Management
Initial Evaluation and Resuscitation
Hospital-Based Evaluation and Treatment
Cardiorespiratory Management
Neurologic Management
Other Management Issues
Hypothermia Management
Prognosis
Prevention
Bibliography
Chapter 92: Burn Injuries
Keywords
Epidemiology
Prevention
Acute Care, Resuscitation, and Assessment
Indications for Admission
First Aid Measures
Emergency Care
Classification of Burns
Estimation of Body Surface Area for a Burn
Treatment
Outpatient Management of Minor Burns
Fluid Resuscitation
Prevention of Infection and Surgical Management of the Burn Wound
Nutritional Support
Topical Therapy
Inhalational Injury
Pain Relief and Psychologic Adjustment
Reconstruction and Rehabilitation
School Reentry and Long-Term Outcome
Electrical Burns
Bibliography
Useful Links
Chapter 93: Cold Injuries
Keywords
Pathophysiology
Etiology
Clinical Manifestations
Frostnip
Immersion Foot (Trench Foot)
Frostbite
Hypothermia
Chilblain (Pernio)
Cold-Induced Fat Necrosis (Panniculitis)
Bibliography
Part IX: Human Genetics
Chapter 94: Integration of Genetics Into Pediatric Practice
Keywords
Diagnostic Testing
Predictive Testing
Predispositional Testing
Pharmacogenetic Testing
94.1 Genetic Counseling
Talking to Families
Genetic Counseling
Specific Condition or Conditions
Knowledge of the Diagnosis of the Particular Condition
Natural History of the Condition
Genetic Aspects of the Condition and Recurrence Risk
Prenatal Diagnosis and Prevention
Therapies and Referral
Support Groups
Follow-up
Nondirective Counseling
94.2 Management and Treatment of Genetic Disorders
Physiologic Therapies
Replacement Therapies
Enzyme Replacement
Transplantation
Bibliography
Chapter 95: The Genetic Approach in Pediatric Medicine
Keywords
The Burden of Genetic Disorders in Childhood
The Changing Paradigm of Genetics in Medicine
Ethics Issues
Bibliography
Chapter 96: The Human Genome
Keywords
Fundamentals of Molecular Genetics
Genetic Variation
Genotype-Phenotype Correlations in Genetic Disease
Human Genome Project
Bibliography
Chapter 97: Patterns of Genetic Transmission
Keywords
Family History and Pedigree Notation
Mendelian Inheritance
Autosomal Dominant Inheritance
Autosomal Recessive Inheritance
Pseudodominant Inheritance
X-Linked Inheritance
Y-Linked Inheritance
Inheritance Associated With Pseudoautosomal Regions
Digenic Inheritance
Pseudogenetic Inheritance and Familial Clustering
Nontraditional Inheritance
Mitochondrial Inheritance
Triplet Repeat Expansion Disorders
Genetic Imprinting
Multifactorial and Polygenic Inheritance
Bibliography
Chapter 98: Cytogenetics
Keywords
98.1 Methods of Chromosome Analysis
Bibliography
Methods of Chromosome Analysis
98.2 Down Syndrome and Other Abnormalities of Chromosome Number
Aneuploidy and Polyploidy
Down Syndrome
Bibliography
Down Syndrome and Other Abnormalities of Chromosome Number
98.3 Abnormalities of Chromosome Structure
Translocations
Inversions
Deletions and Duplications
Insertions
Isochromosomes
Marker and Ring Chromosomes
Bibliography
Abnormalities of Chromosome Structure
98.4 Sex Chromosome Aneuploidy
Turner Syndrome
Klinefelter Syndrome
47,XYY
Bibliography
Sex Chromosome Aneuploidy
98.5 Fragile Chromosome Sites
Bibliography
Fragile Chromosome Sites
98.6 Mosaicism
Pallister-Killian Syndrome
Hypomelanosis of Ito
98.7 Chromosome Instability Syndromes
98.8 Uniparental Disomy and Imprinting
Uniparental Disomy
Imprinting
Bibliography
Uniparental Disomy and Imprinting
Chapter 99: Genetics of Common Disorders
Keywords
99.1 Major Genetic Approaches to the Study of Common Pediatric Disorders
Linkage Mapping
Genetic Association
Bibliography
Chapter 100: Epigenome-Wide Association Studies and Disease
Keywords
Epigenetic Mechanisms of Disease: Viable Yellow Mouse Model
Epigenetics and Regulation of Gene Expression
Evolution of the Term “Epigenetics”
Pediatric Diseases Involving Epigenetic Processes
Epigenome-Wide Association Studies: DNA Methylation
Cell Fate Variability as Model for Epigenetics and Disease
Epigenetic Disease and Therapeutic Interventions
Bibliography
Chapter 101: Genetic Approaches to Rare and Undiagnosed Diseases
Keywords
Scope of Genetic Disease
NIH Undiagnosed Diseases Program
Clinical Evaluation
Commercial Genetic Studies
Single Nucleotide Polymorphism Arrays
Exome Sequencing
Gene Function Studies
Pediatric Issues
Considerations for Families of Undiagnosed Children
The Diagnostic Spectrum
Bibliography
Part X: Metabolic Disorders
Chapter 102: An Approach to Inborn Errors of Metabolism
Keywords
Newborn Screening
Clinical Manifestations of Genetic Metabolic Diseases
Treatment
Bibliography
Chapter 103: Defects in Metabolism of Amino Acids
103.1 Phenylalanine
Keywords
Severe Phenylalanine Hydroxylase Deficiency (Classic Phenylketonuria)
Clinical Manifestations
Non-PKU Hyperphenylalaninemia
Diagnosis
Neonatal Screening for Hyperphenylalaninemia
Treatment
Pregnancy in Women With PAH Deficiency (Maternal Phenylketonuria)
Hyperphenylalaninemia Caused by Deficiency of the Cofactor Tetrahydrobiopterin
Clinical Manifestations
Diagnosis
Measurement of Neopterin and Biopterin.
Cerebrospinal Fluid Studies.
BH4 Loading Test.
Molecular Testing.
Enzyme Assay.
Treatment
Genetics and Prevalence
Tetrahydrobiopterin Defects Without Hyperphenylalaninemia
Bibliography
103.2 Tyrosine
Keywords
Tyrosinemia Type I (Fumarylacetoacetate Hydrolase Deficiency, Hepatorenal Tyrosinemia)
Clinical Manifestations and Natural History
Laboratory Findings
Treatment and Outcome
Genetics and Prevalence
Tyrosinemia Type II (Tyrosine Aminotransferase Deficiency, Richner-Hanhart Syndrome, Oculocutaneous Tyrosinemia)
Tyrosinemia Type III (Primary Deficiency of 4-Hydroxyphenylpyruvate Dioxygenase)
Hawkinsinuria
Transient Tyrosinemia of the Newborn
Alkaptonuria
Tyrosine Hydroxylase Deficiency
Albinism
Oculocutaneous (Generalized) Albinism
OCA1 (Tyrosinase-Deficient Albinism)
OCA1 A (Tyrosinase-Negative OCA)
OCA1 B
OCA2 (Tyrosinase-Positive OCA)
OCA3 (Rufous Albinism)
OCA4
Ocular Albinism
Ocular Albinism Type 1 (Nettleship-Falls Ocular Albinism)
Syndromic Forms of Generalized Albinism
Hermansky-Pudlak Syndrome
Chédiak-Higashi Syndrome
Other Disorders Featuring Generalized Albinism
Localized Albinism
Piebaldism
Waardenburg Syndrome
Bibliography
103.3 Methionine
Keywords
Homocystinuria (Homocystinemia)
Homocystinuria Caused by Cystathionine β-Synthase Deficiency (Classic Homocystinuria)
Homocystinuria Caused by Defects in Methylcobalamin Formation
Homocystinuria Caused by Deficiency of Methylenetetrahydrofolate Reductase (MTHFR Deficiency)
Hypermethioninemia
Primary (Genetic) Hypermethioninemia
Classic Homocystinuria.
Hepatic Methionine Adenosyltransferase (MAT I/MAT III) Deficiency (Mudd Disease).
Glycine N-Methyltransferase Deficiency.
S-Adenosylhomocysteine Hydrolase (SAHH) Deficiency.
Tyrosinemia Type I.
Citrin Deficiency.
Acquired (Nongenetic) Hypermethioninemia
Primary Cystathioninemia (Cystathioninuria)
Bibliography
103.4 Cysteine and Cystine
Keywords
Sulfite Oxidase Deficiency and Molybdenum Cofactor Deficiency
Bibliography
103.5 Tryptophan
Keywords
Hartnup Disorder
Bibliography
103.6 Isoleucine, Leucine, Valine, and Related Organic Acidemias
Keywords
Maple Syrup Urine Disease
Classic Maple Syrup Urine Disease
Intermediate (Mild) Maple Syrup Urine Disease
Intermittent Maple Syrup Urine Disease
Thiamine-Responsive Maple Syrup Urine Disease
Maple Syrup Urine Disease Caused by Deficiency of E3 Subunit (MSUD Type 3)
Genetics and Prevalence of Maple Syrup Urine Disease
Branched-Chain α-Ketoacid Dehydrogenase Kinase Deficiency
Branched-Chain Amino Acid Transporter Deficiency
Isovaleric Acidemia
Multiple Carboxylase Deficiencies (Defects of Biotin Cycle)
Holocarboxylase Synthetase Deficiency
Biotinidase Deficiency
Multiple Carboxylase Deficiency Caused by Acquired Biotin Deficiency
3-Methylcrotonyl-CoA Carboxylase Deficiency
3-Methylglutaconic Acidurias
3-Methylglutaconyl-CoA Hydratase Deficiency
Barth Syndrome (TAZ-Related Disorder)
OPA3-Related 3-Methylglutaconic Aciduria (Costeff Syndrome)
Disorders Formerly Described as 3-Methylglutaconic Aciduria Type IV
DCMA Syndrome (DNAJC19-Related Syndrome, 3-Methylglutaconic Aciduria Type V)
β-Ketothiolase (3-Oxothiolase) Deficiency (Mitochondrial Acetoacetyl-CoA Thiolase [T2] Deficiency)
Cytosolic Acetoacetyl-CoA Thiolase Deficiency
Mitochondrial 3-Hydroxy-3-Methylglutaryl-CoA Synthase Deficiency
3-Hydroxy-3-Methylglutaryl-CoA Lyase Deficiency (3-Hydroxy-3-Methylglutaric Aciduria)
Succinyl-CoA:3-Oxoacid-CoA Transferase Deficiency
Mevalonate Kinase Deficiency
Mevalonic Aciduria
Hyperimmunoglobulinemia D Syndrome (Hyperimmunoglobulinemia D and Periodic Fever Syndrome)
Propionic Acidemia (Propionyl-CoA Carboxylase Deficiency)
Isolated Methylmalonic Acidemias
Combined Methylmalonic Aciduria and Homocystinuria (cblC, cblD, cblF, cblJ, and cblX Defects)
Isolated Homocystinuria
Combined Malonic and Methylmalonic Aciduria (ACSF3-Related Disorder)
Bibliography
103.7 Glycine
Hypoglycinemia
Hyperglycinemia
Nonketotic Hyperglycinemia (Glycine Encephalopathy)
Neonatal Nonketotic Hyperglycinemia
Infantile Nonketotic Hyperglycinemia
Late-Onset Nonketotic Hyperglycinemia
Transient Nonketotic Hyperglycinemia
Diagnosis and Treatment
Sarcosinemia
Primary Trimethylaminuria
Hyperoxaluria and Oxalosis
Primary Hyperoxaluria Type 1
Primary Hyperoxaluria Type 2 (l-Glyceric Aciduria)
Primary Hyperoxaluria Type 3
Creatine Deficiency Disorders
Bibliography
103.8 Serine Deficiency Disorders (Serine Biosynthesis and Transport Defects)
Keywords
3-Phosphoglycerate Dehydrogenase Deficiency
Phosphoserine Aminotransferase Deficiency
3-Phosphoserine Phosphatase Deficiency
Bibliography
103.9 Proline
Keywords
Hyperprolinemia Type I
Hyperprolinemia Type II
Prolidase Deficiency
Disorders of De Novo Proline Synthesis
Bibliography
103.10 Glutamic Acid
Keywords
Glutathione Synthetase Deficiency
Glutathione Synthetase Deficiency, Severe and Moderate Forms
Glutathione Synthetase Deficiency, Mild Form
5-Oxoprolinase Deficiency
γ-Glutamylcysteine Synthetase Deficiency (Glutamate-Cysteine Ligase Deficiency)
γ-Glutamyl Transpeptidase Deficiency (Glutathionemia)
Genetic Disorders of Metabolism of γ-Aminobutyric Acid
Bibliography
103.11 Genetic Disorders of Neurotransmitters
Keywords
Tyrosine Hydroxylase Deficiency (Infantile Parkinsonism, Autosomal Recessive Dopa-Responsive Dystonia, Autosomal Recessive Segawa Syndrome)
Aromatic l-Amino Acid Decarboxylase Deficiency
Tetrahydrobiopterin Deficiency
Tetrahydrobiopterin Deficiency With Hyperphenylalaninemia
Tetrahydrobiopterin Deficiency Without Hyperphenylalaninemia
GTP Cyclohydrolase 1 Deficiency (Hereditary Progressive Dystonia, Autosomal Dominant Dopa-Responsive Dystonia, Autosomal Dominant Segawa Syndrome)
Sepiapterin Reductase Deficiency
Dopamine β-Hydroxylase Deficiency
Monoamine Oxidase a Deficiency
Disorders of γ-Aminobutyric Acid (GABA) Metabolism
γ-Aminobutyric Acid Transaminase Deficiency
Succinic Semialdehyde Dehydrogenase Deficiency (γ-Hydroxybutyric Aciduria)
Defects in Neurotransmitter Transporter Proteins
Dopamine Transporter Protein Deficiency
Dopamine-Serotonin Vesicular Transporter Disease (Vesicular Monoamine Transporter Deficiency)
Histidine Decarboxylase Deficiency
Hyperprolinemia
3-Phosphoglycerate Dehydrogenase Deficiency
Phosphoserine Aminotransferase Deficiency
Nonketotic Hyperglycinemia
Bibliography
103.12 Urea Cycle and Hyperammonemia (Arginine, Citrulline, Ornithine)
Keywords
Genetic Causes of Hyperammonemia
Clinical Manifestations of Hyperammonemia
Diagnosis
Treatment of Acute Hyperammonemia
Long-Term Therapy
Carbamoyl Phosphate Synthetase 1 and N-Acetylglutamate Synthase Deficiencies
Ornithine Transcarbamylase Deficiency
Citrullinemia
Citrullinemia Type I (Argininosuccinate Synthetase Deficiency, Classic Citrullinemia)
Citrin Deficiency (Citrullinemia Type II)
Neonatal Intrahepatic Cholestasis (Citrullinemia Type II, Neonatal Form)
Citrullinemia Type II, Adult Form (Adult-Onset Citrullinemia; Citrullinemia Type II, Mild Form)
Argininosuccinate Lyase Deficiency (Argininosuccinic Aciduria)
Arginase 1 Deficiency (Hyperargininemia)
Transient Hyperammonemia of the Newborn
Disorders of Ornithine Metabolism
Gyrate Atrophy of the Retina and Choroid
Hyperammonemia-Hyperornithinemia-Homocitrullinemia Syndrome
Congenital Glutamine Deficiency
Bibliography
103.13 Histidine
Keywords
Bibliography
103.14 Lysine
Keywords
Pyridoxine (Vitamin B6)–Dependent Epilepsy
Antiquitin (α-Aminoadipic Semialdehyde Dehydrogenase) Deficiency
Pyridox(am)ine 5′-Phosphate Oxidase (PNPO) Deficiency
Sulfite Oxidase Deficiency and Molybdenum Cofactor Deficiency
Hyperprolinemia Type II
Hypophosphatasia
Glutaric Aciduria Type 1 (Glutaryl-CoA Dehydrogenase Deficiency)
Clinical Manifestations
Laboratory Findings
Treatment
Lysinuric Protein Intolerance (Familial Protein Intolerance)
Bibliography
103.15 N-Acetylaspartic Acid (Canavan Disease)
Keywords
Etiology and Pathology
Clinical Manifestations
Atypical Canavan Disease
Diagnosis
Treatment and Prevention
Bibliography
Chapter 104: Defects in Metabolism of Lipids
104.1 Disorders of Mitochondrial Fatty Acid β-Oxidation
Keywords
Defects in the β-Oxidation Cycle
Medium-Chain Acyl-CoA Dehydrogenase Deficiency
Clinical Manifestations
Laboratory Findings
Treatment
Prognosis
Very-Long-Chain Acyl-CoA Dehydrogenase Deficiency
Short-Chain Acyl-CoA Dehydrogenase Deficiency
Long-Chain 3-Hydroxyacyl-CoA Dehydrogenase/Mitochondrial Trifunctional Protein Deficiency
Short-Chain 3-Hydroxyacyl-CoA Dehydrogenase Deficiency
Short-Chain 2,3-Enoyl-CoA Hydratase Deficiency
Defects in the Carnitine Cycle
Plasma Membrane Carnitine Transport Defect (Primary Carnitine Deficiency)
Carnitine Palmitoyltransferase-IA Deficiency
Carnitine:Acylcarnitine Translocase Deficiency
Carnitine Palmitoyltransferase-II Deficiency
Defects in the Electron Transfer Pathway
Electron Transfer Flavoprotein and Electron Transfer Flavoprotein Dehydrogenase Deficiencies (Glutaric Acidemia Type 2, Multiple Acyl-CoA Dehydrogenation Defects)
Defects in the Ketone Synthesis Pathway
β-Hydroxy-β-Methylglutaryl-CoA Synthase Deficiency
β-Hydroxy-β-Methylglutaryl-CoA Lyase Deficiency (3 Hydroxy-3-Methylgutaric Aciduria)
Defects in Ketone Body Utilization
Monocarboxylate Transporter-1 Deficiency
Succinyl-CoA:3-Ketoacid-CoA Transferase Deficiency
β-Ketothiolase Deficiency
Bibliography
104.2 Disorders of Very-Long-Chain Fatty Acids and Other Peroxisomal Functions
Keywords
Peroxisomal Disorders
Etiology
Epidemiology
Pathology
Pathogenesis
Zellweger Spectrum Disorder
Rhizomelic Chondrodysplasia Punctata
Isolated Defects of Peroxisomal Fatty Acid Oxidation
Isolated Defects of Plasmalogen Synthesis
Adult (Classic) Refsum Disease
2-Methylacyl-CoA Racemase Deficiency (AMACR)
Laboratory Findings
Diagnosis
Complications
Treatment
Genetic Counseling
Adrenoleukodystrophy
Etiology
Epidemiology
Pathology
Pathogenesis
Clinical Manifestations
Laboratory and Radiographic Findings
Neuroimaging
Impaired Adrenal Function
Diagnosis and Differential Diagnosis
Complications
Treatment
Bone Marrow Transplantation
Lorenzo’s Oil Therapy
Supportive Therapy
Genetic Counseling and Prevention
Bibliography
Peroxisomal Disorders
Adrenoleukodystrophy
104.3 Disorders of Lipoprotein Metabolism and Transport
Keywords
Epidemiology of Blood Lipids and Cardiovascular Disease
Blood Lipids and Atherogenesis
Plasma Lipoprotein Metabolism and Transport
Transport of Exogenous (Dietary) Lipids
Transport of Endogenous Lipids From the Liver
High-Density Lipoprotein and Reverse Cholesterol Transport
Hyperlipoproteinemias
Hypercholesterolemia
Familial Hypercholesterolemia
Homozygous Familial Hypercholesterolemia
Heterozygous Familial Hypercholesterolemia
Familial Defective ApoB-100
Autosomal Recessive Hypercholesterolemia
Sitosterolemia
Polygenic Hypercholesterolemia
Hypercholesterolemia With Hypertriglyceridemia
Familial Combined Hyperlipidemia
Familial Dysbetalipoproteinemia (Type III Hyperlipoproteinemia)
Hypertriglyceridemias
Familial Chylomicronemia (Type I Hyperlipidemia)
Familial Hypertriglyceridemia (Type IV Hyperlipidemia)
Hepatic Lipase Deficiency
Disorders of High-Density Lipoprotein Metabolism
Primary Hypoalphalipoproteinemia
Familial Hyperalphalipoproteinemia
Familial Apolipoprotein A-I Deficiency
Tangier Disease
Familial Lecithin–Cholesterol Acyltransferase (LCAT) Deficiency
Cholesteryl Ester Transfer Protein Deficiency
Conditions Associated With Low Cholesterol
Abetalipoproteinemia
Familial Hypobetalipoproteinemia
Smith-Lemli-Opitz Syndrome
Disorders of Intracellular Cholesterol Metabolism
Cerebrotendinous Xanthomatosis
Wolman Disease and Cholesterol Ester Storage Disease
Niemann-Pick Disease Type C
Lipoprotein Patterns in Children and Adolescents
Blood Cholesterol Screening
Risk Assessment and Treatment of Hyperlipidemia
Pharmacologic Therapy.
Bibliography
104.4 Lipidoses (Lysosomal Storage Disorders)
Keywords
GM1 Gangliosidosis
The GM2 Gangliosidoses
Gaucher Disease
Niemann-Pick Disease
Fabry Disease
Fucosidosis
Schindler Disease
Metachromatic Leukodystrophy
Multiple-Sulfatase Deficiency
Krabbe Disease
Farber Disease
Wolman Disease and Cholesterol Ester Storage Disease
Bibliography
104.5 Mucolipidoses
Keywords
I-Cell Disease
Pseudo-Hurler Polydystrophy
Bibliography
Chapter 105: Defects in Metabolism of Carbohydrates
105.1 Glycogen Storage Diseases
Keywords
Liver Glycogenoses
Type I Glycogen Storage Disease (Glucose-6-Phosphatase or Translocase Deficiency, Von Gierke Disease)
Clinical Manifestations
Diagnosis
Treatment
Prognosis
Type III Glycogen Storage Disease (Debrancher Deficiency, Limit Dextrinosis)
Clinical Manifestations
Diagnosis
Treatment
Type IV Glycogen Storage Disease (Branching Enzyme Deficiency, Amylopectinosis, Polyglucosan Disease, or Andersen Disease)
Clinical Manifestations
Diagnosis
Treatment
Type VI Glycogen Storage Disease (Liver Phosphorylase Deficiency, Hers Disease)
Type IX Glycogen Storage Disease (Phosphorylase Kinase Deficiency)
X-Linked Liver Phosphorylase Kinase Deficiency (From PHKA2 Variants)
Autosomal Liver and Muscle Phosphorylase Kinase Deficiency (From PHKB Variants)
Autosomal Liver Phosphorylase Kinase Deficiency (From PHKG2 Variants)
Phosphorylase Kinase Deficiency Limited to Heart
Diagnosis
Treatment and Prognosis
Liver Glycogen Synthase Deficiency
Hepatic Glycogenosis With Renal Fanconi Syndrome (Fanconi-Bickel Syndrome)
Muscle Glycogenoses
Type II Glycogen Storage Disease (Lysosomal Acid α-1,4-Glucosidase Deficiency, Pompe Disease)
Clinical Manifestations
Laboratory Findings
Diagnosis
Treatment
Glycogen Storage Diseases Mimicking Hypertrophic Cardiomyopathy (Danon Disease)
Adenosine Monophosphate–Activated Protein Kinase γ2 Deficiency (PRKAG2 Deficiency)
Muscle Glycogen Synthase Deficiency
Late-Onset Polyglucosan Body Myopathy (From GYG1 Variants)
Type V Glycogen Storage Disease (Muscle Phosphorylase Deficiency, McArdle Disease)
Clinical Manifestations
Diagnosis
Treatment
Type VII Glycogen Storage Disease (Muscle Phosphofructokinase Deficiency, Tarui Disease)
Clinical Manifestations
Diagnosis
Treatment
Muscle-Specific Phosphorylase Kinase Deficiency (From PHKA1 Variants)
Other Muscle Glycogenoses With Muscle Energy Impairment
Bibliography
105.2 Defects in Galactose Metabolism
Keywords
Galactose-1-Phosphate Uridyl Transferase Deficiency Galactosemia
Clinical Manifestations
Diagnosis
Genetics
Treatment and Prognosis
Galactokinase Deficiency
Uridine Diphosphate Galactose-4-Epimerase Deficiency
Bibliography
105.3 Defects in Fructose Metabolism
Keywords
Deficiency of Fructokinase (Essential or Benign Fructosuria)
Deficiency of Fructose-1,6-Bisphosphate Aldolase (Aldolase B, Hereditary Fructose Intolerance)
Epidemiology and Genetics
Clinical Manifestations
Diagnosis
Treatment
Bibliography
105.4 Defects in Intermediary Carbohydrate Metabolism Associated With Lactic Acidosis
Keywords
Disorders of Gluconeogenesis
Deficiency of Glucose-6-Phosphatase (Type I Glycogen Storage Disease)
Fructose-1,6-Diphosphatase Deficiency
Phosphoenolpyruvate Carboxykinase Deficiency
Disorders of Pyruvate Metabolism
Pyruvate Dehydrogenase Complex Deficiency
Clinical Manifestations
Treatment
Deficiency of Pyruvate Carboxylase
Deficiency of Pyruvate Carboxylase Secondary to Deficiency of Holocarboxylase Synthase or Biotinidase
Mitochondrial Respiratory Chain Defects (Oxidative Phosphorylation Disease)
Leigh Disease (Subacute Necrotizing Encephalomyelopathy)
Bibliography
105.5 Defects in Pentose Metabolism
Keywords
Essential Pentosuria
Transaldolase Deficiency
Ribose-5-Phosphate Isomerase Deficiency
Bibliography
105.6 Disorders of Glycoprotein Degradation and Structure
Sialidosis and Galactosialidosis
Aspartylglucosaminuria
α-Mannosidosis
Bibliography
105.7 Congenital Disorders of Glycosylation
Keywords
Congenital Disorders of Protein N-Glycosylation
Phosphomannomutase-2 Deficiency (PMM2-CDG)
Clinical Manifestations
Pathophysiology
Diagnosis
Treatment
Mannosephosphoisomerase Deficiency (MPI-CDG)
Clinical Manifestations
Pathophysiology
Diagnosis
Treatment
Glucosyltransferase-1 Deficiency (ALG6-CDG)
Clinical Manifestations
Pathophysiology
Diagnosis
Treatment
UDP-GlcNAc:Dol-P-GlcNAc-P Transferase Deficiency (DPAGT1-CDG)
Clinical Manifestations
Pathophysiology
Diagnosis
Treatment
Congenital Disorders of Protein O-Glycosylation
Cerebro-Ocular Dysplasia–Muscular Dystrophy and Muscle-Eye-Brain Disease Spectrum (POMT1-CDG, POMT2-CDG, POMGNT1-CDG)
Defects in Lipid Glycosylation and in Glycosylphosphatidylinositol Anchor Biosynthesis
Hyperphosphatasia–Intellectual Disability Syndromes: PIGA Deficiency (PIGA-CDG)
Defects in Multiple Glycosylation Pathways and in Other Pathways, Including Dolicholphosphate Biosynthesis Defects
Steroid 5α-Reductase Deficiency (SRD5A3-CDG)
Clinical Manifestations
Pathophysiology
Diagnosis
Treatment
Autosomal Recessive Cutis Laxa Type 2 (ARCL-2A or ATP6V0A2-CDG, ATP6V1A-CDG and ATP6V1E1-CDG)
Clinical Manifestations
Pathophysiology
Diagnosis
Treatment
Golgi-α1-2 Mannosidase-1 Deficiency (MAN1B1-CDG)
Clinical Manifestations
Pathophysiology
Diagnosis
Treatment
Phosphoglucomutase-1 Deficiency (PGM1-CDG)
Clinical Manifestations
Pathophysiology
Diagnosis
Treatment
Disorders of Golgi Homeostasis: TMEM199-, CCDC115-, ATP6AP2-CDG, and ATP6AP1-CDG
Clinical Manifestations
Pathophysiology
Diagnosis
Treatment
Manganese Transporter Defect: SLC39A8-CDG
Clinical Manifestations
Pathophysiology
Diagnosis
Treatment
Congenital Disorders of Deglycosylation
N-Glycanase 1 Deficiency (NGLY1 Defect)
Clinical Manifestations
Pathophysiology
Diagnosis
Treatment
Therapeutic Summary
Bibliography
Chapter 106: Mitochondrial Disease Diagnosis
Keywords
Overview of Mitochondrial Disease
When to Suspect Mitochondrial Disease
Mitochondrial Disease Inheritance
Diagnostic Testing for Mitochondrial Disease
Treatment Principles for Mitochondrial Disease
Bibliography
Chapter 107: Mucopolysaccharidoses
Keywords
Disease Entities
Mucopolysaccharidosis I
Hurler Syndrome
Hurler-Scheie Syndrome
Scheie Syndrome
Mucopolysaccharidosis II
Mucopolysaccharidosis III
Mucopolysaccharidosis IV
Mucopolysaccharidosis VI
Mucopolysaccharidosis VII
Mucopolysaccharidosis IX
Mucopolysaccharidosis Plus Syndrome
Diagnosis and Differential Diagnosis
Treatment
Bibliography
Chapter 108: Disorders of Purine and Pyrimidine Metabolism
Keywords
Gout
Genetics
Treatment
Abnormalities in Purine Salvage
Hypoxanthine-Guanine Phosphoribosyltransferase (HPRT) Deficiency
Genetics
Pathology
Clinical Manifestations
Diagnosis
Treatment
Adenine Phosphoribosyltransferase Deficiency (Dihydroxyadeninuria)
Genetics
Clinical Manifestations
Laboratory Findings
Treatment
Disorders Linked to Purine Nucleotide Synthesis
Phosphoribosylpyrophosphate Synthetase Superactivity and Deficiency
Genetics
Clinical Manifestations
Laboratory Findings
Treatment
Adenylosuccinase Lyase Deficiency (Succinylpurinuria)
Genetics
Clinical Manifestations
Pathology
Treatment
Aminoimidazole Carboxamide Ribotide (AICAR) Transformylase/Inosine Monophosphate (IMP) Cyclohydrolase Deficiency
Genetics
Clinical Features
Laboratory Findings
Treatment
Disorders Resulting From Abnormalities in Purine Catabolism
Myoadenylate Deaminase Deficiency (Muscle Adenosine Monophosphate Deaminase Deficiency)
Clinical Manifestations
Genetics
Laboratory Findings
Treatment
Adenosine Deaminase Deficiency
Purine Nucleoside Phosphorylase Deficiency
Xanthine Oxidoreductase Deficiency (Hereditary Xanthinuria/Molybdenum Cofactor Deficiency)
Genetics
Laboratory Findings
Treatment
Disorders of Pyrimidine Metabolism
Uridine Monophosphate Synthase Type 1 Deficiency (Hereditary Orotic Aciduria)
Genetics
Clinical Manifestations
Laboratory Findings
Treatment
Dihydroorotate Dehydrogenase Deficiency (Miller Syndrome)
Clinical Manifestations
Laboratory Findings
Treatment
Dihydropyrimidine Dehydrogenase Deficiency (Thymine-Uraciluria, Pyrimidinuria)
Genetics
Clinical Manifestations
Laboratory Findings
Treatment
Dihydropyrimidinase Deficiency (Dihydropyrimidinuria)
Genetics
Clinical Manifestations
Laboratory Findings
Deficiency of β-Ureidopropionase (N-Carbamyl-β-Amino Aciduria)
Genetics
Clinical Manifestations
Laboratory Findings
Treatment
Pyrimidine 5′-Nucleotidase Deficiency
Genetics
Clinical Manifestations
Laboratory Findings
Treatment
Overactive Cytosolic 5′-Nucleotidase (Pyrimidine Nucleotide Depletion)
Clinical Manifestations
Treatment
Thymidine Phosphorylase Deficiency (Mitochondrial Neurogastrointestinal Encephalomyopathy)
Genetics
Clinical Manifestations
Laboratory Findings
Treatment
Thymidine Kinase 2 Deficiency
Genetics
Clinical Manifestations
Treatment
Bibliography
Gout
Abnormalities in Purine Salvage
Adenine Phosphoribosyltransferase Deficiency (Dihydroxyadeninuria)
Disorders Linked to Purine Nucleotide Synthesis
Adenylosuccinate Lyase Deficiency (Succinylpurinuria)
Adenylosuccinate Lyase Deficiency (Succinylpurinuria)
AICAR Transformylase/IMP Cyclohydrolase (ATIC) Deficiency (AICA-Ribosiduria)
Disorders Resulting From Abnormalities in Purine Catabolism
Xanthine Oxidoreductase Deficiency (Hereditary Xanthinuria/Molybdenum Cofactor Deficiency)
Orotic Aciduria
Dihydroorotate Dehydrogenase Deficiency (Miller Syndrome)
Dihydropyrimidine Dehydrogenase Deficiency (Thymine-Uraciluria, Pyrimidinuria)
Dihydropyrimidinase Deficiency (Dihydropyrimidinuria)
Deficiency of β-Ureidopropionase (N-Carbamyl-β-Amino Aciduria)
Pyrimidine 5′-Nucleotidase Deficiency
Overactive Cytosolic 5′ Nucleotidase (Pyrimidine Nucleotide Depletion)
Thymidine Phosphorylase Deficiency (Mitochondrial Neurogastrointestinal Encephalomyopathy, MNGIE)
Thymidine Kinase 2 (TK2) Deficiency
Chapter 109: Hutchinson-Gilford Progeria Syndrome (Progeria)
Keywords
Clinical Manifestations
Dermatologic Changes
Failure to Thrive
Ocular Abnormalities
Craniofacial and Dental Phenotypes
Bone and Cartilaginous Abnormalities
Hearing
Cardiovascular Disease
Cerebrovascular Arteriopathy and Stroke
Sexual Development
Normally Functioning Systems
Laboratory Findings
Molecular Pathogenesis
Diagnosis and Differential Diagnosis
Treatment and Prognosis
Patient Resources
Bibliography
Chapter 110: The Porphyrias
Keywords
The Heme Biosynthetic Pathway
Classification and Diagnosis of Porphyrias
First-Line Laboratory Diagnostic Testing
Blistering Cutaneous Porphyrias
Nonblistering Cutaneous Porphyria
Second-Line Testing
Testing for Subclinical Porphyria
δ-Aminolevulinic Acid Dehydratase Deficient Porphyria
Etiology
Pathology and Pathogenesis
Clinical Manifestations
Laboratory Findings
Diagnosis and Differential Diagnosis
Treatment
Prognosis
Prevention and Genetic Counseling
Acute Intermittent Porphyria
Etiology
Pathology and Pathogenesis
Neurologic Mechanisms
Epidemiology
Clinical Manifestations
Laboratory Findings
Diagnosis and Differential Diagnosis
Complications
Treatment
Hemin
General and Supportive Measures
Carbohydrate Loading
Other Therapies
Seizures and Other Complications
Safe and Unsafe Drugs
Other Situations
Prognosis
Prevention
Genetic Counseling
Congenital Erythropoietic Porphyria
Etiology
Pathology and Pathogenesis
Clinical Manifestations
Laboratory Findings
Diagnosis and Differential Diagnosis
Treatment
Prognosis
Prevention and Genetic Counseling
Porphyria Cutanea Tarda
Etiology
Epidemiology
Pathology and Pathogenesis
Susceptibility Factors
Iron
Hepatitis C
Human Immunodeficiency Virus
Ethanol
Smoking and Cytochrome P450 Enzymes
Antioxidant Status
Estrogens
Clinical Manifestations
Cutaneous Manifestations
Liver Abnormalities
Other Features and Associations
Laboratory Findings
Diagnosis and Differential Diagnosis
Complications
Treatment
Prognosis
Prevention and Genetic Counseling
Hepatoerythropoietic Porphyria
Etiology
Pathology and Pathogenesis
Clinical Manifestations
Laboratory Findings
Diagnosis and Differential Diagnosis
Treatment and Prognosis
Prevention and Genetic Counseling
Hereditary Coproporphyria
Etiology
Epidemiology
Pathology and Pathogenesis
Clinical Manifestations
Laboratory Findings
Diagnosis and Differential Diagnosis
Treatment and Prognosis
Variegate Porphyria
Etiology
Epidemiology
Pathology and Pathogenesis
Clinical Manifestations
Laboratory Findings
Diagnosis and Differential Diagnosis
Treatment
Prognosis and Prevention
Erythropoietic Protoporphyria and X-Linked Protoporphyria
Etiology
Epidemiology
Pathology and Pathogenesis
Clinical Manifestations
Laboratory Findings
Diagnosis and Differential Diagnosis
Complications
Treatment
Prognosis
Prevention and Genetic Counseling
Dual Porphyria
Porphyria Resulting From Tumors
Bibliography
Acute Porphyrias
Congenital Erythropoietic Porphyria
Porphyria Cutanea Tarda
Erythropoietic Protoporphyria and X-Linked Protoporphyria
Chapter 111: Hypoglycemia
Keywords
Definition
Significance and Sequelae
Substrate, Enzyme, and Hormonal Integration of Glucose Homeostasis
In the Newborn
In Older Infants and Children
Clinical Manifestations
Classification of Hypoglycemia in Infants and Children
Neonatal, Transient, Small-for-Gestational-Age, and Premature Infants
Infants Born to Diabetic Mothers
Persistent or Recurrent Hypoglycemia in Infants and Children
Hyperinsulinism
Endocrine Deficiencies
Substrate-Limited Etiologies
Ketotic Hypoglycemia
Branched-Chain Ketonuria (Maple Syrup Urine Disease)
Glycogen Storage Disease
Glucose-6-Phosphatase Deficiency (Type I Glycogen Storage Disease)
Amylo-1,6-Glucosidase Deficiency (Debrancher Enzyme Deficiency; Type III Glycogen Storage Disease)
Liver Phosphorylase Deficiency (Type VI Glycogen Storage Disease)
Glycogen Synthetase Deficiency
Disorders of Gluconeogenesis
Fructose-1,6-Diphosphatase Deficiency
Defects in Fatty Acid Oxidation
Acute Alcohol Intoxication
Salicylate Intoxication
Phosphoenolpyruvate Carboxykinase Deficiency
Pyruvate Carboxylase Deficiency
Other Enzyme Defects
Galactosemia (Galactose-1-Phosphate Uridyl Transferase Deficiency)
Fructose Intolerance (Fructose-1-Phosphate Aldolase Deficiency)
Defects in Glucose Transporters
GLUT-1 Deficiency
GLUT-2 Deficiency
Systemic Disorders
Diagnosis and Differential Diagnosis
Treatment
Prognosis
Bibliography
Part XI: The Fetus and the Neonatal Infant
Chapter 112: Overview of Morbidity and Mortality
Keywords
Infant Mortality
Rankings and Trends
Major Causes of Infant Death
Racial Disparity and Infant Mortality
Congenital Malformations
Sleep-Related Deaths (SUID, SIDS)
Infant Mortality Reduction
Preterm Birth
The Late Preterm Neonate
The Moderate and Early Preterm Neonate
Low Birthweight, Intrauterine Growth Restriction, and Small for Gestational Age
Bibliography
Chapter 113: The Newborn Infant
Keywords
113.1 History in Neonatal Pediatrics
113.2 Physical Examination of the Newborn Infant
General Appearance
Skin
Skull
Face
Eyes
Ears
Nose
Mouth
Neck
Chest
Lungs
Heart
Abdomen
Genitals
Anus
Extremities
Neurologic Examination
Bibliography
113.3 Routine Newborn Care
Maintenance of Body Heat
Antiseptic Skin and Cord Care
Newborn Prophylaxis and Screening
Bibliography
113.4 Circumcision
Bibliography
113.5 Parent–Infant Bonding
Rooming-in and Breastfeeding
Drugs and Breastfeeding
Contraindications to Breastfeeding
Bibliography
Chapter 114: High-Risk Pregnancies
Keywords
Genetic Factors
Maternal Factors
Bibliography
Chapter 115: The Fetus
Keywords
115.1 Fetal Growth and Maturity
Bibliography
115.2 Fetal Distress
Bibliography
115.3 Maternal Disease and the Fetus
Infectious Diseases
Noninfectious Diseases (see Table 114.2)
Bibliography
115.4 Medication and Teratogen Exposure
Bibliography
115.5 Radiation
Bibliography
115.6 Intrauterine Diagnosis of Fetal Disease
Bibliography
115.7 Treatment and Prevention of Fetal Disease
Bibliography
Chapter 116: Fetal Intervention and Surgery
Keywords
Fetal Therapy Ethics
Obstructive Uropathy
Nonobstructive Renal Disease
Congenital Diaphragmatic Hernia
Congenital Pulmonary Airway Malformation
Myelomeningocele
Other Indications
Fetal Centers
Bibliography
Chapter 117: The High-Risk Infant
117.1 Multiple-Gestation Pregnancies
Keywords
Monozygotic vs Dizygotic Twins
Examination of the Placenta
Incidence
Etiology
Atypical Twinning
Complications
Twin Syndromes (TRAP, TTTS)
Diagnosis
Prognosis
Treatment
Bibliography
117.2 Extremely and Very Preterm Infants
Keywords
Incidence
Etiology
Assessment of Gestational Age
Nursery Care
Thermal Control
Oxygen Administration
Nutrition for the High-Risk Infant
Early Parenteral Nutrition
Benefits of Human Milk
Enteral Nutrition
Standardized Feeding Guidelines
Transitioning to Discharge Nutrition
Prevention of Infection
Immaturity of Drug Metabolism
Morbidity and Mortality
Bibliography
117.3 Moderate and Late Preterm Infants
Keywords
Moderate Preterm Infant
Late Preterm Infant
Bibliography
117.4 Term and Postterm Infants
Keywords
Small for Gestational Age and IUGR
Large-for-Gestational-Age Infants
Postterm Infants
Bibliography
117.5 Follow-Up of High-Risk Infants After Discharge
Keywords
Discharge From the Hospital
Postdischarge Follow-Up
Medical Follow-Up
Developmental Follow-Up
Bibliography
Chapter 118: Transport of the Critically Ill Newborn
Keywords
Regionalized Care of Newborns
Levels of Neonatal Care
Transport of the Critically Ill Neonate
Bibliography
Chapter 119: Clinical Manifestations of Diseases in the Newborn Period
Keywords
Abnormal Movements
Altered Mental Status
Apnea
Congenital Anomalies
Cyanosis
Gastrointestinal Disturbances
Hypotension
Jaundice
Pain
119.1 Hyperthermia
Bibliography
119.2 Hypothermia and Cold Stress
Bibliography
119.3 Edema
Bibliography
119.4 Hypocalcemia
Bibliography
119.5 Hypermagnesemia
Bibliography
Chapter 120: Nervous System Disorders
Keywords
120.1 The Cranium
120.2 Traumatic, Epidural, Subdural, and Subarachnoid Hemorrhage
120.3 Intracranial-Intraventricular Hemorrhage and Periventricular Leukomalacia
Etiology
Epidemiology
Pathogenesis
Clinical Manifestations
Diagnosis
Prognosis
Prevention
Treatment
Bibliography
120.4 Hypoxic-Ischemic Encephalopathy
Etiology
Pathophysiology and Pathology
Clinical Manifestations
Diagnosis
Treatment
Prognosis
Bibliography
120.5 Spine and Spinal Cord
Bibliography
120.6 Peripheral Nerve Injuries
Brachial Palsy
Phrenic Nerve Paralysis
Facial Nerve Palsy
Bibliography
Chapter 121: Neonatal Resuscitation and Delivery Room Emergencies
Keywords
Neonatal Resuscitation
Resuscitation of the Preterm Infant
Special Circumstances in the Delivery Room
Meconium
Placental Abruption
Neonatal Encephalopathy
Airway Obstruction
Respiratory Distress
Abdominal Wall and Neural Tube Defects
Injury During Delivery
Central Nervous System
Fractures
Brachial Plexus Injuries
Ongoing Care After Resuscitation
Bibliography
Chapter 122: Respiratory Tract Disorders
122.1 Transition to Pulmonary Respiration
Keywords
The First Breath
Breathing Patterns in Newborns
122.2 Apnea
Keywords
Apnea of Prematurity
Treatment
Prognosis
Apnea of Prematurity and Sudden Infant Death Syndrome
Bibliography
122.3 Respiratory Distress Syndrome (Hyaline Membrane Disease)
Keywords
Incidence
Etiology and Pathophysiology
Clinical Manifestations
Diagnosis
Prevention
Treatment
Nasal Continuous Positive Airway Pressure
Mechanical Ventilation
Modes of Mechanical Ventilation
Permissive Hypercapnia and Avoidance of Hyperoxia
Discontinuation of Mechanical Ventilation
Surfactant Replacement Therapy
Other Pharmacologic Therapies
Complications
Bibliography
122.4 Bronchopulmonary Dysplasia
Keywords
Incidence
Etiology and Pathophysiology
Clinical Manifestations
Diagnosis
Prevention
Treatment
Diuretics and Fluid Restriction
Bronchodilators
Corticosteroids
Pulmonary Vasodilators
Chronic Respiratory Support
Prognosis
Bibliography
122.5 Patent Ductus Arteriosus
Keywords
Incidence and Pathophysiology
Clinical Manifestations
Treatment
Pharmacologic Closure
Surgical Ligation
Bibliography
122.6 Transient Tachypnea of the Newborn
Keywords
Bibliography
122.7 Aspiration of Foreign Material (Fetal Aspiration Syndrome, Aspiration Pneumonia)
Keywords
122.8 Meconium Aspiration
Keywords
Clinical Manifestations
Prevention
Treatment
Prognosis
Bibliography
122.9 Persistent Pulmonary Hypertension of the Newborn (Persistent Fetal Circulation)
Keywords
Pathophysiology
Clinical Manifestations
Diagnosis
Treatment
Prognosis
Bibliography
122.10 Diaphragmatic Hernia
Keywords
Congenital Diaphragmatic Hernia (Bochdalek)
Pathology and Etiology
Epidemiology
Diagnosis and Clinical Presentation
Treatment
Initial Management
Ventilation Strategies
Extracorporeal Membrane Oxygenation
Novel Strategies for Infants With Congenital Diaphragmatic Hernia
Surgical Repair
Outcome and Long-Term Survival
Bibliography
122.11 Foramen of Morgagni Hernia
122.12 Paraesophageal Hernia
122.13 Eventration
Bibliography
122.14 Extrapulmonary Air Leaks: Pneumothorax, Pneumomediastinum, Pulmonary Interstitial Emphysema, Pneumopericardium
Keywords
Etiology and Pathophysiology
Clinical Manifestations
Diagnosis
Treatment
Bibliography
122.15 Pulmonary Hemorrhage
Keywords
Bibliography
Chapter 123: Digestive System Disorders
123.1 Meconium Ileus, Peritonitis, and Intestinal Obstruction
Keywords
Meconium Plugs
Meconium Ileus
Meconium Peritonitis
Bibliography
123.2 Necrotizing Enterocolitis
Keywords
Pathology and Pathogenesis
Clinical Manifestations
Diagnosis
Treatment
Prognosis
Prevention
Bibliography
123.3 Jaundice and Hyperbilirubinemia in the Newborn
Keywords
Etiology
Clinical Manifestations
Differential Diagnosis
Physiologic Jaundice (Icterus Neonatorum)
Pathologic Hyperbilirubinemia
Jaundice Associated With Breastfeeding
Neonatal Cholestasis
Congenital Atresia of the Bile Ducts
Bibliography
123.4 Kernicterus
Keywords
Clinical Manifestations
Incidence and Prognosis
Prevention
Treatment of Hyperbilirubinemia
Phototherapy
Intravenous Immune Globulin
Metalloporphyrins
Exchange Transfusion
Bibliography
Chapter 124: Blood Disorders
124.1 Anemia in the Newborn Infant
Keywords
Normal Hematocrit and Hemoglobin Concentrations in Newborn Infants
Classification of Anemia and Diagnostic Evaluation
Red Blood Cell Loss
Red Blood Cell Destruction
Red Blood Cell Production
Treatment Options for Neonatal Anemia
Packed Red Blood Cell Transfusions
Erythropoietin
Bibliography
124.2 Hemolytic Disease of the Fetus and Newborn
Keywords
Hemolytic Disease Caused by Rh Incompatibility
Pathogenesis
Clinical Manifestations
Laboratory Data
Diagnosis
Antenatal Diagnosis
Postnatal Diagnosis
Treatment
Treatment of the Unborn Fetus
Treatment of the Liveborn Infant
Exchange Transfusion
Procedure
Intravenous Immune Globulin
Late Complications
Prevention of Rh Sensitization
Hemolytic Disease Caused by Blood Group A and B Incompatibility
Clinical Manifestations
Diagnosis
Treatment
Other Forms of Hemolytic Disease
Bibliography
124.3 Neonatal Polycythemia
Keywords
Bibliography
124.4 Hemorrhage in the Newborn Infant
Keywords
Vitamin K Deficiency Bleeding
Disseminated Intravascular Coagulopathy
Neonatal Thrombocytopenia
Bibliography
124.5 Nonimmune Hydrops
Keywords
Bibliography
Chapter 125: The Umbilicus
Keywords
Umbilical Cord
Hemorrhage
Granuloma
Infections
Umbilical Hernia
Congenital Omphalocele
Tumors
Bibliography
Chapter 126: Abstinence Syndromes
126.1 Neonatal Abstinence (Withdrawal)
Keywords
Treatment
Bibliography
126.2 Maternal Selective Serotonin Reuptake Inhibitors and Neonatal Behavioral Syndromes
Keywords
Bibliography
126.3 Fetal Alcohol Exposure
Keywords
Epidemiology
Diagnostic Criteria
Clinical Features
Interventions and Treatment
Outcomes
The Pediatrician’s Role
Bibliography
Chapter 127: The Endocrine System
Keywords
127.1 Infants of Diabetic Mothers
Keywords
Pathophysiology
Clinical Manifestations
Treatment
Prognosis
Bibliography
Bibliography
Chapter 128: Dysmorphology
Keywords
Classification of Birth Defects
Malformations and Dysplasias
Deformations
Disruptions
Multiple Anomalies: Syndromes and Sequences
Molecular Mechanisms of Malformations
Inborn Errors of Development
Sonic Hedgehog Pathway As Model
Cytogenetic Aberrations and Chromosomal Imbalance
Approach to the Dysmorphic Child
Medical History
Physical Examination
Imaging Studies
Diagnosis
Laboratory Studies and Genetic Testing
Management and Counseling
Bibliography
Chapter 129: Epidemiology of Infections
Keywords
Incidence and Epidemiology
Early- and Late-Onset Neonatal Infections
Pathogenesis
Early-Onset Infections
Late-Onset Infections
Infection in Premature Infants
Clinical Manifestations
Bacterial Sepsis
Systemic Inflammatory Response Syndrome
Temperature Instability
Respiratory and Cardiovascular Symptoms
Conjunctivitis
Skin and Soft Tissue Infection
Omphalitis
Tetanus
Laboratory Findings
General Approach to Management
Prevention
Bibliography
Chapter 130: Healthcare-Acquired Infections
Keywords
Incidence
Epidemiology
Pathogenesis
Types of Infection
Central Line–Associated Bloodstream Infection
Healthcare-Associated Pneumonia
Skin and Soft Tissue Infection
Invasive Fungal Infection
Viral Infection
Prevention
Hand Hygiene
Central Line–Associated Bloodstream Infection
Ventilator-Associated Pneumonia
Early Feeding and Human Milk
Antifungal Prophylaxis
Nasal Decolonization
Bibliography
Chapter 131: Congenital and Perinatal Infections
Keywords
131.1 Congenital Infections
General Approach
Pathogenesis
Clinical Manifestations
Diagnosis
During Pregnancy
Newborn Infant
Specific Infectious Agents
Bacteria
Viruses
Parasite
131.2 Perinatal Infections
General Approach
Pathogenesis
Clinical Manifestations
Specific Infectious Agents
Bacteria
Viruses
Fungi
Diagnosis
Bibliography
Part XII: Adolescent Medicine
Chapter 132: Adolescent Physical and Social Development
Keywords
Physical Development
Sexual Development
Somatic Growth
Neurologic, Cognitive, and Moral Development
Psychosocial Development
Implications for Providers, Parents, and Policymakers
Bibliography
Chapter 133: Gender and Sexual Identity
Keywords
Terms and Definitions
Sex and Sexual Identity
Sex Assigned at Birth
Gender Terms
Sexual Orientation and Behavior
Transgender
Factors That Influence Sexual Identity Development
Nonconformity in Gender Expression Among Children and Adolescents
Prevalence
Etiology of Gender-Nonconforming Behavior
Stigma, Stigma Management, and Advocacy
Transgender and Gender-Nonconforming Identities Among Children and Adolescents
Prevalence
Etiology of Transgender or Gender-Nonconforming Identities
Clinical Presentation
The Diagnosis of Gender Dysphoria: Criteria and Critique
Transgender Identity Development
Interventions and Treatment
Bibliography
Chapter 134: Gay, Lesbian, and Bisexual Adolescents
Keywords
Prevalence of Homosexuality and Bisexuality in Youth
Development of Sexual Orientation in Childhood and Adolescence
Stigma, Risk, and Resilience
Health
Depression and Suicidality
Sexually Transmitted Infections
Substance Abuse
Obesity and Disordered Eating
Psychosocial Problems
Recommendations for Care
Evaluation
Medical and Sexual Health
Mental Health
Bibliography
Chapter 135: Transgender Care
Keywords
Cultural and Clinical Competence
Gender Literacy
Assessment
Treatment
Families
Bibliography
Chapter 136: The Epidemiology of Adolescent Health Problems
Keywords
Access to Healthcare
Bibliography
Chapter 137: Delivery of Healthcare to Adolescents
Keywords
137.1 Legal Issues
Bibliography
137.2 Screening Procedures
Interviewing the Adolescent
Psychosocial Assessment
Physical Examination
Vision Testing
Audiometry
Blood Pressure Determination
Scoliosis
Breast Examination
Scrotum Examination
Pelvic Examination
Laboratory Testing
Bibliography
Bibliography
Chapter 138: Transitioning to Adult Care
Keywords
Bibliography
Chapter 139: Violent Behavior
Keywords
Epidemiology
Etiology
Clinical Manifestations
Diagnosis
Treatment
Prevention
Bibliography
Chapter 140: Substance Abuse
Keywords
Etiology
Epidemiology
Clinical Manifestations
Screening for Substance Abuse Disorders
Diagnosis
Complications
Treatment
Prognosis
Prevention
140.1 Alcohol
Pharmacology and Pathophysiology
Clinical Manifestations
Diagnosis
Treatment
Bibliography
140.2 Tobacco and Electronic Nicotine Delivery Systems
Keywords
Cigarettes
Pharmacology
Clinical Manifestations
Electronic Cigarettes (E-Cigarettes)
Hookah
Treatment
Bibliography
140.3 Marijuana
Keywords
Clinical Manifestations
Synthetic Marijuana
Bibliography
140.4 Inhalants
Keywords
Clinical Manifestations
Complications
Diagnosis
Treatment
Bibliography
140.5 Hallucinogens
Keywords
Lysergic Acid Diethylamide
Clinical Manifestations
Treatment
Methylenedioxymethamphetamine
Clinical Manifestations
Phencyclidine
Clinical Manifestations
Treatment
Bibliography
140.6 Cocaine
Clinical Manifestations
Treatment
Bibliography
140.7 Amphetamines
Clinical Manifestations
Treatment
Bibliography
140.8 Stimulant Abuse and Diversion
Clinical Manifestations
Treatment
Bibliography
140.9 Opiates
Clinical Manifestations
Withdrawal
Overdose Syndrome
Treatment
Bibliography
140.10 Bath Salts
Clinical Manifestations
Treatment
Bibliography
Bibliography
Chapter 141: The Breast
Keywords
Female Disorders
Male Disorders
Bibliography
Chapter 142: Menstrual Problems
Keywords
Normal Menstruation
Menstrual Irregularities
142.1 Amenorrhea
Keywords
History and Physical Examination
Laboratory Studies
Treatment
Bibliography
142.2 Abnormal Uterine Bleeding
Keywords
Irregular Menstrual Bleeding
Heavy and Prolonged Menstrual Bleeding
Laboratory Findings
Treatment
Bibliography
142.3 Dysmenorrhea
Keywords
Bibliography
142.4 Premenstrual Syndrome and Premenstrual Dysphoric Disorder
Keywords
Bibliography
Bibliography
Chapter 143: Contraception
Keywords
Contraceptive Effectiveness
143.1 Contraceptive Use
Keywords
Sexual Activity
Use of Contraception Among Teens
Bibliography
143.2 Contraceptive Counseling
Keywords
Bibliography
143.3 Long-Acting Reversible Contraception
Keywords
Intrauterine Devices
Implants
Bibliography
143.4 Other Progestin-Only Methods
Keywords
Depo-Provera
Progestin-Only Pills
Bibliography
143.5 Combined Hormonal Contraceptives
Keywords
Combined Oral Contraceptives
Transdermal Patch
Vaginal Ring
Contraindications
Bibliography
143.6 Emergency Contraception
Keywords
Copper IUD
Ulipristal Acetate
Levonorgestrel
Bibliography
143.7 Dual Protection
Keywords
Condoms
Bibliography
143.8 Other Barrier Methods
Keywords
Diaphragm, Cervical Cap, and Sponge
143.9 Other Contraceptive Methods
Keywords
Spermicides
Withdrawal
Fertility Awareness–Based Methods
Lactational Amenorrhea Method
Bibliography
Chapter 144: Adolescent Pregnancy
Keywords
Epidemiology
Etiology
Clinical Manifestations
Diagnosis
Pregnancy Counseling and Initial Management
The Importance of Prevention
Adolescent Fathers
Medical Complications of Mothers and Babies
Psychosocial Outcomes/Risks for Mother and Child
Educational Issues
Substance Use
Repeat Pregnancy
Children Born to Teen Mothers
Prevention of Teen Pregnancies
Bibliography
Chapter 145: Adolescent Sexual Assault
Keywords
Epidemiology
Types of Rape
Clinical Manifestations
Interview and Physical Examination
Laboratory Data
Treatment
Prevention
Bibliography
Chapter 146: Sexually Transmitted Infections
Keywords
Etiology
Epidemiology
Pathogenesis
Screening
Common Infections and Clinical Manifestations
Urethritis
Epididymitis
Vaginitis
Cervicitis
Pelvic Inflammatory Disease
Genital Ulcer Syndromes
Genital Lesions and Ectoparasites
HIV Disease and Hepatitis B
Diagnosis
Treatment
Prevention
Bibliography
Chapter 147: Chronic Overlapping Pain Conditions
Keywords
Prevalence
Symptom/Disorder Overlap
Psychiatric Comorbidities
Predisposing Factors
Natural History
Proposed Pathophysiology
Treatment
147.1 Chronic Fatigue Syndrome
Keywords
Epidemiology
Pathogenesis
Clinical Manifestations
Diagnosis
Management
Prognosis
Bibliography
Bibliography
Part XIII: Immunology
Section 1: Evaluation of the Immune System
Chapter 148: Evaluation of Suspected Immunodeficiency
Keywords
Advanced Testing
Bibliography
Section 2: The T-, B-, and NK-Cell Systems
Chapter 149: Lymphocyte Development and Function
Keywords
Lymphopoiesis in the Fetus
T-Cell Development and Differentiation
B-Cell Development and Differentiation
Natural Killer–Cell Development
Lymphocyte Choreography
Postnatal Lymphocyte Behavior
Lymphoid Organ Development
Inheritance of Abnormalities in T-, B-, and NK-Cell Development
Bibliography
Chapter 150: Primary Defects of Antibody Production
Keywords
X-Linked Agammaglobulinemia
Genetics and Pathogenesis
Clinical Manifestations
Diagnosis
Common Variable Immunodeficiency
Genetics and Pathogenesis
Clinical Manifestations
Selective IgA Deficiency
Clinical Manifestations
IgG Subclass Deficiencies
Immunoglobulin Heavy- and Light-Chain Deletions
Transient Hypogammaglobulinemia of Infancy
Class Switch Defects
X-Linked Hyper-IgM Caused by Mutations in CD40 Ligand Gene
Genetics and Pathogenesis
Clinical Manifestations
Treatment
Autosomal Recessive Hyper-IgM
Genetics and Pathogenesis
Clinical Manifestations
Treatment and Prognosis
X-Linked Lymphoproliferative Disease
Genetics and Pathogenesis
Clinical Manifestations
150.1 Treatment of B-Cell Defects
Bibliography
Bibliography
Chapter 151: Primary Defects of Cellular Immunity
Keywords
Chromosome 22Q11.2 Deletion Syndrome
Genetics and Pathogenesis
Clinical Manifestations
Treatment
T-Cell Activation Defects
Chronic Mucocutaneous Candidiasis
Autoimmune Polyendocrinopathy- Candidiasis–Ectodermal Dysplasia
Bibliography
Chapter 152: Immunodeficiencies Affecting Multiple Cell Types
152.1 Severe Combined Immunodeficiency
Keywords
Pathogenesis
Clinical Manifestations
Treatment
Genetics
Bibliography
152.2 Combined Immunodeficiency
Keywords
Cartilage-Hair Hypoplasia
Genetics and Pathogenesis
Clinical Manifestations
Wiskott-Aldrich Syndrome
Genetics and Pathogenesis
Clinical Manifestations
Treatment
Ataxia-Telangiectasia
Genetics and Pathogenesis
Clinical Manifestations
Autosomal Dominant Hyper-IgE Syndrome
Genetics and Pathogenesis
Clinical Manifestations
DOCK8 Deficiency
Bibliography
152.3 Defects of Innate Immunity
Keywords
Interferon-γ Receptors 1 and 2, IL-12 Receptor β1, and IL-12P40 Defects
Pathogenesis
Clinical Manifestations
IL-1R–Associated Kinase 4 Deficiency and Myeloid Differentiation Factor 88
Pathogenesis
Clinical Manifestations
Natural Killer Cell Deficiency
Defects in Innate Responses to Viral Infection
Defects in Innate Responses to Fungi
Bibliography
152.4 Treatment of Cellular or Combined Immunodeficiency
152.5 Immune Dysregulation With Autoimmunity or Lymphoproliferation
Keywords
Autoimmune Lymphoproliferative Syndrome
Clinical Manifestations
Diagnosis
Treatment
Immune Dysregulation, Polyendocrinopathy, Enteropathy, X-Linked Syndrome
Clinical Manifestations
Treatment
Cytotoxic T-Lymphocyte Antigen 4 (CTLA4) Deficiency
Lipopolysaccharide (Lps)-Responsive Beige-Like Anchor Protein (LRBA) Deficiency
Activated Phosphoinositide 3-Kinase (PI3K) δ Syndromes
Signal Transducer and Activator of Transcription (STAT) Pathway Defects
Nuclear Factor-κB Pathway Defects
Tetratricopeptide Repeat Domain 7A (TTC7A) Deficiency
Deficiency of Adenosine Deaminase 2 (DADA2)
Bibliography
Section 3: The Phagocytic System
Chapter 153: Neutrophils
Keywords
The Phagocytic Inflammatory Response
Hematopoiesis
Neutrophil Maturation and Kinetics
Neutrophil Function
Bibliography
Chapter 154: Monocytes, Macrophages, and Dendritic Cells
Keywords
Development
Activation
Functional Activities
Dendritic Cells
Abnormalities of Monocyte-Macrophage or Dendritic Cell Function
Bibliography
Chapter 155: Eosinophils
Keywords
Diseases Associated With Eosinophilia
Allergic Diseases
Infectious Diseases
Hypereosinophilic Syndrome
Miscellaneous Diseases
Bibliography
Chapter 156: Disorders of Phagocyte Function
Keywords
Leukocyte Adhesion Deficiency
Genetics and Pathogenesis
Clinical Manifestations
Laboratory Findings
Treatment
Prognosis
Chédiak-Higashi Syndrome
Genetics and Pathogenesis
Clinical Manifestations
Laboratory Findings
Treatment
Myeloperoxidase Deficiency
Clinical Manifestations
Laboratory Findings
Treatment
Chronic Granulomatous Disease
Genetics and Pathogenesis
Clinical Manifestations
Laboratory Findings
Treatment
Genetic Counseling
Prognosis
Bibliography
Chapter 157: Leukopenia
Keywords
Neutropenia
Clinical Manifestations of Neutropenia
Laboratory Findings
Acquired Neutropenia
Infection-Related Neutropenia
Drug-Induced Neutropenia
Nutrition-Related Neutropenia
Immune-Mediated Neutropenia
Neutropenia Secondary to Bone Marrow Replacement
Neutropenia Secondary to Reticuloendothelial Sequestration
Inherited Neutropenia
Primary Disorders of Granulocytopoiesis
Disorders of Molecular Processing
Vesicular Trafficking Disorders
Disorders of Metabolism
Neutropenia in Disorders of Immune Dysfunction
Unclassified Neutropenic Disorders
Treatment
Lymphopenia
Acquired Lymphopenia
Inherited Lymphopenia
Bibliography
Chapter 158: Leukocytosis
Keywords
Neutrophilia
Acute Acquired Neutrophilia
Chronic Acquired Neutrophilia
Lifelong Neutrophilia
Additional Forms of Leukocytosis
Monocytosis
Eosinophilia
Basophilia
Lymphocytosis
Bibliography
Section 4: The Complement System
Chapter 159: Complement Components and Pathways
Keywords
Classical and Lectin Pathways
Alternative Pathway
Membrane Attack Complex
Control Mechanisms
Participation in Host Defense
Bibliography
Chapter 160: Disorders of the Complement System
160.1 Evaluation of the Complement System
Keywords
Bibliography
160.2 Genetic Deficiencies of Complement Components
Keywords
Bibliography
160.3 Deficiencies of Plasma, Membrane, or Serosal Complement Control Proteins
Keywords
Bibliography
160.4 Secondary Disorders of Complement
Keywords
Bibliography
160.5 Treatment of Complement Disorders
Keywords
Bibliography
Section 5: Hematopoietic Stem Cell Transplantation
Chapter 161: Principles and Clinical Indications of Hematopoietic Stem Cell Transplantation
Keywords
HSCT From an HLA-Identical Sibling Donor
Acute Lymphoblastic Leukemia
Acute Myeloid Leukemia
Chronic Myelogenous Leukemia
Juvenile Myelomonocytic Leukemia
Myelodysplastic Syndromes Other Than Juvenile Myelomonocytic Leukemia
Non-Hodgkin Lymphoma and Hodgkin Disease
Acquired Aplastic Anemia
Inherited Bone Marrow Failure Syndromes
Thalassemia
Sickle Cell Disease
Immunodeficiency Disorders
Inherited Metabolic Diseases
Bibliography
Chapter 162: Hematopoietic Stem Cell Transplantation From Alternative Sources and Donors
Keywords
Unrelated Donor Transplants
Umbilical Cord Blood Transplants
Haploidentical Transplants
Donor Versus Recipient NK-Cell Alloreactivity
Autologous Hematopoietic Stem Cell Transplantation
Bibliography
Chapter 163: Graft-Versus-Host Disease, Rejection, and Venoocclusive Disease
Keywords
Acute Graft-Versus-Host Disease
Chronic Graft-Versus-Host Disease
Graft Failure
Venoocclusive Disease
Bibliography
Chapter 164: Infectious Complications of Hematopoietic Stem Cell Transplantation
Keywords
Bibliography
Chapter 165: Late Effects of Hematopoietic Stem Cell Transplantation
Keywords
Endocrine Effects
Cardiovascular Effects
Secondary Malignancy
Graft-Versus-Host Disease
Other Effects
Special Considerations
Bibliography
Part XIV: Allergic Disorders
Chapter 166: Allergy and the Immunologic Basis of Atopic Disease
Keywords
Key Elements of Allergic Diseases
Allergens
T Cells
Innate Lymphoid Cells
Antigen-Presenting Cells
Immunoglobulin E and Its Receptors
Eosinophils
Mast Cells
Mechanisms of Allergic Tissue Inflammation
Genetic Basis of Atopy
Bibliography
Chapter 167: Diagnosis of Allergic Disease
Keywords
Allergy History
Physical Examination
Diagnostic Testing
In Vitro Tests
In Vivo Tests
Bibliography
Chapter 168: Allergic Rhinitis
Keywords
Etiology and Classification
Pathogenesis
Clinical Manifestations
Differential Diagnosis
Complications
Laboratory Findings
Treatment
Prognosis
Bibliography
Chapter 169: Childhood Asthma
Keywords
Etiology
Genetics
Environment
Epidemiology
Types of Childhood Asthma
Pathogenesis
Clinical Manifestations and Diagnosis
Differential Diagnosis
Laboratory Findings
Pulmonary Function Testing
Exhaled Nitric Oxide (FeNO)
Radiology
Treatment
Component 1: Regular Assessment and Monitoring
Component 2: Patient Education
Adherence
Component 3: Control of Factors Contributing to Asthma Severity
Eliminating and Reducing Problematic Environmental Exposures
Treating Comorbid Conditions
Component 4: Principles of Asthma Pharmacotherapy
“Step-Up, Step-Down” Approach
Referral to Asthma Specialist
Long-Term Controller Medications
Inhaled Corticosteroids
Systemic Corticosteroids
Long-Acting Inhaled β-Agonists
Combination ICS/LABA Therapy
Leukotriene-Modifying Agents
Nonsteroidal Antiinflammatory Agents
Long-Acting Inhaled Anticholinergics
Allergen Immunotherapy
Biologic Therapies
Omalizumab (Anti-IgE Antibody).
Mepolizumab (Anti–IL-5 Antibody).
Dupilumab (Anti–IL-4 Receptor α Antibody).
Quick-Reliever Medications
Short-Acting Inhaled β-Agonists
Anticholinergic Agents
Delivery Devices and Inhalation Technique
Asthma Exacerbations and Their Management
Home Management of Asthma Exacerbations
Emergency Department Management of Asthma Exacerbations
Hospital Management of Asthma Exacerbations
Special Management Circumstances
Management of Infants and Young Children
Asthma Management During Surgery
Prognosis
Prevention
Bibliography
Chapter 170: Atopic Dermatitis (Atopic Eczema)
Keywords
Etiology
Pathology
Pathogenesis
Clinical Manifestations
Laboratory Findings
Diagnosis and Differential Diagnosis
Treatment
Cutaneous Hydration
Topical Corticosteroids
Topical Calcineurin Inhibitors
Phosphodiesterase Inhibitor
Tar Preparations
Antihistamines
Systemic Corticosteroids
Cyclosporine
Dupilumab
Antimetabolites
Phototherapy
Unproven Therapies
Interferon-γ
Omalizumab
Allergen Immunotherapy
Probiotics
Chinese Herbal Medications
Vitamin D
Avoiding Triggers
Irritants
Foods
Aeroallergens
Infections
Complications
Prognosis
Prevention
Bibliography
Chapter 171: Insect Allergy
Keywords
Etiology
Pathogenesis
Clinical Manifestations
Diagnosis
Treatment
Venom Immunotherapy
Inhalant Allergy
Prevention
Bibliography
Chapter 172: Ocular Allergies
Keywords
Clinical Manifestations
Allergic Conjunctivitis
Vernal Keratoconjunctivitis
Atopic Keratoconjunctivitis
Giant Papillary Conjunctivitis
Contact Allergy
Diagnosis
Treatment
Bibliography
Chapter 173: Urticaria (Hives) and Angioedema
Keywords
Etiology and Pathogenesis
Physical Urticaria
Cold-Dependent Disorders
Cholinergic Urticaria
Dermatographism
Pressure-Induced Urticaria and Angioedema
Solar Urticaria
Aquagenic Urticaria
Chronic Idiopathic Urticaria and Angioedema
Diagnosis
Treatment
Hereditary Angioedema
Bibliography
Chapter 174: Anaphylaxis
Keywords
Etiology
Epidemiology
Pathogenesis
Clinical Manifestations
Laboratory Findings
Diagnosis
Treatment
Prevention
Bibliography
Chapter 175: Serum Sickness
Keywords
Etiology
Pathogenesis
Clinical Manifestations
Differential Diagnosis
Diagnosis
Treatment
Prevention
Bibliography
Chapter 176: Food Allergy and Adverse Reactions to Foods
Keywords
Genetics
Pathogenesis
Clinical Manifestations
Gastrointestinal Manifestations
Skin Manifestations
Respiratory Manifestations
Anaphylaxis
Diagnosis
Treatment
Prevention
Bibliography
Chapter 177: Adverse Reactions to Drugs
Keywords
Epidemiology
Pathogenesis and Clinical Manifestations
Drug Metabolism and Adverse Reactions
Risk Factors for Hypersensitivity Reactions
Diagnosis
Treatment
β-Lactam Hypersensitivity
Sulfonamides
Stevens-Johnson Syndrome and Toxic Epidermal Necrolysis
Hypersensitivity to Antiretroviral Agents
Chemotherapeutic Agents
Biologics
Vaccines
Perioperative Agents
Local Anesthetics
Insulin
Drug-Induced Hypersensitivity Syndrome
Red Man Syndrome
Radiocontrast Media
Narcotic Analgesics
Aspirin and Nonsteroidal Antiinflammatory Drugs
Bibliography
Part XV: Rheumatic Diseases of Childhood (Connective Tissue Disease, Collagen Vascular Diseases)
Chapter 178: Evaluation of Suspected Rheumatic Disease
Keywords
Symptoms Suggestive of Rheumatic Disease
Signs Suggestive of Rheumatic Disease
Laboratory Testing
Imaging Studies
Bibliography
Chapter 179: Treatment of Rheumatic Diseases
Keywords
Pediatric Rheumatology Teams and Primary Care Physicians
Therapeutics
Nonsteroidal Antiinflammatory Drugs
Nonbiologic Disease-Modifying Antirheumatic Drugs
Methotrexate
Hydroxychloroquine
Leflunomide
Sulfasalazine
Mycophenolate Mofetil
Glucocorticoids
Biologic Agents
Tumor Necrosis Factor-α Antagonists
Modulator of T-Cell Activation
B-Cell Depletion
Interleukin-1 Antagonists
Interleukin-6 Receptor Antagonist
Intravenous Immune Globulin
Cytotoxics
Cyclophosphamide
Other Drugs
Bibliography
Chapter 180: Juvenile Idiopathic Arthritis
Keywords
Epidemiology
Etiology
Pathogenesis
Clinical Manifestations
Diagnosis
Differential Diagnosis
Laboratory Findings
Treatment
Prognosis
Bibliography
Chapter 181: Ankylosing Spondylitis and Other Spondyloarthritides
Keywords
Epidemiology
Etiology and Pathogenesis
Clinical Manifestations and Diagnosis
Enthesitis-Related Arthritis
Psoriatic Arthritis
Juvenile Ankylosing Spondylitis
Arthritis With Inflammatory Bowel Disease
Laboratory Findings
Imaging
Differential Diagnosis
Treatment
Prognosis
Bibliography
Chapter 182: Reactive and Postinfectious Arthritis
Keywords
Pathogenesis
Clinical Manifestations and Differential Diagnosis
Diagnosis
Treatment
Complications and Prognosis
Bibliography
Chapter 183: Systemic Lupus Erythematosus
Keywords
Etiology
Epidemiology
Pathology
Pathogenesis
Clinical Manifestations
Diagnosis
Differential Diagnosis
Laboratory Findings
Treatment
Complications
Prognosis
183.1 Neonatal Lupus
Keywords
Bibliography
Bibliography
Chapter 184: Juvenile Dermatomyositis
Keywords
Etiology
Epidemiology
Pathogenesis
Clinical Manifestations
Diagnosis
Differential Diagnosis
Laboratory Findings
Treatment
Complications
Prognosis
Bibliography
Chapter 185: Scleroderma and Raynaud Phenomenon
Keywords
Etiology and Pathogenesis
Classification
Epidemiology
Clinical Manifestations
Localized Scleroderma
Systemic Scleroderma
Raynaud Phenomenon
Diagnosis
Differential Diagnosis
Laboratory Findings
Treatment
Prognosis
Bibliography
Chapter 186: Behçet Disease
Keywords
Epidemiology
Etiology and Pathogenesis
Clinical Manifestations and Diagnosis
Treatment and Prognosis
Bibliography
Chapter 187: Sjögren Syndrome
Keywords
Epidemiology
Etiology and Pathogenesis
Clinical Manifestations
Diagnosis
Differential Diagnosis
Treatment
Complications and Prognosis
Bibliography
Chapter 188: Hereditary Periodic Fever Syndromes and Other Systemic Autoinflammatory Diseases
Keywords
Classification of Autoinflammatory Disorders
Autoinflammatory Diseases With Periodic or Prominent Fevers
Familial Mediterranean Fever
Hyperimmunoglobulinemia D With Periodic Fever Syndrome
Tumor Necrosis Factor Receptor–Associated Periodic Syndrome
Cryopyrin-Associated Periodic Fever Syndromes
Other Mendelian Autoinflammatory Diseases
Syndrome of Pyogenic Arthritis With Pyoderma Gangrenosum and Acne
Deficiency of Interleukin-1 Receptor Antagonist
Blau Syndrome
Autoinflammation With Phospholipase Cγ2–Associated Antibody Deficiency and Immune Dysregulation
Deficiency of Adenosine Deaminase 2
Sideroblastic Anemia With Immunodeficiency, Fevers, and Development Delay
Deficiency of Interleukin-36 Receptor Antagonist (DITRA)
Familial Cold Autoinflammatory Syndrome Type 2
Autoinflammation With Enterocolitis
Majeed Syndrome
Interferonopathies
Chronic Atypical Neutrophilic Dermatosis With Lipodystrophy and Elevated Temperature
STING-Associated Vasculopathy With Onset in Infancy
Genetically Complex Autoinflammatory Diseases
Periodic Fever, Aphthous Stomatitis, Pharyngitis, and Adenitis
Chronic Recurrent Multifocal Osteomyelitis
Bibliography
Chapter 189: Amyloidosis
Keywords
Etiology
Epidemiology
Pathogenesis
Clinical Manifestations
Diagnosis
Laboratory Findings
Treatment
Prognosis
Prevention
Bibliography
Chapter 190: Sarcoidosis
Keywords
Etiology
Epidemiology
Pathology and Pathogenesis
Clinical Manifestations
Laboratory Findings
Diagnosis
Differential Diagnosis
Treatment
Prognosis
Bibliography
Chapter 191: Kawasaki Disease
Keywords
Etiology
Epidemiology
Pathology
Clinical Manifestations
Laboratory and Radiology Findings
Diagnosis
Differential Diagnosis
Treatment
Complications
Prognosis
Bibliography
Chapter 192: Vasculitis Syndromes
Keywords
192.1 Henoch-Schönlein Purpura
Epidemiology
Pathology
Pathogenesis
Clinical Manifestations
Diagnosis
Laboratory Findings
Treatment
Complications
Prognosis
Bibliography
192.2 Takayasu Arteritis
Epidemiology
Pathology
Pathogenesis
Clinical Manifestations
Diagnosis
Differential Diagnosis
Laboratory Findings
Treatment
Complications
Prognosis
Bibliography
192.3 Polyarteritis Nodosa and Cutaneous Polyarteritis Nodosa
Epidemiology
Pathology
Pathogenesis
Clinical Manifestations
Diagnosis
Differential Diagnosis
Laboratory Findings
Treatment
Complications
Prognosis
Bibliography
192.4 Antineutrophilic Cytoplasmic Antibody–Associated Vasculitis
Epidemiology
Pathology
Pathogenesis
Clinical Manifestations
Diagnosis
Differential Diagnosis
Laboratory Findings
Treatment
Complications
Prognosis
Bibliography
192.5 Other Vasculitis Syndromes
Bibliography
Bibliography
Chapter 193: Musculoskeletal Pain Syndromes
Keywords
Clinical Manifestations
Diagnosis and Differential Diagnosis
Treatment
Complications and Prognosis
193.1 Growing Pains
Bibliography
193.2 Small Fiber Polyneuropathy
Bibliography
193.3 Fibromyalgia
Bibliography
193.4 Complex Regional Pain Syndrome
Bibliography
193.5 Erythromelalgia
Bibliography
Bibliography
Chapter 194: Miscellaneous Conditions Associated With Arthritis
Keywords
Relapsing Polychondritis
Mucha-Habermann Disease/Pityriasis Lichenoides Et Varioliformis Acuta
Sweet Syndrome
Hypertrophic Osteoarthropathy
Plant Thorn Synovitis
Pigmented Villonodular Synovitis
Bibliography
Part XVI: Infectious Diseases
Section 1: General Considerations
Chapter 195: Diagnostic Microbiology
Keywords
Specimen Collection
Laboratory Diagnosis of Bacterial and Fungal Infections
Microscopy
Isolation and Identification
Blood Culture
Cerebrospinal Fluid Culture
Urine Culture
Genital Culture
Throat and Respiratory Culture
Detection of Enteric Pathogens
Culture of Other Fluids and Tissues
Screening/Surveillance Cultures
Antimicrobial Susceptibility Testing
Fungal Cultures
Point-of-Care Diagnostics
Laboratory Detection of Parasitic Infections
Serologic Diagnosis
Laboratory Diagnosis of Viral Infections
Specimens
Antigen Detection Tests
Viral Culture
Molecular Diagnostics
Bibliography
Chapter 196: The Microbiome and Pediatric Health
Keywords
Measuring the Microbiome
Early Childhood Development of the Microbiome
The Microbiome and Physiologic Development
Microbiome and Metabolism
Microbiome, Inflammation, and Immunity
Microbiome-Neurobiologic Connections
Contributions of Microbiome to Disease
Microbiome of Premature Birth
Changes in the Microbiome With Necrotizing Enterocolitis
Microbiome and Allergic Disorders
Airway Microbiome of Cystic Fibrosis
Microbiome During Antibiotic-Associated Diarrhea and Clostridium difficile Colitis
Microbiome and Association With Inflammatory Bowel Disease
Microbiome of Obesity
Microbiome During Malnutrition
Therapeutic Manipulation of the Microbiome
Prebiotics
Probiotics
Bibliography
Section 2: Preventive Measures
Chapter 197: Immunization Practices
Keywords
Passive Immunity
Intramuscular Immunoglobulin
Intravenous Immunoglobulin
Subcutaneous Immunoglobulin
Hyperimmune Animal Antisera Preparations
Monoclonal Antibodies
Active Immunization
Vaccination System in the United States
Vaccine Production
Vaccine Policy
Vaccine Financing
Vaccine Safety Monitoring
Vaccine Delivery
Recommended Immunization Schedule
Vaccines Recommended in Special Situations
Precautions and Contraindications
Medical Exemptions
Improving Immunization Coverage
Vaccine Hesitancy
197.1 International Immunization Practices
Bibliography
Bibliography
Chapter 198: Infection Prevention and Control
Keywords
Hand Hygiene
Standard Precautions
Isolation
Additional Measures
Surgical Prophylaxis
Employee Health
Bibliography
Chapter 199: Childcare and Communicable Diseases
Keywords
Epidemiology
Respiratory Tract Infections
Gastrointestinal Tract Infections
Skin Diseases
Invasive Organisms
Herpesviruses
Bloodborne Pathogens
Antibiotic Use and Bacterial Resistance
Prevention
Standards
Bibliography
Chapter 200: Health Advice for Children Traveling Internationally
Keywords
The Pediatric Travel Medicine Consultation
Pediatric Travelers Visiting Friends and Relatives
Safety and Preventive Counseling Topics
Health and Evacuation Insurance, Underlying Health Conditions, and Medications
Safety and Injury Prevention
Animal Contact
Routine Childhood Vaccinations Required for Pediatric Travel
Diphtheria-Tetanus-Pertussis
Haemophilus influenzae Type b
Hepatitis A
Hepatitis B
Influenza and Avian Influenza
Measles-Mumps-Rubella
Pneumococcal Vaccines
Polio Vaccine
Varicella
Specialized Pediatric Travel Vaccinations
Cholera
Hepatitis A Vaccination and Preexposure Immunoglobulin
Japanese Encephalitis
Meningococcal Vaccines
Rabies
Tuberculosis
Typhoid
Yellow Fever
Traveler’s Diarrhea
Guidance on Prevention of Traveler’s Diarrhea
Management of Traveler’s Diarrhea
Presumptive Antibiotic Treatment
Insect-Borne Infections
Malaria Chemoprophylaxis
The Returning Traveler
The Adolescent Traveler
Bibliography
Chapter 201: Fever
Keywords
Pathogenesis
Etiology
Clinical Features
Evaluation
Management
Bibliography
Chapter 202: Fever Without a Focus in the Neonate and Young Infant
Keywords
Etiology and Epidemiology
Clinical Manifestations
Diagnosis
Viral Respiratory Illness
Urinary Tract Infection and Bacterial Meningitis
Laboratory Diagnosis
Complete Blood Count
Blood Culture
Urinalysis
Cerebrospinal Fluid
Herpes Simplex Virus Testing
Enterovirus Testing
Other Diagnostic Studies
Treatment
Antimicrobials
Discharge From the Hospital
Prognosis
Bibliography
Chapter 203: Fever in the Older Child
Keywords
Diagnosis
Viral Infections
Bacterial Infections
Occult Urinary Tract Infection
Occult Bacteremia
General Approach
Overall Appearance and Vital Signs
Symptoms
Physical Examination
Evaluation
Laboratory Testing
Imaging
Management
General Management Principles
Other Considerations
Bibliography
Chapter 204: Fever of Unknown Origin
Keywords
Etiology
Diagnosis
History
Physical Examination
Laboratory Evaluation
Management
Prognosis
Bibliography
Chapter 205: Infections in Immunocompromised Persons
Keywords
205.1 Infections Occurring With Primary Immunodeficiencies
Abnormalities of the Phagocytic System
Defective Splenic Function, Opsonization, or Complement Activity
B Cell Defects (Humoral Immunodeficiencies)
T Cell Defects (Cell-Mediated Immunodeficiencies)
Combined B Cell and T Cell Defects
Bibliography
205.2 Infections Occurring With Acquired Immunodeficiencies
Acquired Immunodeficiency From Infectious Agents
Malignancies
Fever and Neutropenia
Fever Without Neutropenia
Transplantation
Stem Cell Transplantation
Pretransplantation Period
Preengraftment Period
Postengraftment Period
Late Posttransplantation Period
Solid-Organ Transplantation
Timing
Bacterial and Fungal Infections
Viral Infections
Opportunistic Pathogens
Bibliography
205.3 Prevention of Infection in Immunocompromised Persons
Bibliography
Chapter 206: Infection Associated With Medical Devices
Keywords
Intravascular Access Devices
Catheter Types
Catheter-Associated Skin and Soft Tissue Infection
Catheter-Related Bloodstream Infection
Prevention of Infection
Cerebrospinal Fluid Shunts
Prevention of Infection
Urinary Catheters
Prevention of Infection
Peritoneal Dialysis Catheters
Prevention of Infection
Orthopedic Prostheses
Bibliography
Section 3: Antibiotic Therapy
Chapter 207: Principles of Antibacterial Therapy
Keywords
Age- and Risk-Specific Use of Antibiotics in Children
Neonates
Older Children
Immunocompromised and Hospitalized Patients
Infections Associated With Medical Devices
Antibiotics Commonly Used in Pediatric Practice
Penicillins
Cephalosporins
Carbapenems
Glycopeptides
Aminoglycosides
Tetracyclines
Sulfonamides
Macrolides
Lincosamides
Quinolones
Streptogramins and Oxazolidinones
Daptomycin
Miscellaneous Agents
Bibliography
Section 4: Gram-Positive Bacterial Infections
Chapter 208: Staphylococcus
Keywords
208.1 Staphylococcus aureus
Etiology
Epidemiology
Pathogenesis
Clinical Manifestations
Newborn
Skin
Respiratory Tract
Sepsis
Muscle
Bones and Joints
Central Nervous System
Heart
Kidney
Toxic Shock Syndrome
Intestinal Tract
Diagnosis
Differential Diagnosis
Treatment
Prognosis
Prevention
Bibliography
208.2 Toxic Shock Syndrome
Etiology
Epidemiology
Pathogenesis
Clinical Manifestations
Diagnosis
Differential Diagnosis
Treatment
Prevention
Bibliography
208.3 Coagulase-Negative Staphylococci
Epidemiology
Pathogenesis
Clinical Manifestations
Bacteremia
Endocarditis
Central Venous Catheter Infection
Cerebrospinal Fluid Shunts
Urinary Tract Infection
Diagnosis
Treatment
Prognosis
Prevention
Bibliography
Chapter 209: Streptococcus pneumoniae (Pneumococcus)
Keywords
Etiology
Epidemiology
Pathogenesis
Clinical Manifestations
Diagnosis
Treatment
Prognosis
Prevention
Bibliography
Chapter 210: Group A Streptococcus
Keywords
Etiology
Epidemiology
Pathogenesis
Clinical Manifestations
Respiratory Tract Infections
Scarlet Fever
Impetigo
Erysipelas
Perianal Dermatitis
Vaginitis
Severe Invasive Disease
Diagnosis
Differential Diagnosis
Treatment
Complications
Poststreptococcal Reactive Arthritis
Pediatric Autoimmune Neuropsychiatric Disorders Associated with Streptococcus pyogenes
Prognosis
Prevention
210.1 Rheumatic Fever
Keywords
Etiology
Epidemiology
Pathogenesis
Clinical Manifestations and Diagnosis
The 5 Major Criteria
Migratory Polyarthritis
Carditis
Chorea
Erythema Marginatum
Subcutaneous Nodules
Minor Criteria
Recent Group A Streptococcus Infection
Differential Diagnosis
Treatment
Antibiotic Therapy
Antiinflammatory Therapy
Sydenham Chorea
Complications
Prognosis
Prevention
Primary Prevention
Secondary Prevention
Bibliography
Bibliography
Chapter 211: Group B Streptococcus
Keywords
Etiology
Epidemiology
Pathogenesis
Clinical Manifestations
Diagnosis
Laboratory Findings
Treatment
Prognosis
Prevention
Chemoprophylaxis
Maternal Immunization
Bibliography
Chapter 212: Non–Group A or B Streptococci
Keywords
Bibliography
Chapter 213: Enterococcus
Keywords
Etiology
Epidemiology
Pathogenesis
Antimicrobial Resistance
Clinical Manifestations
Neonatal Infections
Infections in Older Children
Treatment
Vancomycin-Resistant Enterococci
Prevention
Bibliography
Chapter 214: Diphtheria (Corynebacterium diphtheriae)
Keywords
Etiology
Epidemiology
Pathogenesis
Clinical Manifestations
Respiratory Tract Diphtheria
Cutaneous Diphtheria
Infection at Other Sites
Diagnosis
Complications
Toxic Cardiomyopathy
Toxic Neuropathy
Treatment
Supportive Care
Prognosis
Prevention
Asymptomatic Case Contacts
Asymptomatic Carriers
Vaccine
Bibliography
Websites
Chapter 215: Listeria monocytogenes
Keywords
Etiology
Epidemiology
Pathology
Pathogenesis
Clinical Manifestations
Listeriosis in Pregnancy
Neonatal Listeriosis
Postneonatal Infections
Diagnosis
Differential Diagnosis
Treatment
Prognosis
Prevention
Bibliography
Chapter 216: Actinomyces
Keywords
Etiology and Epidemiology
Pathogenesis
Diagnosis
Clinical Manifestations
Cervicofacial Actinomycosis
Abdominal and Pelvic Actinomycosis
Thoracic Actinomycosis
Cerebral and Other Forms of Actinomycosis
Differential Diagnosis
Treatment
Prognosis
Bibliography
Chapter 217: Nocardia
Keywords
Etiology
Epidemiology
Pathogenesis
Clinical and Radiographic Manifestations
Diagnosis
Treatment
Prognosis
Bibliography
Section 5: Gram-Negative Bacterial Infections
Chapter 218: Neisseria meningitidis (Meningococcus)
Keywords
Etiology
Epidemiology
Pathogenesis and Pathophysiology
Immunity
Host Factors
Clinical Manifestations
Diagnosis
Differential Diagnosis
Treatment
Antibiotics
Supportive Care
Complications
Prognosis
Prevention
Secondary Prevention
Vaccination
Bibliography
Chapter 219: Neisseria gonorrhoeae (Gonococcus)
Keywords
Etiology
Epidemiology
Pathogenesis and Pathology
Clinical Manifestations
Asymptomatic Gonorrhea
Uncomplicated, Localized Gonorrhea
Disseminated Gonococcal Infection
Diagnosis
Gram Stain and Culture
Nucleic Acid Amplification Tests
Treatment
Complications
Prevention
Bibliography
Chapter 220: Kingella kingae
Keywords
Etiology
Epidemiology
Pathogenesis
Clinical Disease
Septic Arthritis
Osteomyelitis
Spondylodiscitis
Occult Bacteremia
Endocarditis
Diagnosis
Treatment
Prevention
Bibliography
Chapter 221: Haemophilus influenzae
Keywords
Etiology
Epidemiology
Pathogenesis
Antibiotic Resistance
Immunity
Diagnosis
Clinical Manifestations and Treatment
Meningitis
Cellulitis
Preseptal Cellulitis
Orbital Cellulitis
Supraglottitis or Acute Epiglottitis
Pneumonia
Suppurative Arthritis
Pericarditis
Bacteremia Without an Associated Focus
Miscellaneous Infections
Invasive Disease in Neonates
Otitis Media
Conjunctivitis
Sinusitis
Prevention
Vaccine
Prophylaxis
Bibliography
Chapter 222: Chancroid (Haemophilus ducreyi)
Keywords
Etiology and Epidemiology
Clinical Manifestations
Diagnosis
Treatment
Complications
Bibliography
Chapter 223: Moraxella catarrhalis
Keywords
Etiology
Epidemiology
Pathogenesis of Infection
Clinical Manifestations
Acute Otitis Media
Recurrent Otitis Media and Otitis Media With Effusion
Sinusitis
Bacteremia
Diagnosis
Treatment
Prevention
Bibliography
Chapter 224: Pertussis (Bordetella pertussis and Bordetella parapertussis)
Keywords
Etiology
Epidemiology
Pathogenesis
Clinical Manifestations
Diagnosis
Treatment
Antibiotics
Adjunct Therapies
Isolation
Care of Household and Other Close Contacts
Complications
Prevention
DTaP Vaccines
Tdap Vaccines
Bibliography
Chapter 225: Salmonella
Keywords
225.1 Nontyphoidal Salmonellosis
Etiology
Epidemiology
Pathogenesis
Clinical Manifestations
Acute Enteritis
Bacteremia
Nontyphoidal Salmonella Bacteremia as Emerging Disease in Africa
Nontyphoidal Salmonella Bacteremia in Other Geographic Regions
Extraintestinal Focal Infections
Chronic Salmonella Carriage
Complications
Diagnosis
Treatment
Prognosis
Prevention
Bibliography
225.2 Enteric Fever (Typhoid Fever)
Etiology
Epidemiology
Pathogenesis
Clinical Manifestations
Complications
Diagnosis
Differential Diagnosis
Treatment
Prognosis
Prevention
Bibliography
Chapter 226: Shigella
Keywords
Etiology
Epidemiology
Pathogenesis
Immunity
Clinical Manifestations and Complications
Differential Diagnosis
Diagnosis
Treatment
Prevention
Bibliography
Chapter 227: Escherichia coli
Keywords
Enterotoxigenic Escherichia coli
Enteroinvasive Escherichia coli
Enteropathogenic Escherichia coli
Shiga Toxin–Producing Escherichia coli
Enteroaggregative Escherichia coli
Diffusely Adherent Escherichia coli
Enteroaggregative Hemorrhagic Escherichia coli
Diagnosis
Treatment
Prevention of Illness
Bibliography
Chapter 228: Cholera
Keywords
Etiology
Epidemiology
Pathogenesis
Clinical Manifestations
Laboratory Findings
Diagnosis and Differential Diagnosis
Complications
Treatment
Prevention
Bibliography
Chapter 229: Campylobacter
Keywords
Etiology
Epidemiology
Pathogenesis
Clinical Manifestations
Acute Gastroenteritis
Bacteremia
Focal Extraintestinal Infections
Perinatal Infections
Diagnosis
Complications
Reactive Arthritis
Guillain-Barré Syndrome
Other Complications
Treatment
Prognosis
Prevention
Bibliography
Chapter 230: Yersinia
230.1 Yersinia enterocolitica
Keywords
Etiology
Epidemiology
Pathogenesis
Clinical Manifestations
Diagnosis
Differential Diagnosis
Treatment
Complications
Prevention
Bibliography
230.2 Yersinia pseudotuberculosis
Keywords
Etiology
Epidemiology
Pathogenesis
Clinical Manifestations
Diagnosis
Differential Diagnosis
Treatment
Complications
Prevention
Bibliography
230.3 Plague (Yersinia pestis)
Keywords
Etiology
Epidemiology
Pathogenesis
Clinical Manifestations
Diagnosis
Differential Diagnosis
Treatment
Prevention
Bibliography
Chapter 231: Aeromonas and Plesiomonas
Keywords
231.1 Aeromonas
Etiology
Epidemiology
Pathogenesis
Clinical Manifestations
Enteritis
Skin and Soft Tissue Infections
Septicemia
Other Infections
Diagnosis
Treatment
Prevention
Bibliography
231.2 Plesiomonas shigelloides
Etiology
Epidemiology
Pathogenesis
Clinical Manifestations
Diagnosis
Treatment
Bibliography
Chapter 232: Pseudomonas, Burkholderia, and Stenotrophomonas
232.1 Pseudomonas aeruginosa
Keywords
Etiology
Epidemiology
Pathology
Pathogenesis
Clinical Manifestations
Burns and Wound Infection
Cystic Fibrosis
Immunocompromised Persons
Nosocomial Pneumonia
Infants
Diagnosis
Treatment
Supportive Care
Prognosis
Prevention
Bibliography
232.2 Burkholderia cepacia Complex
Keywords
Burkholderia mallei (Glanders)
Burkholderia pseudomallei (Melioidosis)
Bibliography
Burkholderia cepacia Complex
Burkholderia mallei and B. pseudomallei
232.3 Stenotrophomonas
Keywords
Bibliography
Chapter 233: Tularemia (Francisella tularensis)
Keywords
Etiology
Epidemiology
Pathogenesis
Clinical Manifestations
Diagnosis
Differential Diagnosis
Treatment
Prognosis
Prevention
Bibliography
Chapter 234: Brucella
Keywords
Etiology
Epidemiology
Pathogenesis
Clinical Manifestations
Diagnosis
Differential Diagnosis
Treatment
Prognosis
Prevention
Bibliography
Chapter 235: Legionella
Keywords
Etiology
Epidemiology
Pathogenesis
Clinical Manifestations
Pontiac Fever
Diagnosis
Treatment
Prognosis
Bibliography
Chapter 236: Bartonella
236.1 Cat-Scratch Disease (Bartonella henselae)
Keywords
Etiology
Epidemiology
Pathogenesis
Clinical Manifestations
Diagnosis
Differential Diagnosis
Laboratory Findings
Treatment
Complications
Prognosis
Prevention
Bibliography
236.2 Bartonellosis (Bartonella bacilliformis)
Keywords
Etiology
Epidemiology
Pathogenesis
Clinical Manifestations
Diagnosis
Treatment
Prevention
Bibliography
236.3 Trench Fever (Bartonella quintana)
Keywords
Etiology
Epidemiology
Clinical Manifestations
Diagnosis
Treatment
Bibliography
236.4 Bacillary Angiomatosis and Bacillary Peliosis Hepatis (Bartonella henselae and Bartonella quintana)
Keywords
Bacillary Angiomatosis
Bacillary Peliosis
Bacteremia and Endocarditis
Diagnosis
Treatment
Prevention
Bibliography
Bibliography
Section 6: Anaerobic Bacterial Infections
Chapter 237: Botulism (Clostridium botulinum)
Keywords
Etiology
Epidemiology
Pathogenesis
Clinical Manifestations
Diagnosis
Differential Diagnosis
Treatment
Supportive Care
Complications
Prognosis
Prevention
Bibliography
Chapter 238: Tetanus (Clostridium tetani)
Keywords
Etiology
Epidemiology
Pathogenesis
Clinical Manifestations
Diagnosis
Differential Diagnosis
Treatment
Supportive Care
Complications
Prognosis
Prevention
Wound Management
Bibliography
Chapter 239: Clostridium difficile Infection
Keywords
Etiology
Epidemiology
Pathogenesis
Clinical Manifestations
Diagnosis
Treatment
Prognosis
Prevention
Bibliography
Chapter 240: Other Anaerobic Infections
Keywords
Clinical Manifestations
Bacteremia
Central Nervous System
Upper Respiratory Tract
Lower Respiratory Tract
Intraabdominal Infection
Genital Tract
Skin and Soft Tissue
Other Sites
Diagnosis
Treatment
Common Anaerobic Pathogens
Clostridium
Myonecrosis (Gas Gangrene)
Food Poisoning
Bacteroides and Prevotella
Fusobacterium
Veillonella
Anaerobic Cocci
Bibliography
Section 7: Mycobacterial Infections
Chapter 241: Principles of Antimycobacterial Therapy
Keywords
Agents Used Against Mycobacterium Tuberculosis
Commonly Used Agents
Isoniazid
Rifamycins
Pyrazinamide
Ethambutol
Less Commonly Used Agents
Aminoglycosides
Cycloserine
Ethionamide
Fluoroquinolones
Linezolid
Paraaminosalicylic Acid
Bedaquiline Fumarate
Delamanid
Agents Used Against Mycobacterium Leprae
Dapsone
Clofazimine
Agents Used Against Nontuberculous Mycobacteria
Cefoxitin
Doxycycline
Macrolides
Trimethoprim-Sulfamethoxazole
Bibliography
Chapter 242: Tuberculosis (Mycobacterium tuberculosis)
Keywords
Etiology
Clinical Stages
Epidemiology
Transmission
Pathogenesis
Immunity
Clinical Manifestations
Primary Pulmonary Disease
Progressive Primary Pulmonary Disease
Reactivation Tuberculosis
Pleural Effusion
Pericardial Disease
Lymphohematogenous (Disseminated) Disease
Upper Respiratory Tract Disease
Lymph Node Disease
Central Nervous System Disease
Cutaneous Disease
Bone and Joint Disease
Abdominal and Gastrointestinal Disease
Genitourinary Disease
Pregnancy and the Newborn
Perinatal Disease
Disease in HIV-Infected Children
Diagnostic Tools
Tuberculin Skin Testing (TST)
Interferon-γ Release Assay (IGRA)
Mycobacterial Sampling, Susceptibility and Culture
Nucleic Acid Amplification Tests
Treatment
Drug-Resistant Tuberculosis
Corticosteroids
Supportive Care
Mycobacterium tuberculosis Infection
Prevention
Bacille Calmette-Guérin Vaccination
Prevention of Perinatal Tuberculosis
Bibliography
Chapter 243: Hansen Disease (Mycobacterium leprae)
Keywords
Microbiology
Epidemiology
Pathogenesis
Disease Classification
Clinical Manifestations
Skin Involvement
Nerve Involvement
Other Organ Involvement
Immunologic Reactions
Diagnosis
Treatment
Leprosy Reactions
Long-Term Complications
Prevention
Bibliography
Chapter 244: Nontuberculous Mycobacteria
Keywords
Etiology
Epidemiology
Pathogenesis
Clinical Manifestations
Diagnosis
Treatment
Bibliography
Section 8: Spirochetal Infections
Chapter 245: Syphilis (Treponema pallidum)
Keywords
Etiology
Epidemiology
Clinical Manifestations and Laboratory Findings
Congenital Infection
Diagnosis
Congenital Syphilis
Treatment
Acquired Syphilis
Syphilis in Pregnancy
Congenital Syphilis
Prevention
Congenital Syphilis
Bibliography
Additional Websites
Chapter 246: Nonvenereal Treponemal Infections
Keywords
246.1 Yaws (Treponema pertenue)
Bibliography
246.2 Bejel (Endemic Syphilis; Treponema pallidum endemicum)
Bibliography
246.3 Pinta (Treponema carateum)
Bibliography
Chapter 247: Leptospira
Keywords
Etiology
Epidemiology
Pathology and Pathogenesis
Clinical Manifestations
Anicteric Leptospirosis
Icteric Leptospirosis (Weil Syndrome)
Diagnosis
Treatment
Prevention
Bibliography
Chapter 248: Relapsing Fever (Borrelia)
Keywords
Etiology
Epidemiology
Pathology and Pathogenesis
Clinical Manifestations
Diagnosis
Treatment
Prognosis
Prevention
Bibliography
Chapter 249: Lyme Disease (Borrelia burgdorferi)
Keywords
Etiology
Epidemiology
Transmission
Pathology and Pathogenesis
Clinical Manifestations
Early Localized Disease
Early Disseminated Disease
Late Disease
Congenital Lyme Disease
Laboratory Findings
Diagnosis
Serology
Treatment
Prognosis
Prevention
Bibliography
Section 9: Mycoplasmal Infections
Chapter 250: Mycoplasma pneumoniae
Keywords
The Organism
Epidemiology
Pathogenesis
Clinical Manifestations
Respiratory Tract Disease
Extrapulmonary Disease
Diagnosis
Treatment
Antimicrobial Therapy
Adjunctive Therapy
Prevention
Bibliography
Chapter 251: Genital Mycoplasmas (Mycoplasma hominis, Mycoplasma genitalium, and Ureaplasma urealyticum)
Keywords
Etiology
Epidemiology
Transmission
Pathogenesis
Clinical Manifestations
Intrauterine and Neonatal Infections
Chorioamnionitis and Early Onset Infections
Bronchopulmonary Dysplasia
Central Nervous System Infections
Genitourinary Infections
Nongenital Infections
Diagnosis
Genital Tract Infection
Neonates
Treatment
Adolescents and Adults
Neonates
Bibliography
Section 10: Chlamydial Infections
Chapter 252: Chlamydia pneumoniae
Keywords
Etiology
Epidemiology
Pathogenesis
Clinical Manifestations
Diagnosis
Treatment
Prognosis
Bibliography
Chapter 253: Chlamydia trachomatis
253.1 Trachoma
Keywords
Bibliography
253.2 Genital Tract Infections
Keywords
Epidemiology
Clinical Manifestations
Diagnosis
Treatment
Complications
Prevention
Bibliography
253.3 Conjunctivitis and Pneumonia in Newborns
Keywords
Epidemiology
Inclusion Conjunctivitis
Pneumonia
Infections at Other Sites
Diagnosis
Treatment
Prevention
Bibliography
253.4 Lymphogranuloma Venereum
Keywords
Clinical Manifestations
Diagnosis
Treatment
Bibliography
Chapter 254: Psittacosis (Chlamydia psittaci)
Keywords
Etiology
Epidemiology
Clinical Manifestations
Diagnosis
Treatment
Prognosis
Prevention
Bibliography
Section 11: Rickettsial Infections
Chapter 255: Spotted Fever Group Rickettsioses
Keywords
255.1 Rocky Mountain Spotted Fever (Rickettsia rickettsii)
Etiology
Epidemiology
Transmission
Pathology and Pathogenesis
Clinical Manifestations
Other
Laboratory Findings
Diagnosis
Differential Diagnosis
Treatment
Supportive Care
Complications
Prognosis
Prevention
Bibliography
255.2 Mediterranean Spotted Fever or Boutonneuse Fever (Rickettsia conorii)
Etiology
Epidemiology
Transmission
Pathology and Pathogenesis
Clinical Manifestations and Laboratory Findings
Diagnosis
Differential Diagnosis
Treatment and Supportive Care
Complications
Prevention
Bibliography
255.3 Rickettsialpox (Rickettsia akari) and Flea-Borne Spotted Fever
Bibliography
Bibliography
Chapter 256: Scrub Typhus (Orientia tsutsugamushi)
Keywords
Etiology
Epidemiology
Transmission
Pathology and Pathogenesis
Clinical Manifestations and Laboratory Findings
Diagnosis and Differential Diagnosis
Treatment and Supportive Care
Complications
Prevention
Bibliography
Chapter 257: Typhus Group Rickettsioses
Keywords
257.1 Murine (Endemic or Flea-Borne) Typhus (Rickettsia typhi)
Keywords
Etiology
Epidemiology
Transmission
Pathology and Pathogenesis
Clinical Manifestations
Laboratory Findings
Diagnosis and Differential Diagnosis
Treatment
Supportive Care
Complications
Prevention
Bibliography
257.2 Epidemic (Louse-Borne) Typhus (Rickettsia prowazekii)
Keywords
Etiology
Epidemiology
Transmission
Clinical Manifestations
Treatment
Prevention
Bibliography
Bibliography
Chapter 258: Ehrlichiosis and Anaplasmosis
Keywords
Etiology
Epidemiology
Transmission
Pathology and Pathogenesis
Clinical Manifestations
Laboratory Findings
Diagnosis
Differential Diagnosis
Treatment
Complications and Prognosis
Prevention
Bibliography
Chapter 259: Q Fever (Coxiella burnetii)
Keywords
Etiology
Epidemiology
Transmission
Pathology and Pathogenesis
Clinical Manifestations and Complications
Primary (Acute) Q Fever
Persistent Localized Q Fever Infection
Laboratory Findings
Diagnosis and Differential Diagnosis
Treatment
Prevention
Bibliography
Section 12: Fungal Infections
Chapter 260: Principles of Antifungal Therapy
Keywords
Polyenes
Amphotericin B
Pyrimidine Analogs
5-Fluorocytosine
Azoles
Fluconazole
Itraconazole
Voriconazole
Posaconazole
Isavuconazole
Echinocandins
Caspofungin
Micafungin
Anidulafungin
Bibliography
Chapter 261: Candida
Keywords
261.1 Neonatal Infections
Keywords
Epidemiology
Pathogenesis
Clinical Manifestations
Diagnosis
Prophylaxis
Treatment
Prognosis
Bibliography
261.2 Infections in Immunocompetent Children and Adolescents
Keywords
Oral Candidiasis
Diaper Dermatitis
Ungual and Periungual Infections
Vulvovaginitis
Bibliography
261.3 Infections in Immunocompromised Children and Adolescents
Keywords
Etiology
Clinical Manifestations
HIV-Infected Children
Cancer and Transplant Patients
Catheter-Associated Infections
Diagnosis
Treatment
Primary Immune Defects
Bibliography
Chapter 262: Cryptococcus neoformans and Cryptococcus gattii
Keywords
Etiology
Epidemiology
Pathogenesis
Clinical Manifestations
Meningitis
Pneumonia
Cutaneous Infection
Skeletal Infection
Sepsis Syndrome
Diagnosis
Treatment
Prevention
Bibliography
Chapter 263: Malassezia
Keywords
Bibliography
Chapter 264: Aspergillus
264.1 Allergic Disease (Hypersensitivity Syndromes)
Keywords
Asthma
Extrinsic Alveolar Alveolitis
Allergic Bronchopulmonary Aspergillosis
Allergic Aspergillus Sinusitis
Bibliography
264.2 Saprophytic (Noninvasive) Syndromes
Keywords
Pulmonary Aspergilloma
Chronic Pulmonary Aspergillosis
Sinusitis
Otomycosis
Bibliography
264.3 Invasive Disease
Keywords
Invasive Pulmonary Aspergillosis
Diagnosis
Treatment
Special Populations
Cutaneous Aspergillosis
Invasive Sinonasal Disease
Central Nervous System
Eye
Bone
Heart
Empirical Antifungal Therapy
Bibliography
Chapter 265: Histoplasmosis (Histoplasma capsulatum)
Keywords
Etiology
Epidemiology
Pathogenesis
Clinical Manifestations
Diagnosis
Treatment
Bibliography
Chapter 266: Blastomycosis (Blastomyces dermatitidis and Blastomyces gilchristii)
Keywords
Etiology
Epidemiology
Pathogenesis
Clinical Manifestations
Diagnosis
Treatment
Bibliography
Chapter 267: Coccidioidomycosis (Coccidioides Species)
Keywords
Etiology
Epidemiology
Pathogenesis
Clinical Manifestations
Primary Coccidioidomycosis
Complicated Pulmonary Infection
Residual Pulmonary Coccidioidomycosis
Disseminated (Extrapulmonary) Infection
Diagnosis
Culture, Histopathologic Findings, and Antigen Detection
Serology
Imaging Procedures
Treatment
Primary Pulmonary Infection
Diffuse Pneumonia
Disseminated (Extrapulmonary) Infection
Meningitis
Surgical Management
Prevention
Bibliography
Chapter 268: Paracoccidioides brasiliensis
Keywords
Etiology
Epidemiology
Pathogenesis
Clinical Manifestations
Diagnosis
Treatment
Bibliography
Chapter 269: Sporotrichosis (Sporothrix schenckii)
Keywords
Etiology
Epidemiology
Pathogenesis
Clinical Manifestations
Diagnosis
Treatment
Bibliography
Chapter 270: Mucormycosis
Keywords
Etiology
Epidemiology
Pathogenesis
Clinical Manifestations
Diagnosis
Treatment
Bibliography
Chapter 271: Pneumocystis jirovecii
Keywords
Etiology
Epidemiology
Pathogenesis
Pathology
Clinical Manifestations
Laboratory Findings
Diagnosis
Treatment
Supportive Care
Complications
Prognosis
Prevention
Bibliography
Section 13: Viral Infections
Chapter 272: Principles of Antiviral Therapy
Keywords
Antivirals Used for Herpesviruses
Acyclovir
Valacyclovir
Penciclovir and Famciclovir
Ganciclovir and Valganciclovir
Foscarnet
Cidofovir
Trifluridine
Vidarabine
Fomivirsen
New Agents
Antivirals Used for Respiratory Viral Infections
Ribavirin
Amantadine and Rimantadine
Oseltamivir, Zanamivir, and Peramivir
Baloxavir
Antivirals Used for Hepatitis
Antiviral Immune Globulins
Bibliography
Chapter 273: Measles
Keywords
Etiology
Epidemiology
Transmission
Pathology
Pathogenesis
Clinical Manifestations
Modified Measles Infection
Laboratory Findings
Diagnosis
Differential Diagnosis
Complications
Subacute Sclerosing Panencephalitis
Treatment
Vitamin A
Prognosis
Prevention
Vaccine
Postexposure Prophylaxis
Bibliography
Chapter 274: Rubella
Keywords
Etiology
Epidemiology
Pathology
Pathogenesis
Clinical Manifestations
Laboratory Findings
Diagnoses
Differential Diagnoses
Complications
Congenital Rubella Syndrome
Treatment
Supportive Care
Prognosis
Prevention
Vaccination
Bibliography
Chapter 275: Mumps
Keywords
Etiology
Epidemiology
Pathology and Pathogenesis
Clinical Manifestations
Diagnosis
Differential Diagnosis
Complications
Meningitis and Meningoencephalitis
Orchitis and Oophoritis
Pancreatitis
Cardiac Involvement
Arthritis
Thyroiditis
Treatment
Prognosis
Prevention
Bibliography
Chapter 276: Polioviruses
Keywords
Etiology
Epidemiology
Transmission
Pathogenesis
Clinical Manifestations
Abortive Poliomyelitis
Nonparalytic Poliomyelitis
Paralytic Poliomyelitis
Diagnosis
Differential Diagnosis
Treatment
Abortive Poliomyelitis
Nonparalytic Poliomyelitis
Paralytic Poliomyelitis
Complications
Prognosis
Postpolio Syndrome
Prevention
Bibliography
Chapter 277: Nonpolio Enteroviruses
Keywords
Etiology
Epidemiology
Pathogenesis
Clinical Manifestations
Nonspecific Febrile Illness
Hand-Foot-and-Mouth Disease
Herpangina
Respiratory Manifestations
Ocular Manifestations
Myocarditis and Pericarditis
Gastrointestinal and Genitourinary Manifestations
Neurologic Manifestations
Myositis and Arthritis
Neonatal Infections
Transplant Recipients and Patients With Malignancies
Diagnosis
Differential Diagnosis
Treatment
Complications and Prognosis
Prevention
Bibliography
Chapter 278: Parvoviruses
Keywords
Etiology
Epidemiology
Parvovirus B19
Human Bocaviruses
Pathogenesis
Parvovirus B19
Human Bocaviruses
Clinical Manifestations
Parvovirus B19
Erythema Infectiosum (Fifth Disease)
Arthropathy
Transient Aplastic Crisis
Immunocompromised Persons
Fetal Infection
Myocarditis
Other Cutaneous Manifestations
Human Bocaviruses
Diagnosis
Parvovirus B19 Infection
Human Bocavirus Infections
Differential Diagnosis
Parvovirus B19
Human Bocavirus
Treatment
Parvovirus B19
Human Bocavirus
Complications
Parvovirus B19
Human Bocavirus
Prevention
Parvovirus B19
Human Bocavirus
Bibliography
Chapter 279: Herpes Simplex Virus
Keywords
Etiology
Epidemiology
Pathogenesis
Clinical Manifestations
Acute Oropharyngeal Infections
Herpes Labialis
Cutaneous Infections
Genital Herpes
Ocular Infections
Central Nervous System Infections
Infections in Immunocompromised Persons
Perinatal Infections
Diagnosis
Laboratory Findings
Treatment
Acute Mucocutaneous Infections
Genital Herpes
Ocular Infections
Central Nervous System Infections
Infections in Immunocompromised Persons
Perinatal Infections
Prognosis
Prevention
Bibliography
Chapter 280: Varicella-Zoster Virus
Keywords
Etiology
Epidemiology
Pathogenesis
Clinical Manifestations
Varicella in Unvaccinated Individuals
Varicelliform Rashes in Vaccinated Individuals
Neonatal Varicella
Congenital Varicella Syndrome
Complications
Bacterial Infections
Encephalitis and Cerebellar Ataxia
Pneumonia
Progressive Varicella
Herpes Zoster
Diagnosis
Treatment
Varicella
Herpes Zoster
Prognosis
Prevention
Vaccine
Vaccine-Associated Adverse Events
Postexposure Prophylaxis
Bibliography
Chapter 281: Epstein-Barr Virus
Etiology
Epidemiology
Pathogenesis
Clinical Manifestations
Diagnosis
Differential Diagnosis
Laboratory Diagnosis
Detection of Heterophile Antibodies
Detection of Epstein-Barr Virus–Specific Antibodies
Detection of Viral DNA
Complications
Oncogenesis
Treatment
Prognosis
Prevention
Bibliography
Chapter 282: Cytomegalovirus
The Virus and Its Interaction With the Host
Epidemiology
Mechanisms of Disease Associated With Cytomegalovirus Infections
Clinical Manifestations
Normal Host
Immunocompromised Host
Congenital Infection
Perinatal Infection
Diagnosis
Congenital Infections
Noncongenital Infections
Treatment
Prevention
Passive Immunoprophylaxis
Active Immunoprophylaxis
Counseling
Bibliography
Chapter 283: Roseola (Human Herpesviruses 6 and 7)
Keywords
Etiology
Epidemiology
Pathology/Pathogenesis
Clinical Manifestations
Laboratory Findings
Diagnosis
Differential Diagnosis
Complications
Treatment
Prognosis
Prevention
Bibliography
Chapter 284: Human Herpesvirus 8
Keywords
Etiology
Epidemiology
Pathology and Pathogenesis
Clinical Manifestations
Diagnosis
Treatment
Bibliography
Chapter 285: Influenza Viruses
Keywords
Etiology
Epidemiology
Antigenic Variation
Seasonal Influenza
Pathogenesis
Clinical Manifestations
Complications
Laboratory Findings
Diagnosis and Differential Diagnosis
Treatment
Supportive Care
Prognosis
Prevention
Vaccines
Chemoprophylaxis
Bibliography
Chapter 286: Parainfluenza Viruses
Keywords
Etiology
Epidemiology
Pathogenesis
Clinical Manifestations
Diagnosis and Differential Diagnosis
Treatment
Complications
Prognosis
Prevention
Bibliography
Chapter 287: Respiratory Syncytial Virus
Keywords
Etiology
Epidemiology
Pathogenesis
Clinical Manifestations
Diagnosis
Treatment
Prognosis
Prevention
Passive Immunoprophylaxis
Vaccine
Bibliography
Chapter 288: Human Metapneumovirus
Keywords
Etiology
Etiology
Epidemiology
Pathology
Pathogenesis
Clinical Manifestations
Laboratory Findings
Diagnosis and Differential Diagnosis
Complications
Treatment
Supportive Care
Prognosis
Prevention
Bibliography
Chapter 289: Adenoviruses
Keywords
Etiology
Epidemiology
Pathogenesis
Clinical Manifestations
Acute Respiratory Disease
Ocular Infections
Gastrointestinal Infections
Hemorrhagic Cystitis
Other Complications
Adenoviruses in Immunocompromised Patients
Diagnosis
Complications
Treatment
Prevention
Bibliography
Chapter 290: Rhinoviruses
Keywords
Etiology
Epidemiology
Pathogenesis
Clinical Manifestations
Diagnosis
Complications
Treatment
Prevention
Bibliography
Chapter 291: Coronaviruses
Keywords
Etiology
Epidemiology
Pathogenesis of SARS and MERS
Clinical Manifestations
Respiratory Infections
Nonrespiratory Sequelae
Severe Acute Respiratory Syndrome–Associated Coronavirus
Middle East Respiratory Syndrome Coronavirus
Diagnosis
Treatment and Prevention
Bibliography
Chapter 292: Rotaviruses, Caliciviruses, and Astroviruses
Keywords
Etiology
Epidemiology
Pathogenesis
Clinical Manifestations
Diagnosis
Laboratory Findings
Differential Diagnosis
Treatment
Supportive Treatment
Prognosis
Prevention
Vaccines
Bibliography
Chapter 293: Human Papillomaviruses
Keywords
Etiology
Epidemiology
Pathogenesis
Clinical Manifestations
Skin Lesions
Genital Warts
Squamous Intraepithelial Lesions and Cancers
Laryngeal Papillomatosis
Diagnosis
Differential Diagnosis
Treatment
Complications
Prognosis
Prevention
Bibliography
Chapter 294: Arboviral Infections
Etiology
Diagnosis
Prevention
294.1 Eastern Equine Encephalitis
294.2 Western Equine Encephalitis
294.3 St. Louis Encephalitis
294.4 West Nile Encephalitis
Bibliography
294.5 Powassan Encephalitis
Bibliography
294.6 La Crosse and California Encephalitis
294.7 Colorado Tick Fever
294.8 Chikungunya Fever
Bibliography
294.9 Venezuelan Equine Encephalitis
294.10 Japanese Encephalitis
Bibliography
294.11 Tick-Borne Encephalitis
Bibliography
294.12 Zika Virus
Epidemiology
Clinical Features
Management
Laboratory Diagnosis
Prognosis
Differential Diagnosis
Prevention
Bibliography
Bibliography
Chapter 295: Dengue Fever, Dengue Hemorrhagic Fever, and Severe Dengue
Etiology
Epidemiology
Dengue-Like Diseases
Dengue Hemorrhagic Fever
Pathogenesis
Clinical Manifestations
Dengue Fever
Dengue Hemorrhagic Fever and Dengue Shock Syndrome (DHF/DSS)
Dengue With Warning Signs and Severe Dengue
Diagnosis
Differential Diagnosis
Laboratory Findings
Treatment
Dengue Hemorrhagic Fever and Dengue Shock Syndrome
Complications
Prognosis
Dengue Fever
Dengue Hemorrhagic Fever
Prevention
Bibliography
Chapter 296: Yellow Fever
Etiology
Epidemiology
Pathogenesis
Clinical Manifestations
Diagnosis
Treatment
Complications
Prevention
Bibliography
Chapter 297: Ebola and Other Viral Hemorrhagic Fevers
Etiology
Epidemiology
Crimean-Congo Hemorrhagic Fever
Kyasanur Forest Disease
Omsk Hemorrhagic Fever
Rift Valley Fever
Argentine Hemorrhagic Fever
Bolivian Hemorrhagic Fever
Venezuelan Hemorrhagic Fever
Lassa Fever
Marburg Disease
Ebola Hemorrhagic Fever
Hemorrhagic Fever With Renal Syndrome
Clinical Manifestations
Crimean-Congo Hemorrhagic Fever
Kyasanur Forest Disease and Omsk Hemorrhagic Fever
Rift Valley Fever
Argentine, Venezuelan, and Bolivian Hemorrhagic Fevers and Lassa Fever
Marburg Disease and Ebola Hemorrhagic Fever
Hemorrhagic Fever With Renal Syndrome
Diagnosis
Differential Diagnosis
Treatment
Prevention
Bibliography
Chapter 298: Lymphocytic Choriomeningitis Virus
Keywords
Etiology
Epidemiology
Pathogenesis
Clinical Manifestations
Acquired (Postnatal) Lymphocytic Choriomeningitis Virus Infection
Congenital Lymphocytic Choriomeningitis Virus Infection
Laboratory Findings
Diagnosis and Differential Diagnosis
Complications
Treatment
Supportive Care
Prognosis
Prevention
Bibliography
Chapter 299: Hantavirus Pulmonary Syndrome
Keywords
Etiology
Epidemiology
Pathogenesis
Clinical Manifestations
Diagnosis
Laboratory Findings
Differential Diagnosis
Treatment
Prognosis
Prevention
Bibliography
Chapter 300: Rabies
Keywords
Epidemiology
Transmission
Pathogenesis
Clinical Manifestations
Differential Diagnosis
Diagnosis
Treatment and Prognosis
Prevention
Immunization and Fertility Control of Animal Reservoirs
Postexposure Prophylaxis
Preexposure Prophylaxis
Bibliography
Chapter 301: Polyomaviruses
Keywords
Bibliography
Chapter 302: Human Immunodeficiency Virus and Acquired Immunodeficiency Syndrome
Keywords
Etiology
Epidemiology
Transmission
Pathogenesis
Clinical Manifestations
Infections
Central Nervous System
Respiratory Tract
Cardiovascular System
Gastrointestinal and Hepatobiliary Tract
Renal Disease
Skin Manifestations
Hematologic and Malignant Diseases
Diagnosis
Treatment
Combination Therapy
Adherence
Initiation of Therapy
Dosages
Changing Antiretroviral Therapy
Monitoring Antiretroviral Therapy
Resistance to Antiretroviral Therapy
Supportive Care
Prognosis
Prevention
Bibliography
Chapter 303: Human T-Lymphotropic Viruses (1 and 2)
Keywords
Etiology
Epidemiology and Modes of Transmission
Diagnosis
Clinical Manifestations
Adult T-Cell Leukemia/Lymphoma
Human T-Cell Lymphotropic Virus-1–Associated Myelopathy
Human T-Cell Lymphotropic Virus-2
Prevention
Bibliography
Chapter 304: Transmissible Spongiform Encephalopathies
Keywords
Etiology
Epidemiology
Pathogenesis and Pathology
Clinical Manifestations
Diagnosis
Laboratory Findings
Treatment
Genetic Counseling
Prognosis
Family Support
Prevention
Bibliography
Section 14: Antiparasitic Therapy
Chapter 305: Principles of Antiparasitic Therapy
Keywords
Selected Antiparasitic Drugs for Protozoans
Nitazoxanide (Alinia)
Tinidazole (Tindamax)
Atovaquone/Proguanil (Malarone)
Artemisinin Derivatives (Artemether, Artesunate) and Combination Therapies (Artemether/Lumefantrine or Coartem)
Selected Antiparasitic Drugs for Helminths and Ectoparasites
Albendazole (Albenza)
Ivermectin (Stromectol, Mectizan)
Praziquantel (Biltricide)
Section 15: Protozoan Diseases
Chapter 306: Primary Amebic Meningoencephalitis
Keywords
Etiology
Epidemiology
Pathogenesis
Clinical Manifestations
Diagnosis
Treatment
Bibliography
Chapter 307: Amebiasis
Keywords
Etiology
Epidemiology
Pathogenesis
Clinical Manifestations
Amebic Colitis
Amebic Liver Abscess
Laboratory Findings
Diagnosis and Differential Diagnosis
Complications
Treatment
Prognosis
Prevention
Bibliography
Chapter 308: Giardiasis and Balantidiasis
Keywords
308.1 Giardia duodenalis
Etiology
Epidemiology
Clinical Manifestations
Diagnosis
Treatment
Prognosis
Prevention
Bibliography
308.2 Balantidiasis
Bibliography
Chapter 309: Cryptosporidium, Cystoisospora, Cyclospora, and Microsporidia
Keywords
Cryptosporidium
Etiology
Epidemiology
Clinical Manifestations
Diagnosis
Treatment
Cystoisospora
Cyclospora
Microsporidia
Bibliography
Chapter 310: Trichomoniasis (Trichomonas vaginalis)
Epidemiology
Pathogenesis
Clinical Manifestations
Diagnosis
Complications
Treatment
Prevention
Bibliography
Chapter 311: Leishmaniasis (Leishmania)
Keywords
Etiology
Epidemiology
Pathology
Pathogenesis
Clinical Manifestations
Localized Cutaneous Leishmaniasis
Diffuse Cutaneous Leishmaniasis
Disseminated Leishmaniasis
Mucosal Leishmaniasis
Visceral Leishmaniasis
Laboratory Findings
Differential Diagnosis
Diagnosis
Treatment
Prevention
Bibliography
Chapter 312: African Trypanosomiasis (Sleeping Sickness; Trypanosoma brucei Complex)
Keywords
Etiology
Life Cycle
Epidemiology
Pathogenesis
Clinical Manifestations
Trypanosomal Chancre
Hemolymphatic Stage (Stage 1)
Meningoencephalitic Stage (Stage 2)
Diagnosis
Treatment
Stage 1 Treatment
Stage 2 Treatment
Prevention
Bibliography
Chapter 313: American Trypanosomiasis (Chagas Disease; Trypanosoma cruzi)
Keywords
Etiology
Life Cycle
Epidemiology
Pathogenesis
Acute Disease
Chronic Disease
Clinical Manifestations
Immunocompromised Persons
Diagnosis
Treatment
Prevention
Bibliography
Chapter 314: Malaria (Plasmodium)
Keywords
Etiology
Epidemiology
Pathogenesis
Clinical Manifestations
Diagnosis
Differential Diagnosis
Treatment
Plasmodium Falciparum Malaria
Plasmodium Vivax, P. Ovale, P. Malariae, or P. Knowlesi Malaria
Complications of Plasmodium Falciparum MALARIA
Prevention
Bibliography
Chapter 315: Babesiosis (Babesia)
Keywords
Etiology
Epidemiology
Pathogenesis
Clinical Manifestations
Diagnosis
Treatment
Prognosis
Prevention
Bibliography
Chapter 316: Toxoplasmosis (Toxoplasma gondii)
Keywords
Etiology
Epidemiology
Congenital Toxoplasmosis
Pathogenesis
Congenital Toxoplasmosis
Clinical Manifestations
Acquired Toxoplasmosis
Ocular Toxoplasmosis
Immunocompromised Persons
Congenital Toxoplasmosis
Systemic Signs
Skin
Endocrine Abnormalities
Central Nervous System
Eyes
Ears
Diagnosis
Isolation
Serologic Testing
Acquired Toxoplasmosis
Ocular Toxoplasmosis
Immunocompromised Persons
Congenital Toxoplasmosis
Treatment
Acquired Toxoplasmosis
Ocular Toxoplasmosis
Immunocompromised Persons
Congenital Toxoplasmosis
Pregnant Women With Toxoplasma gondii Infection
Prognosis
Prevention
Acknowledgment
Bibliography
Section 16: Helminthic Diseases
Chapter 317: Ascariasis (Ascaris lumbricoides)
Keywords
Etiology
Epidemiology
Pathogenesis
Clinical Manifestations
Diagnosis
Treatment
Prevention
Bibliography
Chapter 318: Hookworms (Necator americanus and Ancylostoma spp.)
Keywords
Etiology
Epidemiology
Pathogenesis
Clinical Manifestations
Diagnosis
Treatment
Prevention
318.1 Cutaneous Larva Migrans
Keywords
Etiology
Epidemiology
Clinical Manifestations
Diagnosis
Treatment
Bibliography
Bibliography
Chapter 319: Trichuriasis (Trichuris trichiura)
Keywords
Etiology
Epidemiology
Clinical Manifestations
Diagnosis
Treatment
Prevention
Bibliography
Chapter 320: Enterobiasis (Enterobius vermicularis)
Keywords
Etiology
Epidemiology
Pathogenesis
Clinical Manifestations
Diagnosis
Treatment
Prevention
Bibliography
Chapter 321: Strongyloidiasis (Strongyloides stercoralis)
Keywords
Etiology
Epidemiology
Pathogenesis
Clinical Manifestations
Diagnosis
Treatment
Prevention
Bibliography
Chapter 322: Lymphatic Filariasis (Brugia malayi, Brugia timori, and Wuchereria bancrofti)
Keywords
Etiology
Epidemiology
Clinical Manifestations
Tropical Pulmonary Eosinophilia
Diagnosis
Treatment
Bibliography
Chapter 323: Other Tissue Nematodes
Keywords
Onchocerciasis (Onchocerca Volvulus)
Loiasis (Loa Loa)
Infection With Animal Filariae
Angiostrongylus Cantonensis
Angiostrongylus Costaricensis
Dracunculiasis (Dracunculus Medinensis)
Gnathostoma Spinigerum
Bibliography
Onchocerciasis (Onchocerca volvulus)
Loiasis (Loa loa)
Infection With Animal Filariae
Angiostrongylus cantonensis
Angiostrongylus costaricensis and Gnathostoma spinigerum
Dracunculiasis (Dracunculus medinensis)
Chapter 324: Toxocariasis (Visceral and Ocular Larva Migrans)
Keywords
Etiology
Epidemiology
Pathogenesis
Clinical Manifestations
Diagnosis
Treatment
Prevention
Bibliography
Chapter 325: Trichinellosis (Trichinella spiralis)
Keywords
Etiology
Epidemiology
Pathogenesis
Clinical Manifestations
Diagnosis
Treatment
Prevention
Bibliography
Chapter 326: Schistosomiasis (Schistosoma)
Keywords
Etiology
Epidemiology
Pathogenesis
Clinical Manifestations
Diagnosis
Treatment
Prevention
Bibliography
Chapter 327: Flukes (Liver, Lung, and Intestinal)
Keywords
Liver Flukes
Fascioliasis (Fasciola hepatica)
Clonorchiasis (Clonorchis sinensis)
Opisthorchiasis (Opisthorchis spp.)
Lung Flukes
Paragonimiasis (Paragonimus spp.)
Intestinal Flukes
Bibliography
Chapter 328: Adult Tapeworm Infections
Keywords
Etiology
Epidemiology
Pathogenesis
Clinical Manifestations
Diagnosis
Differential Diagnosis
Treatment
Prevention
Diphyllobothriasis (Diphyllobothrium Spp.)
Etiology
Epidemiology
Pathogenesis
Clinical Manifestations
Diagnosis
Differential Diagnosis
Treatment
Prevention
Hymenolepiasis (Hymenolepis)
Dipylidiasis (Dipylidium Caninum)
Bibliography
Chapter 329: Cysticercosis
Keywords
Etiology
Epidemiology
Pathogenesis
Clinical Manifestations
Diagnosis
Differential Diagnosis
Treatment
Prevention
Bibliography
Chapter 330: Echinococcosis (Echinococcus granulosus and Echinococcus multilocularis)
Keywords
Etiology
Epidemiology
Pathogenesis
Clinical Manifestations
Diagnosis
Differential Diagnosis
Treatment
Prognosis
Prevention
Bibliography
Part XVII: The Digestive System
Section 1: Clinical Manifestations of Gastrointestinal Disease
Chapter 331: Normal Digestive Tract Phenomena
Keywords
Bibliography
Chapter 332: Major Symptoms and Signs of Digestive Tract Disorders
Keywords
Dysphagia
Regurgitation
Anorexia
Vomiting
Diarrhea
Constipation
Abdominal Pain
Gastrointestinal Hemorrhage
Abdominal Distention and Abdominal Masses
Bibliography
Section 2: The Oral Cavity
Chapter 333: Development and Developmental Anomalies of the Teeth
Keywords
Development of Teeth
Initiation
Histodifferentiation–Morphodifferentiation
Calcification
Eruption
Anomalies Associated With Tooth Development
Bibliography
Chapter 334: Disorders of the Oral Cavity Associated With Other Conditions
Keywords
Chapter 335: Malocclusion
Keywords
Variations in Growth Patterns
Crossbite
Open and Closed Bites
Dental Crowding
Digit Sucking
Chapter 336: Cleft Lip and Palate
Keywords
Incidence and Epidemiology
Clinical Manifestations
Treatment
Postoperative Management
Sequelae
Velopharyngeal Dysfunction
Clinical Manifestations
Bibliography
Chapter 337: Syndromes With Oral Manifestations
Keywords
Bibliography
Chapter 338: Dental Caries
Keywords
Etiology
Epidemiology
Clinical Manifestations
Complications
Treatment
Prevention
Fluoride
Oral Hygiene
Diet
Dental Sealant
Bibliography
Chapter 339: Periodontal Diseases
Keywords
Gingivitis
Aggressive Periodontitis in Children (Prepubertal Periodontitis)
Aggressive Periodontitis in Adolescents
Cyclosporine- or Phenytoin-Induced Gingival Overgrowth
Acute Pericoronitis
Necrotizing Periodontal Disease (Acute Necrotizing Ulcerative Gingivitis)
Bibliography
Chapter 340: Dental Trauma
Keywords
Injuries to Teeth
Injuries to Periodontal Structures
Concussion
Subluxation
Intrusion
Extrusion
Avulsion
Prevention
Additional Considerations
Bibliography
Chapter 341: Common Lesions of the Oral Soft Tissues
Keywords
Oropharyngeal Candidiasis
Aphthous Ulcers
Herpetic Gingivostomatitis
Recurrent Herpes Labialis
Parulis
Cheilitis
Ankyloglossia
Geographic Tongue
Fissured Tongue
Developmental (Normal) Variations
Bohn Nodules
Dental Lamina Cysts
Epstein Pearls
Fordyce Granules
Bibliography
Chapter 342: Diseases of the Salivary Glands and Jaws
Keywords
Parotitis
Ranula
Mucocele
Congenital Lip Pits
Eruption Cyst
Xerostomia
Salivary Gland Tumors
Histiocytic Disorders
Tumors of the Jaw
Bibliography
Chapter 343: Diagnostic Radiology in Dental Assessment
Keywords
Bibliography
Section 3: The Esophagus
Chapter 344: Embryology, Anatomy, and Function of the Esophagus
Keywords
Embryology
Anatomy
Function
344.1 Common Clinical Manifestations and Diagnostic Aids
Keywords
Diagnostic Aids
Bibliography
Bibliography
Chapter 345: Congenital Anomalies
345.1 Esophageal Atresia and Tracheoesophageal Fistula
Keywords
Presentation
Diagnosis
Management
Outcome
Bibliography
345.2 Laryngotracheoesophageal Clefts
Bibliography
345.3 Congenital Esophageal Stenosis
Keywords
Bibliography
Chapter 346: Obstructing and Motility Disorders of the Esophagus
Keywords
Extrinsic
Intrinsic
Bibliography
Chapter 347: Dysmotility
Keywords
Upper Esophageal and Upper Esophageal Sphincter Dysmotility (Striated Muscle)
Lower Esophageal and Lower Esophageal Sphincter Dysfunction (Smooth Muscle)
Bibliography
Chapter 348: Hiatal Hernia
Keywords
Chapter 349: Gastroesophageal Reflux Disease
Keywords
Pathophysiology
Epidemiology and Natural History
Clinical Manifestations
Diagnosis
Management
349.1 Complications of Gastroesophageal Reflux Disease
Keywords
Esophageal: Esophagitis and Sequelae—Stricture, Barrett Esophagus, Adenocarcinoma
Nutritional
Extraesophageal: Respiratory (“Atypical”) Presentations
Apnea and Stridor
Bibliography
Bibliography
Chapter 350: Eosinophilic Esophagitis, Pill Esophagitis, and Infective Esophagitis
Keywords
Eosinophilic Esophagitis
Infective Esophagitis
Pill Esophagitis
Bibliography
Chapter 351: Esophageal Perforation
Keywords
Bibliography
Chapter 352: Esophageal Varices
Keywords
Bibliography
Chapter 353: Ingestions
Keywords
353.1 Foreign Bodies in the Esophagus
Bibliography
353.2 Caustic Ingestions
Keywords
Bibliography
Section 4: Stomach and Intestines
Chapter 354: Normal Development, Structure, and Function of the Stomach and Intestines
Keywords
Development
Digestion and Absorption
Chapter 355: Pyloric Stenosis and Other Congenital Anomalies of the Stomach
Keywords
355.1 Hypertrophic Pyloric Stenosis
Etiology
Clinical Manifestations
Differential Diagnosis
Treatment
Acknowledgment
Bibliography
355.2 Congenital Gastric Outlet Obstruction
Clinical Manifestations
Diagnosis
Treatment
355.3 Gastric Duplication
Acknowledgment
Bibliography
355.4 Gastric Volvulus
Acknowledgment
Bibliography
355.5 Hypertrophic Gastropathy
Pathogenesis
Clinical Manifestations
Diagnosis and Differential Diagnosis
Treatment
Acknowledgment
Bibliography
Chapter 356: Intestinal Atresia, Stenosis, and Malrotation
Keywords
356.1 Duodenal Obstruction
Clinical Manifestations and Diagnosis
Treatment
Bibliography
356.2 Jejunal and Ileal Atresia and Obstruction
Clinical Manifestation and Diagnosis
Treatment
Bibliography
356.3 Malrotation
Clinical Manifestations
Treatment
Acknowledgment
Bibliography
Chapter 357: Intestinal Duplications, Meckel Diverticulum, and Other Remnants of the Omphalomesenteric Duct
Keywords
357.1 Intestinal Duplication
Clinical Manifestations
357.2 Meckel Diverticulum and Other Remnants of the Omphalomesenteric Duct
Clinical Manifestations
Diagnosis
Bibliography
Chapter 358: Motility Disorders and Hirschsprung Disease
Keywords
358.1 Chronic Intestinal Pseudoobstruction
Clinical Manifestations
Diagnosis
Treatment
Bibliography
358.2 Mitochondrial Neurogastrointestinal Encephalomyopathy
Bibliography
358.3 Encopresis and Functional Constipation
Clinical Manifestations
Diagnosis
Treatment
Bibliography
358.4 Congenital Aganglionic Megacolon (Hirschsprung Disease)
Pathology
Clinical Manifestations
Diagnosis
Treatment
Bibliography
358.5 Intestinal Neuronal Dysplasia
Bibliography
358.6 Superior Mesenteric Artery Syndrome (Wilkie Syndrome, Cast Syndrome, Arteriomesenteric Duodenal Compression Syndrome)
Acknowledgment
Bibliography
Chapter 359: Ileus, Adhesions, Intussusception, and Closed-Loop Obstructions
Keywords
359.1 Ileus
Bibliography
359.2 Adhesions
Acknowledgment
Bibliography
359.3 Intussusception
Etiology and Epidemiology
Pathology
Clinical Manifestations
Diagnosis
Differential Diagnosis
Treatment
Prognosis
Bibliography
359.4 Closed-Loop Obstructions
Bibliography
Chapter 360: Foreign Bodies and Bezoars
Keywords
360.1 Foreign Bodies in the Stomach and Intestine
Bibliography
360.2 Bezoars
Bibliography
Chapter 361: Peptic Ulcer Disease in Children
Keywords
Pathogenesis
Acid Secretion
Mucosal Defense
Clinical Manifestations
Diagnosis
Primary Ulcers
Helicobacter pylori Gastritis
Idiopathic Ulcers
Secondary Ulcers
Aspirin and Other Nonsteroidal Antiinflammatory Drugs
“Stress” Ulceration
Treatment
Treatment of Helicobacter pylori–Related Peptic Ulcer Disease
Surgical Therapy
361.1 Zollinger-Ellison Syndrome
Bibliography
Bibliography
Chapter 362: Inflammatory Bowel Disease
Keywords
362.1 Chronic Ulcerative Colitis
Keywords
Clinical Manifestations
Differential Diagnosis
Diagnosis
Treatment
Medical
Surgical
Support
Prognosis
Bibliography
362.2 Crohn Disease (Regional Enteritis, Regional Ileitis, Granulomatous Colitis)
Keywords
Clinical Manifestations
Differential Diagnosis
Diagnosis
Treatment
Medical
5-Aminosalicylates
Antibiotics/Probiotics
Corticosteroids
Immunomodulators
Biologic Therapy
Enteral Nutritional Therapy
Surgery
Support
Prognosis
Bibliography
362.3 Very Early Onset Inflammatory Bowel Disease
Keywords
Bibliography
Chapter 363: Eosinophilic Gastroenteritis
Keywords
Bibliography
Chapter 364: Disorders of Malabsorption
Keywords
Clinical Approach
364.1 Evaluation of Children With Suspected Intestinal Malabsorption
Keywords
Investigations for Carbohydrate Malabsorption
Investigations for Fat Malabsorption
Investigations for Protein-Losing Enteropathy
Investigations for Exocrine Pancreatic Function
Investigations for Intestinal Mucosal Disorders
Imaging Procedures
Bibliography
364.2 Celiac Disease
Keywords
Etiology and Epidemiology
Genetics and Pathogenesis
Clinical Presentation and Associated Disorders
Diagnosis
Treatment
The Spectrum of Gluten-Related Disorders
Bibliography
364.3 Other Malabsorptive Syndromes
Keywords
Defects of Enterocyte Differentiation and Polarization
Microvillus Inclusion Disease (Congenital Microvillus Atrophy)
Tufting Enteropathy (Congenital Tufting Enteropathy)
Tricho-Hepato-Enteric Syndrome (Syndromic Diarrhea)
Defects in Enteroendocrine Cells Differentiation
Enteric Anendocrinosis
Proprotein Convertase 1/3 Deficiency
Mitchell-Riley Syndrome
Aristaless-Related Homeobox Gene Mutations
Autoimmune Enteropathy
Autoimmune Polyglandular Syndrome Type 1
Defects in Lipids Transport and Metabolism
Abetalipoproteinemia
Homozygous Hypobetalipoproteinemia
Chylomicron Retention Disease (Anderson Disease)
DGAT1 Mutation
Wolman Disease
Tangier Disease
Sitosterolemia
Bile Acid Malabsorption
Protein-Losing Enteropathy
Bibliography
364.4 Intestinal Infections and Infestations Associated With Malabsorption
Keywords
Postinfectious Diarrhea
Proximal Intestinal Bacterial Overgrowth
Environmental Enteropathy (Tropical Sprue)
Whipple Disease
Bibliography
364.5 Immunodeficiency Disorders
Keywords
Bibliography
364.6 Immunoproliferative Small Intestinal Disease
Keywords
Bibliography
364.7 Short Bowel Syndrome
Keywords
Treatment
Complications
Bibliography
364.8 Chronic Malnutrition
Keywords
Bibliography
364.9 Enzyme Deficiencies
Keywords
Carbohydrate Malabsorption
Lactase Deficiency
Fructose Malabsorption
Sucrase-Isomaltase Deficiency
Glucose-Galactose Malabsorption
Exocrine Pancreatic Insufficiency
Enterokinase (Enteropeptidase) Deficiency
Trehalase Deficiency
Trypsinogen Deficiency
Bibliography
364.10 Liver and Biliary Disorders Causing Malabsorption
Keywords
364.11 Rare Inborn Defects Causing Malabsorption
Keywords
Disorders of Carbohydrate Absorption
Disorders of Amino Acid and Peptide Absorption
Disorders of Fat Transport
Disorders of Vitamin Absorption
Disorders of Electrolyte and Mineral Absorption
Bibliography
Chapter 365: Intestinal Transplantation in Children With Intestinal Failure
Keywords
Indications for Intestinal Transplant
Paucity of Venous Access
Life-Threatening Infections
Liver Disease
Transplantation Operation
Donor Selection
Types of Intestinal Grafts
The Recipient Operation
Postoperative Management
Immunosuppression
Allograft Assessment
Rejection and Graft-Versus-Host Disease
Infections
Outcomes
Bibliography
Chapter 366: Acute Gastroenteritis in Children
Keywords
Burden of Childhood Diarrhea
Pathogens
Epidemiology in the United States and Other Middle- and High-Income Countries
Epidemiology in Low- and Middle-Income Countries
Host Risk Factors
Pathogenesis of Infectious Diarrhea
Clinical Manifestation of Diarrhea
Intestinal and Extraintestinal Complications
Differential Diagnosis
Clinical Evaluation of Diarrhea
Laboratory Diagnosis
Treatment
Hydration
Enteral Feeding and Diet Selection
Zinc Supplementation
Additional Therapies
Antibiotic Therapy
Prevention
Promotion of Exclusive Breastfeeding and Vitamin A
Rotavirus Immunization
Improved Water and Sanitary Facilities and Promotion of Personal and Domestic Hygiene
366.1 Traveler’s Diarrhea
Keywords
Treatment
Prevention
Bibliography
Bibliography
Chapter 367: Chronic Diarrhea
Keywords
Definition of Epidemiology
Pathophysiology
Etiology
Infectious
Inflammatory/Immunologic
Pancreatic Deficiency
Liver and Bile Acids Disorders
Carbohydrate Malabsorption
Protein-Losing Enteropathy
Motility Disorders
Short Bowel Syndrome
Nonspecific Diarrhea, Including Toddler’s Diarrhea
Congenital Diarrheal Disorders
Evaluation of Patients
Investigations
Treatment
367.1 Diarrhea From Neuroendocrine Tumors
KeyWords
Bibliography
Bibliography
Chapter 368: Functional Gastrointestinal Disorders
Keywords
Functional Gastrointestinal Disorders in Children and Adolescents
Functional Abdominal Pain Disorders
Functional Dyspepsia
Pediatric IBS
Abdominal Migraine
Functional Abdominal Pain Not Otherwise Specified
Functional Defecation Disorders
Functional Constipation
Nonretentive Fecal Incontinence
Bibliography
Chapter 369: Cyclic Vomiting Syndrome
Keywords
Bibliography
Chapter 370: Acute Appendicitis
Epidemiology
Pathophysiology
Clinical Features
Physical Examination
Appendicitis Risk Scoring Systems
Laboratory Findings
Imaging Studies
Plain Radiographs
Ultrasound
Computed Tomography
Magnetic Resonance Imaging and White Blood Cell Scans
Diagnosis and Treatment
Differential Diagnosis
Antibiotics
Surgical Intervention
Perforated Appendicitis
Nonoperative Management of Uncomplicated Appendicitis
Recurrent Appendicitis
Interval Appendectomy
Incidental Appendicoliths
Bibliography
Chapter 371: Surgical Conditions of the Anus and Rectum
371.1 Anorectal Malformations
Keywords
Embryology
Associated Anomalies
Manifestations and Diagnosis
Low Lesions
High Lesions
Persistent Cloaca
Rectal Atresia
Approach to the Patient
Operative Repair
Outcome
Bibliography
371.2 Anal Fissure
Keywords
Clinical Manifestations
Treatment
Bibliography
371.3 Perianal Abscess and Fistula
Keywords
Clinical Manifestations
Treatment
Bibliography
371.4 Hemorrhoids
Keywords
Clinical Manifestations
Treatment
Bibliography
371.5 Rectal Mucosal Prolapse
Keywords
Clinical Manifestations
Treatment
Bibliography
371.6 Pilonidal Sinus and Abscess
Keywords
Bibliography
Chapter 372: Tumors of the Digestive Tract
Keywords
Hamartomatous Tumors
Juvenile Polyposis Syndrome
Peutz-Jeghers Syndrome
Phosphatase and Tensin Homolog Hamartoma Tumor Syndromes
Adenomatous Tumors
Adenomatous Polyposis Coli-Associated Polyposis Syndromes
Carcinoma
Other Gastrointestinal Tumors
Lymphoma
Nodular Lymphoid Hyperplasia
Carcinoid Tumor
Leiomyoma
Gastrointestinal Stromal Cell Tumors
Vascular Tumors
Bibliography
Chapter 373: Inguinal Hernias
Embryology and Pathogenesis
Incidence
Clinical Presentation and Diagnosis
Evaluation of Acute Inguinal–Scrotal Swelling
Incarcerated Hernia
Ambiguous Genitalia
Indications for Surgery
Open Inguinal Hernia Repair
Laparoscopic Inguinal Hernia Repair
Contralateral Inguinal Exploration
Direct Inguinal Hernia
Femoral Hernia
Complications
Wound Infection
Recurrent Hernia
Iatrogenic Cryptorchidism (Trapped Testicle)
Incarceration
Injury to the Vas Deferens and Male Fertility
Bibliography
Section 5: Exocrine Pancreas
Chapter 374: Embryology, Anatomy, and Physiology of the Pancreas
Keywords
374.1 Pancreatic Anatomic Abnormalities
Bibliography
374.2 Pancreatic Physiology
Bibliography
Bibliography
Chapter 375: Pancreatic Function Tests
Keywords
Direct Test
Bibliography
Chapter 376: Disorders of the Exocrine Pancreas
Keywords
Disorders Associated With Pancreatic Insufficiency
Cystic Fibrosis (see Chapter 432)
Shwachman-Diamond Syndrome (see Chapter 157)
Pearson Syndrome
Johanson-Blizzard Syndrome
Isolated Enzyme Deficiencies
Other Syndromes Associated With Pancreatic Insufficiency
Bibliography
Chapter 377: Treatment of Pancreatic Insufficiency
Keywords
Bibliography
Chapter 378: Pancreatitis
378.1 Acute Pancreatitis
Keywords
Clinical Manifestations
Diagnosis
Treatment
Prognosis
Bibliography
378.2 Acute Recurrent and Chronic Pancreatitis
Keywords
Bibliography
Chapter 379: Pancreatic Fluid Collections
Keywords
Treatment of Fluid Collections and Necrosis
Bibliography
Chapter 380: Pancreatic Tumors
Keywords
Bibliography
Section 6: The Liver and Biliary System
Chapter 381: Morphogenesis of the Liver and Biliary System
Keywords
Hepatic Ultrastructure
Metabolic Functions of the Liver
Carbohydrate Metabolism
Protein Metabolism
Lipid Metabolism
Biotransformation
Hepatic Excretory Function
Bibliography
Chapter 382: Manifestations of Liver Disease
Keywords
Pathologic Manifestations
Clinical Manifestations
Hepatomegaly
Jaundice (Icterus)
Pruritus
Spider Angiomas
Palmar Erythema
Xanthomas
Portal Hypertension
Ascites
Gastrointestinal Bleeding
Encephalopathy
Endocrine Abnormalities
Renal Dysfunction
Pulmonary Involvement
Recurrent Cholangitis
Miscellaneous Manifestations of Liver Dysfunction
382.1 Evaluation of Patients With Possible Liver Dysfunction
Keywords
Biochemical Tests
Liver Biopsy
Hepatic Imaging Procedures
Diagnostic Approach to Infants With Jaundice
Bibliography
Bibliography
Chapter 383: Cholestasis
Keywords
383.1 Neonatal Cholestasis
Mechanisms
Evaluation
Intrahepatic Cholestasis
Neonatal Hepatitis
Disorders of Transport, Secretion, Conjugation, and Biosynthesis of Bile Acids
Bile Acid-Coenzyme a Ligase Deficiency
Disorders of Embryogenesis
Biliary Atresia
Differentiation of Idiopathic Neonatal Hepatitis From Biliary Atresia
Management of Patients With Suspected Biliary Atresia
Management of Chronic Cholestasis
Prognosis
383.2 Cholestasis in the Older Child
Bibliography
Chapter 384: Metabolic Diseases of the Liver
384.1 Inherited Deficient Conjugation of Bilirubin (Familial Nonhemolytic Unconjugated Hyperbilirubinemia)
Crigler-Najjar Syndrome Type I (Glucuronyl Transferase Deficiency)
Clinical Manifestations
Diagnosis
Treatment
Crigler-Najjar Syndrome Type II (Partial Glucuronyl Transferase Deficiency)
Clinical Manifestations
Diagnosis
Treatment
Inherited Conjugated Hyperbilirubinemia
Dubin-Johnson Syndrome
Rotor Syndrome
Bibliography
384.2 Wilson Disease
Pathogenesis
Clinical Manifestations
Pathology
Diagnosis
Treatment
Prognosis
Bibliography
384.3 Indian Childhood Cirrhosis
Bibliography
384.4 Gestational Alloimmune Liver Disease (Neonatal Iron Storage Disease)
Bibliography
384.5 Miscellaneous Metabolic Diseases of the Liver
α1-Antitrypsin Deficiency
Citrin Deficiency
Bibliography
Chapter 385: Viral Hepatitis
Issues Common to All Forms of Viral Hepatitis
Differential Diagnosis
Pathogenesis
Common Biochemical Profiles in the Acute Infectious Phase
Hepatitis A
Etiology
Epidemiology
Clinical Manifestations
Diagnosis
Complications
Treatment
Prevention
Immunoglobulin
Vaccine
Prognosis
Hepatitis B
Etiology
Epidemiology
Pathogenesis
Clinical Manifestations
Diagnosis
Complications
Treatment
Treatment Strategies
Prevention
Universal Vaccination
Postexposure Prophylaxis
Special Populations
Prognosis
Hepatitis C
Etiology
Epidemiology
Pathogenesis
Clinical Manifestations
Diagnosis
Complications
Treatment
Newer Treatments
Prevention
Prognosis
Hepatitis D
Etiology
Epidemiology
Pathogenesis
Clinical Manifestations
Diagnosis
Treatment
Prevention
Hepatitis E
Etiology
Epidemiology
Pathogenesis
Clinical Manifestations
Diagnosis
Prevention
Approach to Acute or Chronic Hepatitis
Bibliography
Hepatitis A
Hepatitis B
Hepatitis C
Hepatitis E
Chapter 386: Liver Abscess
Keywords
Bibliography
Chapter 387: Liver Disease Associated With Systemic Disorders
Keywords
Inflammatory Bowel Disease
Bacterial Sepsis
Celiac Disease
Cardiac Disease
Cholestasis Associated With Chronic Total Parenteral Nutrition
Cystic Fibrosis
Bone Marrow Transplantation
Hemoglobinopathies
Histiocytic Disorders
387.1 Nonalcoholic Fatty Liver Disease
Bibliography
Bibliography
Chapter 388: Mitochondrial Hepatopathies
Keywords
Epidemiology
Clinical Manifestations
Primary Mitochondrial Hepatopathies
Neonatal Liver Failure
Alpers Syndrome (Alpers-Huttenlocher Syndrome or Alpers Hepatopathic Poliodystrophy)
Mitochondrial DNA Depletion Syndrome
Navajo Neurohepatopathy
Pearson Syndrome
Villous Atrophy Syndrome
GRACILE Syndrome
Mutations in Nuclear Translation and Elongation Factor Genes
Secondary Mitochondrial Hepatopathies
Diagnostic Evaluation
Treatment of Mitochondrial Hepatopathies
Bibliography
Chapter 389: Autoimmune Hepatitis
Keywords
Autoimmune Hepatitis
Chronic Liver Disease
Etiology
T Lymphocytes
Autoimmune Regulator Gene
Pathology
Clinical Manifestations
Laboratory Findings
Diagnosis
Treatment
Prognosis
Bibliography
Chapter 390: Drug- and Toxin-Induced Liver Injury
Keywords
Treatment
Prognosis
Prevention
Bibliography
Chapter 391: Acute Hepatic Failure
Keywords
Etiology
Infection
Autoimmune Hepatitis
Metabolic Diseases
Neoplasm
Gestational Alloimmune Liver Disease
Drug-Induced Liver Injury
Vascular
Idiopathic Acute Liver Failure
Pathology
Pathogenesis
Clinical Manifestations
Laboratory Findings
Treatment
Prognosis
Bibliography
Chapter 392: Cystic Diseases of the Biliary Tract and Liver
Keywords
Choledochal Cysts
Autosomal Recessive Polycystic Kidney Disease
Cystic Dilation of the Intrahepatic Bile Ducts (Caroli Disease/Caroli Syndrome)
Congenital Hepatic Fibrosis
Autosomal Dominant Polycystic Kidney Disease
Autosomal Dominant Polycystic Liver Disease
Bibliography
Chapter 393: Diseases of the Gallbladder
Keywords
Anomalies
Acute Hydrops
Cholecystitis and Cholelithiasis
Biliary Dyskinesia
Bibliography
Chapter 394: Portal Hypertension and Varices
Keywords
Etiology
Pathophysiology
Clinical Manifestations
Diagnosis
Treatment
Prognosis
Bibliography
Chapter 395: Liver Transplantation
Keywords
Indications
Technical Innovations
Immunosuppression
Complications
Outcomes
Bibliography
Section 7: Peritoneum
Chapter 396: Peritoneal Malformations
Keywords
Acknowledgment
Bibliography
Chapter 397: Ascites
Keywords
397.1 Chylous Ascites
Bibliography
Chapter 398: Peritonitis
Keywords
398.1 Acute Primary Peritonitis
Etiology and Epidemiology
Clinical Manifestations
Diagnosis and Treatment
Bibliography
398.2 Acute Secondary Peritonitis
Clinical Manifestations
Treatment
Bibliography
398.3 Acute Secondary Localized Peritonitis (Peritoneal Abscess)
Etiology
Clinical Manifestations
Treatment
Bibliography
Bibliography
Chapter 399: Epigastric Hernia
Clinical Presentation
399.1 Incisional Hernia
Bibliography
Part XVIII: The Respiratory System
Section 1: Development and Function
Chapter 400: Diagnostic Approach to Respiratory Disease
Keywords
History
Physical Examination
Blood Gas Analysis
Transillumination of the Chest
Radiographic Techniques
Chest X-Rays
Upper Airway Film
Sinus and Nasal Images
Chest Computed Tomography and Magnetic Resonance Imaging
Fluoroscopy
Barium Swallow
Pulmonary Arteriography and Aortograms
Ventilation-Perfusion Relation and Radionuclide Lung Scans
Pulmonary Function Testing
Microbiology: Examination of Lung Secretions
The Microbiome (see Chapter 196)
Exercise Testing
Sleep Studies
Airway Visualization and Lung Specimen–Based Diagnostic Tests
Laryngoscopy
Bronchoscopy and Bronchoalveolar Lavage
Thoracoscopy
Thoracentesis
Lung Tap
Lung Biopsy
Sweat Testing
Bibliography
Chapter 401: Chronic or Recurrent Respiratory Symptoms
Keywords
Judging the Seriousness of Chronic Respiratory Complaints
Recurrent or Persistent Cough
Frequently Recurring or Persistent Stridor
Recurrent or Persistent Wheeze
Recurrent and Persistent Lung Infiltrates
401.1 Extrapulmonary Diseases With Pulmonary Manifestations
Evaluation
Bibliography
Bibliography
Chapter 402: Sudden Infant Death Syndrome
Keywords
Epidemiology
Pathology
Environmental Risk Factors
Nonmodifiable Environmental Risk Factors
Modifiable Environmental Risk Factors
Pregnancy-Related Factors
Cigarette Smoking
Drug and Alcohol Use
Infant Sleep Environment
Side-Sleeping: Significant Risk Factor.
Soft Sleep Surfaces and Soft or Loose Bedding.
Overheating.
Bed Sharing.
Infant Feeding Care Practices and Exposures
Breastfeeding is Associated With a Lower Risk of Sudden Infant Death Syndrome.
Sudden Infant Death Syndrome Rates Remain Higher Among American Indians, Alaska Natives, and African Americans.
Genetic Risk Factors
Gene-Environment Interactions
Infant Groups at Increased Risk for Sudden Infant Death Syndrome
Subsequent Siblings of an Infant who Died of Sudden Infant Death Syndrome
Prematurity
Physiologic Studies
Clinical Strategies
Home Monitoring
Reducing the Risk of Sudden Infant Death Syndrome
402.1 Sudden Unexpected Postnatal Collapse
Keywords
Epidemiology
Pathogenesis
Risk Factors
Diagnosis and Differential Diagnosis
Outcome
Treatment
Prevention
Bibliography
Bibliography
Chapter 403: Brief Resolved Unexplained Events and Other Acute Events in Infants
Keywords
Background
Definition
Epidemiology
Initial History
Additional History
Physical Examination
Testing
Management
Bibliography
Section 2: Disorders of the Respiratory Tract
Chapter 404: Congenital Disorders of the Nose
Keywords
Normal Newborn Nose
Physiology
Congenital Disorders
Choanal Atresia
Clinical Manifestations
Diagnosis
Treatment
Congenital Defects of the Nasal Septum
Pyriform Aperture Stenosis
Congenital Midline Nasal Masses
Diagnosis and Treatment
Bibliography
Chapter 405: Acquired Disorders of the Nose
Keywords
405.1 Nasal Foreign Bodies
Keywords
Etiology
Diagnosis
Treatment
Complications
Prevention
Bibliography
405.2 Epistaxis
Keywords
Anatomy
Etiology
Clinical Manifestations
Treatment
Prevention
Bibliography
Chapter 406: Nasal Polyps
Keywords
Etiology
Clinical Manifestations
Diagnosis and Differential Diagnosis
Treatment
Bibliography
Chapter 407: The Common Cold
Keywords
Etiology
Epidemiology
Pathogenesis
Clinical Manifestations
Diagnosis
Laboratory Findings
Treatment
Antiviral Treatment
Supportive Care and Symptomatic Treatment
Fever
Nasal Obstruction
Rhinorrhea
Sore Throat
Cough
Ineffective Treatments
Complications
Prevention
Bibliography
Chapter 408: Sinusitis
Keywords
Etiology
Epidemiology
Pathogenesis
Clinical Manifestations
Diagnosis
Treatment
Complications
Prevention
Bibliography
Chapter 409: Acute Pharyngitis
Keywords
Infectious Etiologies
Viruses
Bacteria Other Than Group A Streptococcus
Group A Streptococcus
Diagnosis
Treatment
Chronic Group a Streptococcus Carriers
Recurrent Pharyngitis
Complications and Prognosis
Prevention
Bibliography
Chapter 410: Retropharyngeal Abscess, Lateral Pharyngeal (Parapharyngeal) Abscess, and Peritonsillar Cellulitis/Abscess
Keywords
Retropharyngeal and Lateral Pharyngeal Abscess
Peritonsillar Cellulitis and/or Abscess
Bibliography
Chapter 411: Tonsils and Adenoids
Keywords
Anatomy
Normal Function
Pathology
Acute Infection
Chronic Infection
Airway Obstruction
Tonsillar Neoplasm
Clinical Manifestations
Acute Infection
Chronic Infection
Airway Obstruction
Tonsillar Neoplasm
Treatment
Medical Management
Tonsillectomy
Adenoidectomy
Tonsillectomy and Adenoidectomy
Complications
Poststreptococcal Glomerulonephritis and Acute Rheumatic Fever
Peritonsillar Infection
Retropharyngeal Space Infection
Parapharyngeal Space Infection
Recurrent or Chronic Pharyngotonsillitis
Chronic Airway Obstruction
Tonsillectomy and Adenoidectomy
Bibliography
Chapter 412: Acute Inflammatory Upper Airway Obstruction (Croup, Epiglottitis, Laryngitis, and Bacterial Tracheitis)
Keywords
412.1 Infectious Upper Airway Obstruction
Etiology and Epidemiology
Clinical Manifestations
Croup (Laryngotracheobronchitis)
Acute Epiglottitis (Supraglottitis)
Acute Infectious Laryngitis
Spasmodic Croup
Differential Diagnosis
Complications
Treatment
Endotracheal/Nasotracheal Intubation and Tracheotomy
Prognosis
Bibliography
Laryngotracheobronchitis
Epiglottitis
412.2 Bacterial Tracheitis
Clinical Manifestations
Diagnosis
Treatment
Complications
Prognosis
Bibliography
Chapter 413: Congenital Anomalies of the Larynx, Trachea, and Bronchi
Keywords
413.1 Laryngomalacia
Keywords
Clinical Manifestations
Diagnosis
Treatment
Bibliography
413.2 Congenital Subglottic Stenosis
Keywords
Clinical Manifestations
Diagnosis
Bibliography
413.3 Vocal Cord Paralysis
Keywords
Diagnosis
Treatment
Bibliography
413.4 Congenital Laryngeal Webs and Atresia
Keywords
Bibliography
413.5 Congenital Subglottic Hemangioma
Keywords
Bibliography
413.6 Laryngoceles and Saccular Cysts
Keywords
Bibliography
413.7 Posterior Laryngeal Cleft and Laryngotracheoesophageal Cleft
Bibliography
413.8 Vascular and Cardiac Anomalies
Keywords
Bibliography
413.9 Tracheal Stenoses, Webs, and Atresia
Bibliography
413.10 Foregut Cysts
Bibliography
413.11 Tracheomalacia and Bronchomalacia
Bibliography
Chapter 414: Foreign Bodies in the Airway
Keywords
Epidemiology and Etiology
Clinical Manifestations
Diagnosis
Treatment
414.1 Laryngeal Foreign Bodies
414.2 Tracheal Foreign Bodies
414.3 Bronchial Foreign Bodies
Bibliography
Chapter 415: Laryngotracheal Stenosis and Subglottic Stenosis
Keywords
415.1 Congenital Subglottic Stenosis
Keywords
415.2 Acquired Laryngotracheal Stenosis
Keywords
Clinical Manifestations
Diagnosis
Treatment
Bibliography
Chapter 416: Bronchomalacia and Tracheomalacia
Keywords
Clinical Manifestations
Diagnosis
Treatment
Prognosis
Bibliography
Chapter 417: Neoplasms of the Larynx, Trachea, and Bronchi
417.1 Vocal Nodules
Keywords
417.2 Recurrent Respiratory Papillomatosis
Keywords
Clinical Manifestations
Treatment
Bibliography
417.3 Congenital Subglottic Hemangioma
Keywords
Clinical Manifestations
Treatment
Bibliography
417.4 Vascular Anomalies
Keywords
Bibliography
417.5 Other Laryngeal Neoplasms
Keywords
417.6 Tracheal Neoplasms
Keywords
Bibliography
417.7 Bronchial Tumors
Keywords
Bibliography
Chapter 418: Wheezing, Bronchiolitis, and Bronchitis
418.1 Wheezing in Infants: Bronchiolitis
Keywords
General Pathophysiology of Wheezing in Infants
Acute Bronchiolitis
Clinical Manifestations
History and Physical Examination
Diagnostic Evaluation
Treatment
Prognosis
Prevention
Bibliography
418.2 Bronchitis
Keywords
Acute Bronchitis
Clinical Manifestations
Differential Diagnosis
Treatment
Chronic Bronchitis
Cigarette Smoking and Air Pollution
Bibliography
Chapter 419: Plastic Bronchitis
Keywords
Epidemiology
Pathogenesis
Clinical Manifestations
Diagnosis
Treatment
Complications and Prognosis
Bibliography
Chapter 420: Emphysema and Overinflation
Keywords
Localized Obstructive Overinflation
Unilateral Hyperlucent Lung
Swyer-James or Macleod Syndrome
Congenital Lobar Emphysema (Congenital Large Hyperlucent Lobe)
Pulmonary Vascular Abnormalities
Generalized Obstructive Overinflation
Pathology
Clinical Manifestations
Diagnosis
Bullous Emphysema
Subcutaneous Emphysema
Bibliography
Chapter 421: α1-Antitrypsin Deficiency and Emphysema
Keywords
Pathogenesis
Clinical Manifestations
Laboratory Findings
Treatment
Supportive Therapy
Bibliography
Chapter 422: Other Distal Airway Diseases
422.1 Bronchiolitis Obliterans
Keywords
Epidemiology
Pathogenesis
Clinical Manifestations and Diagnosis
Treatment
Prognosis
Bibliography
422.2 Follicular Bronchitis
Keywords
Bibliography
422.3 Pulmonary Alveolar Microlithiasis
Keyword
Epidemiology and Etiology
Clinical Manifestations
Diagnosis
Treatment
Prognosis
Bibliography
Chapter 423: Congenital Disorders of the Lung
Keywords
423.1 Pulmonary Agenesis and Aplasia
Etiology and Pathology
Clinical Manifestations and Prognosis
Diagnosis and Treatment
Bibliography
423.2 Pulmonary Hypoplasia
Etiology and Pathology
Clinical Manifestations
Diagnosis and Treatment
Bibliography
423.3 Congenital Cystic Malformation (Congenital Pulmonary Airway Malformation)
Pathology
Etiology
Diagnosis
Clinical Manifestations
Treatment
Bibliography
423.4 Pulmonary Sequestration
Pathophysiology
Clinical Manifestations and Diagnosis
Treatment
Bibliography
423.5 Bronchogenic Cysts
Etiology and Pathology
Clinical Manifestations and Treatment
Bibliography
423.6 Congenital Pulmonary Lymphangiectasia
Etiology and Pathology
Clinical Manifestations and Treatment
Bibliography
423.7 Lung Hernia
Etiology and Pathology
Clinical Manifestations and Treatment
Bibliography
423.8 Other Congenital Malformations of the Lung
Congenital Lobar Emphysema and Pulmonary Cysts
Pulmonary Arteriovenous Malformation
Bronchobiliary Fistula
Bibliography
Chapter 424: Pulmonary Edema
Keywords
Pathophysiology
Etiology
Clinical Manifestations
Treatment
Bibliography
Chapter 425: Aspiration Syndromes
Keywords
Aspiration Syndromes
Gastric Contents
Treatment
Prevention
Hydrocarbon Aspiration
Treatment
Bibliography
Chapter 426: Chronic Recurrent Aspiration
Keywords
Etiology
Diagnosis
Treatment
Bibliography
Chapter 427: Immune and Inflammatory Lung Disease
427.1 Hypersensitivity Pneumonia
Keywords
Etiology
Pathogenesis
Clinical Manifestations and Classification
Laboratory
Radiology
Bronchoalveolar Lavage
Lung Biopsy
Antigen Challenge by Inhalation
Treatment
Bibliography
427.2 Occupational and Environmental Lung Disease
Keywords
Classification and Pathogenesis
Occupational and Environmental Asthma
Reactive Airways Disease Syndrome and Irritant-Induced Asthma
Treatment
Bibliography
427.3 Granulomatous Lung Disease
Keywords
Granulomatosis With Polyangiitis
Etiology and Epidemiology
Pathogenesis
Clinical Manifestations
Laboratory and Pathology
Radiology
Treatment
Sarcoidosis
Epidemiology and Pathogenesis
Clinical Manifestations
Diagnostic Laboratory Testing
Histopathology
Radiology
Treatment
Berylliosis
Pathogenesis
Clinical Manifestations
Laboratory Testing
Radiography
Treatment
Granulomatous Lung Disease in Primary Immune Deficiency
Pathogenesis
Clinical Manifestations of Granulomatous Lung Disease in Primary Immune Deficiency
Radiography
Laboratory and Pulmonary Function Testing
Therapy
Bibliography
427.4 Eosinophilic Lung Disease
Keywords
Etiology
Pathology and Pathogenesis
Clinical Manifestations
Chest Imaging
Löffler Syndrome
Acute Eosinophilic Pneumonia
Chronic Eosinophilic Pneumonia
Eosinophilic Granulomatosis With Polyangiitis (the Churg-Strauss Syndrome)
Allergic Bronchopulmonary Aspergillosis
Radiography
Treatment
Hypereosinophilic Syndrome
Bibliography
427.5 Interstitial Lung Disease
Keywords
Classification and Pathology
Neuroendocrine Cell Hyperplasia of Infancy
Disorders of Surfactant Metabolism
Interstitial Lung Disease Due to Systemic Disease
Clinical Manifestations
Diagnosis
Radiography
Pulmonary Function Tests
Bronchoalveolar Lavage
Lung Biopsy
Treatment
Genetic Counseling
Prognosis
Anti-Glomerular Basement Membrane Disease (Anti-GBM Disease)
Pathophysiology
Immunology Factors
Genetic Factors
Environmental Factors
Clinical Manifestations
Laboratory
Chest Radiography
Pulmonary Function Testing
Bronchoscopy and Bronchoalveolar Lavage
Lung Biopsy
Treatment
Bibliography
427.6 Neuroendocrine Cell Hyperplasia of Infancy
Keywords
Background/Summary
Epidemiology
Pathophysiology
Clinical Presentation
Diagnosis
Natural Course and Treatment
Bibliography
427.7 Fibrotic Lung Disease
Keywords
Clinical Presentation
Evaluation
Treatment
Outcomes
Bibliography
Chapter 428: Community-Acquired Pneumonia
Keywords
Epidemiology
Etiology
Pathogenesis
Clinical Manifestations
Diagnosis
Treatment
Prognosis
Complications
Prevention
Bibliography
Chapter 429: Pleurisy, Pleural Effusions, and Empyema
Keyword
429.1 Dry Pleurisy
Etiology
Pathology and Pathogenesis
Clinical Manifestations
Laboratory Findings
Differential Diagnosis
Treatment
Bibliography
429.2 Serofibrinous or Serosanguineous Pleurisy With Pleural Effusion
Keywords
Etiology
Pathogenesis
Clinical Manifestations
Laboratory Findings
Diagnosis and Differential Diagnosis
Complications
Treatment
Bibliography
429.3 Empyema
Keywords
Etiology
Epidemiology
Pathology
Clinical Manifestations
Laboratory Findings
Complications
Treatment
Bibliography
Chapter 430: Bronchiectasis
Keywords
Pathophysiology and Pathogenesis
Clinical Manifestations
Diagnosis
Treatment
Prognosis
Bibliography
Chapter 431: Pulmonary Abscess
Keywords
Pathology and Pathogenesis
Clinical Manifestations
Diagnosis
Treatment
Prognosis
Bibliography
Chapter 432: Cystic Fibrosis
Genetics
Pathogenesis
Pathology
Clinical Manifestations
Respiratory Tract
Intestinal Tract
Biliary Tract
Cystic Fibrosis–Related Diabetes and Pancreatitis
Genitourinary Tract
Sweat Glands
Diagnosis and Assessment
Sweat Testing
DNA Testing
Other Diagnostic Tests
Pancreatic Function
Radiology
Pulmonary Function
Microbiologic Studies
Newborn Screening
Treatment
General Approach to Care
Pulmonary Therapy
Inhalation Therapy
Airway Clearance Therapy
Antibiotic Therapy
Oral Antibiotic Therapy
Aerosolized Antibiotic Therapy
Intravenous Antibiotic Therapy
Bronchodilator Therapy
Antiinflammatory Agents
Cystic Fibrosis Transmembrane Conductance Regulator Modulator Therapies
Other Therapies
Treatment of Pulmonary Complications
Atelectasis
Hemoptysis
Pneumothorax
Allergic Bronchopulmonary Aspergillosis
Nontuberculous Mycobacteria Infection
Bone and Joint Complications
Sleep-Disordered Breathing
Acute Respiratory Failure
Chronic Respiratory Failure
Pulmonary Hypertension and Cor Pulmonale
Nutritional Therapy
Diet
Pancreatic Enzyme Replacement
Vitamin and Mineral Supplements
Treatment of Intestinal Complications
Meconium Ileus
Distal Intestinal Obstruction Syndrome and Other Causes of Abdominal Symptoms
Rectal Prolapse
Hepatobiliary Disease
Pancreatitis
Cystic Fibrosis-Related Hyperglycemia and Diabetes
Other Complications
Nasal Polyps
Rhinosinusitis
Salt Depletion
Surgery
Prognosis
Bibliography
Chapter 433: Primary Ciliary Dyskinesia (Immotile Cilia Syndrome, Kartagener Syndrome)
Keywords
Normal Ciliary Ultrastructure and Function
Genetics of Primary Ciliary Dyskinesia
Clinical Manifestations of Primary Ciliary Dyskinesia
Diagnosis of Primary Ciliary Dyskinesia
Treatment
Prognosis
Bibliography
Chapter 434: Diffuse Lung Diseases in Childhood
Keywords
434.1 Inherited Disorders of Surfactant Metabolism
Pathology
Deficiency of Surfactant Protein B (Surfactant Metabolism Dysfunction, Pulmonary, 1; Smdp1; Omim #265120)
Clinical Manifestations
Genetics
Diagnosis
Surfactant Protein C Gene Abnormalities (Surfactant Metabolism Dysfunction, Pulmonary, 2; Smdp2; Omim #610913)
Clinical Manifestations
Genetics
Diagnosis
Disease Caused by Mutations in ABCA3 (Surfactant Metabolism Dysfunction, Pulmonary, 3; Smdp3; Omim #610921)
Clinical Manifestations
Genetics
Diagnosis
Disease Caused by Mutations in NKX2-1 (Thyroid Transcription Factor 1, Choreoathetosis, Hypothyroidism, and Neonatal Respiratory Distress, Omim #600635)
Clinical Manifestations
Genetics
Diagnosis
Treatment of Surfactant Dysfunction Disorders
Bibliography
434.2 Pulmonary Alveolar Proteinosis
Etiology and Pathophysiology
Primary Alveolar Proteinosis
Secondary Alveolar Proteinosis
Clinical Manifestations
Diagnosis
Treatment
Bibliography
Chapter 435: Pulmonary Hemosiderosis
Keywords
Etiology
Epidemiology
Pathology
Pathophysiology
Diffuse Alveolar Hemorrhage Associated With Pulmonary Capillaritis
Diffuse Alveolar Hemorrhage Not Associated With Pulmonary Capillaritis
Clinical Manifestations
Laboratory Findings and Diagnosis
Treatment
Prognosis
Bibliography
Chapter 436: Pulmonary Embolism, Infarction, and Hemorrhage
436.1 Pulmonary Embolus and Infarction
Keywords
Etiology
Epidemiology
Pathophysiology
Clinical Manifestations
Laboratory Findings and Diagnosis
Treatment
Prognosis
Bibliography
436.2 Pulmonary Hemorrhage and Hemoptysis
Etiology
Epidemiology
Pathophysiology
Clinical Manifestations
Laboratory Findings and Diagnosis
Treatment
Bibliography
Chapter 437: Atelectasis
Keywords
Pathophysiology
Clinical Manifestations
Diagnosis
Treatment
Bibliography
Chapter 438: Pulmonary Tumors
Keywords
Etiology
Clinical Manifestations and Evaluation
Bibliography
Chapter 439: Pneumothorax
Keywords
Etiology and Epidemiology
Pathogenesis
Clinical Manifestations
Diagnosis and Differential Diagnosis
Treatment
Bibliography
Chapter 440: Pneumomediastinum
Keywords
Etiology
Pathogenesis
Clinical Manifestations
Laboratory Findings
Treatment
Complications
Bibliography
Chapter 441: Hydrothorax
Keywords
Etiology
Clinical Manifestations
Laboratory Findings
Treatment
Bibliography
Chapter 442: Hemothorax
Keywords
Etiology
Clinical Manifestations
Diagnosis
Treatment
Bibliography
Chapter 443: Chylothorax
Keywords
Etiology
Clinical Manifestations
Laboratory Findings
Treatment
Complications
Bibliography
Chapter 444: Bronchopulmonary Dysplasia
Keywords
Bronchopulmonary Dysplasia
Clinical Manifestations
Treatment
Prognosis
Bibliography
Chapter 445: Skeletal Diseases Influencing Pulmonary Function
445.1 Pectus Excavatum (Funnel Chest)
Keywords
Etiology
Epidemiology
Clinical Manifestations
Laboratory Findings
Treatment
Bibliography
445.2 Pectus Carinatum and Sternal Clefts
Keywords
Pectus Carinatum
Etiology and Epidemiology
Clinical Manifestations
Treatment
Sternal Clefts
Bibliography
445.3 Asphyxiating Thoracic Dystrophy (Thoracic-Pelvic-Phalangeal Dystrophy)
Keywords
Etiology
Clinical Manifestations
Diagnosis
Treatment
Prognosis
Bibliography
445.4 Achondroplasia
Keyword
Etiology
Clinical Manifestations
Diagnosis
Treatment
Prognosis
Bibliography
445.5 Kyphoscoliosis: Adolescent Idiopathic Scoliosis and Congenital Scoliosis
Keywords
Etiology
Clinical Manifestations
Diagnosis
Treatment
Bibliography
445.6 Congenital Rib Anomalies
Keyword
Clinical Manifestations
Diagnosis
Treatment
Bibliography
Chapter 446: Chronic Respiratory Insufficiency
446.1 Chronic Respiratory Failure and Long-Term Mechanical Ventilation
Epidemiology
Modalities for Respiratory Support
Invasive Positive Pressure Ventilation
Optimal Ventilator Support
Other Management Considerations
Airway Clearance
Inhalation Medications
Mucolytics and Anticholinergics
Monitoring
Supplemental Oxygen
Nutrition
Physical, Occupational, Speech Therapy
Preparing for Discharge
Care by the General Pediatrician
Infections
Care by the Subspecialty Team
Weaning Off the Ventilator
Psychosocial Considerations
Adult Transition
Bibliography
446.2 Congenital Central Hypoventilation Syndrome
Keywords
Genetics
Ventilator Dependence and Control of Breathing
Hirschsprung Disease (Chapter 358)
Tumors of Neural Crest Origin (Chapter 525)
Cardiac Asystole
Autonomic Nervous System Dysregulation
Neuropathology
Clinical Manifestations
Differential Diagnosis
Management
Supported Ventilation—Diaphragm Pacing
Monitoring in the Home
Noninvasive Equipment
Positive Pressure Ventilators
Optimizing Neurocognitive Performance
Bibliography
446.3 Other Conditions Affecting Respiration
Myelomeningocele With Arnold-Chiari Type II Malformation
Management
Rapid-Onset Obesity, Hypothalamic Dysfunction, and Autonomic Dysregulation
Obesity Hypoventilation Syndrome
Management
Acquired Alveolar Hypoventilation
Obstructive Sleep Apnea
Epidemiology
Pathophysiology
Clinical Presentation
Treatment
Spinal Cord Injury
Epidemiology
Pathophysiology
Management
Metabolic Disease
Mucopolysaccharidoses
Dysplasias
Glycogenosis Type II
Severe Tracheomalacia and/or Bronchomalacia (Airway Malacia)
Neuropathy of Severe Illness
Mitochondrial Diseases
446.4 Long-Term Mechanical Ventilation
Keywords
Goals and Decision-Making
Noninvasive and Transtracheal Supports
Augmented Secretion Clearance
Aerodigestive and Communication Considerations
Gas-Exchange Goals and Ventilator Strategies
Cardiopulmonary Interactions
Chest Wall/Thoracic Configuration
Nutrition and Weight Gain
Developmental Considerations
Projected Interventions and Needs
Monitoring
Transitioning From Acute Care to Rehabilitation or Community Setting
Routine Health Maintenance
Airway Evaluation
Bacterial Colonization
Dental Care
Immunizations
Radiography, Laboratory Evaluation, Polysomnography, and Pulmonary Function Testing
Long-Term Mechanical Ventilation Weaning and Tracheal Decannulation
Bibliography
Part XIX: The Cardiovascular System
Section 1: Developmental Biology of the Cardiovascular System
Chapter 447: Cardiac Development
Keywords
447.1 Early Cardiac Morphogenesis
447.2 Cardiac Looping
447.3 Cardiac Septation
447.4 Aortic Arch Development
447.5 Cardiac Differentiation
447.6 Developmental Changes in Cardiac Function
Bibliography
Chapter 448: The Fetal to Neonatal Circulatory Transition
448.1 The Fetal Circulation
Keywords
448.2 The Transitional Circulation
448.3 The Neonatal Circulation
448.4 Persistent Pulmonary Hypertension of the Neonate (Persistence of Fetal Circulatory Pathways)
Bibliography
Section 2: Evaluation of the Cardiovascular System and the Child With a Heart Murmur
Chapter 449: History and Physical Examination in Cardiac Evaluation
Keywords
History
General Physical Examination
Cardiac Examination
Bibliography
Chapter 450: Laboratory Cardiac Evaluation
450.1 Radiologic Cardiac Assessment
Keywords
450.2 Electrocardiography
Developmental Changes
Rate and Rhythm
P Waves
QRS Complex
Right Ventricular Hypertrophy
Left Ventricular Hypertrophy
Bundle Branch Block
P-R and Q-T Intervals
ST Segment and T-Wave Abnormalities
Bibliography
450.3 Hematologic Data
450.4 Echocardiography
M-Mode Echocardiography
Two-Dimensional Echocardiography
Doppler Echocardiography
Three-Dimensional Echocardiography
Transesophageal Echocardiography
Fetal Echocardiography
Bibliography
450.5 Exercise Testing
Bibliography
450.6 Cardiac Imaging Studies
Bibliography
450.7 Diagnostic and Interventional Cardiac Catheterization
Diagnostic Cardiac Catheterization
Thermodilution Measurement of Cardiac Output
Angiocardiography
Interventional Cardiac Catheterization
Bibliography
Section 3: Congenital Heart Disease
Chapter 451: Epidemiology and Genetic Basis of Congenital Heart Disease
Keywords
Prevalence
Etiology
Next-Generation Genome Sequencing and Congenital Heart Disease
Genetic Counseling
Bibliography
Chapter 452: Evaluation and Screening of the Infant or Child With Congenital Heart Disease
Keywords
Acyanotic Congenital Heart Lesions
Lesions Resulting in Increased Volume Load
Lesions Resulting in Increased Pressure Load
Cyanotic Congenital Heart Lesions
Cyanotic Lesions With Decreased Pulmonary Blood Flow
Cyanotic Lesions With Increased Pulmonary Blood Flow
Bibliography
Chapter 453: Acyanotic Congenital Heart Disease: Left-to-Right Shunt Lesions
Keywords
453.1 Atrial Septal Defect
Bibliography
453.2 Ostium Secundum Defect
Pathophysiology
Clinical Manifestations
Diagnosis
Complications
Treatment
Prognosis
453.3 Sinus Venosus Atrial Septal Defect
453.4 Partial Anomalous Pulmonary Venous Return
453.5 Atrioventricular Septal Defects (Ostium Primum and Atrioventricular Canal or Endocardial Cushion Defects)
Pathophysiology
Clinical Manifestations
Diagnosis
Treatment
Prognosis
Bibliography
453.6 Ventricular Septal Defect
Pathophysiology
Clinical Manifestations
Diagnosis
Treatment
Prognosis
Bibliography
453.7 Supracristal Ventricular Septal Defect With Aortic Insufficiency
453.8 Patent Ductus Arteriosus
Pathophysiology
Clinical Manifestations
Diagnosis
Prognosis and Complications
Treatment
Patent Ductus Arteriosus in Low-Birthweight Infants
Bibliography
453.9 Aortopulmonary Window Defect
453.10 Coronary Artery Fistula
453.11 Ruptured Sinus of Valsalva Aneurysm
Chapter 454: Acyanotic Congenital Heart Disease: Obstructive Lesions
Keywords
454.1 Pulmonary Valve Stenosis With Intact Ventricular Septum
Pathophysiology
Clinical Manifestations and Laboratory Findings
Treatment
Prognosis and Complications
Bibliography
454.2 Infundibular Pulmonary Stenosis and Double-Chamber Right Ventricle
454.3 Pulmonary Stenosis in Combination With an Intracardiac Shunt
454.4 Peripheral Pulmonary Stenosis
454.5 Aortic Stenosis
Pathophysiology
Clinical Manifestations
Laboratory Findings and Diagnosis
Treatment
Prognosis
Bibliography
454.6 Coarctation of the Aorta
Pathophysiology
Clinical Manifestations
Diagnosis
Treatment
Postcoarctectomy Syndrome
Prognosis
Bibliography
454.7 Coarctation With Ventricular Septal Defect
454.8 Coarctation With Other Cardiac Anomalies and Interrupted Aortic Arch
454.9 Congenital Mitral Stenosis
Bibliography
454.10 Pulmonary Venous Hypertension
Chapter 455: Acyanotic Congenital Heart Disease: Regurgitant Lesions
455.1 Pulmonary Valvular Insufficiency and Congenital Absence of the Pulmonary Valve
Keywords
Bibliography
455.2 Congenital Mitral Insufficiency
455.3 Mitral Valve Prolapse
Bibliography
455.4 Tricuspid Regurgitation
Bibliography
Chapter 456: Cyanotic Congenital Heart Disease: Evaluation of the Critically Ill Neonate With Cyanosis and Respiratory Distress
Keywords
Cardiac Disease Leading to Cyanosis
Differential Diagnosis
Chapter 457: Cyanotic Congenital Heart Disease: Lesions Associated With Decreased Pulmonary Blood Flow
Keywords
457.1 Tetralogy of Fallot
Pathophysiology
Clinical Manifestations
Diagnosis
Complications
Associated Anomalies
Treatment
Prognosis
Bibliography
457.2 Tetralogy of Fallot With Pulmonary Atresia
Pathophysiology
Clinical Manifestations
Diagnosis
Treatment
Bibliography
457.3 Pulmonary Atresia With Intact Ventricular Septum
Pathophysiology
Clinical Manifestations
Diagnosis
Treatment
Bibliography
457.4 Tricuspid Atresia
Pathophysiology
Clinical Manifestations
Diagnosis
Treatment
Bibliography
457.5 Double-Outlet Right Ventricle
457.6 Transposition of the Great Arteries With Ventricular Septal Defect and Pulmonary Stenosis
457.7 Ebstein Anomaly of the Tricuspid Valve
Pathophysiology
Clinical Manifestations
Diagnosis
Prognosis and Complications
Treatment
Bibliography
Chapter 458: Cyanotic Congenital Heart Disease: Lesions Associated With Increased Pulmonary Blood Flow
Keywords
458.1 d-Transposition of the Great Arteries
458.2 d-Transposition of the Great Arteries With Intact Ventricular Septum
Clinical Manifestations
Diagnosis
Treatment
458.3 Transposition of the Great Arteries With Ventricular Septal Defect
458.4 L-Transposition of the Great Arteries (Corrected Transposition)
Clinical Manifestations
Diagnosis
Bibliography
458.5 Double-Outlet Right Ventricle Without Pulmonary Stenosis
458.6 Double-Outlet Right Ventricle With Malposition of the Great Arteries (Taussig-Bing Anomaly)
458.7 Total Anomalous Pulmonary Venous Return
Pathophysiology
Clinical Manifestations
Diagnosis
Treatment
Bibliography
458.8 Truncus Arteriosus
Pathophysiology
Clinical Manifestations
Diagnosis
Prognosis and Complications
Treatment
Bibliography
458.9 Single Ventricle (Double-Inlet Ventricle, Univentricular Heart)
Pathophysiology
Clinical Manifestations
Diagnosis
Prognosis and Complications
Treatment
458.10 Hypoplastic Left Heart Syndrome
Pathophysiology
Clinical Manifestations
Diagnosis
Prognosis and Complications
Treatment
Prevention
Bibliography
458.11 Abnormal Positions of the Heart and the Heterotaxy Syndromes (Asplenia, Polysplenia)
Bibliography
Chapter 459: Other Congenital Heart and Vascular Malformations
Keywords
459.1 Anomalies of the Aortic Arch
Right Aortic Arch
Vascular Rings
Clinical Manifestations
Diagnosis
Treatment
Bibliography
459.2 Anomalous Origin of the Coronary Arteries
Anomalous Origin of Left Coronary Artery From Pulmonary Artery
Clinical Manifestations
Diagnosis
Treatment and Prognosis
Anomalous Origin of Right Coronary Artery From Pulmonary Artery
Ectopic Origin of a Coronary Artery From the Aorta With Aberrant Proximal Course
Bibliography
459.3 Pulmonary Arteriovenous Fistula
Bibliography
459.4 Ectopia Cordis
459.5 Diverticulum of the Left Ventricle
Chapter 460: Pulmonary Hypertension
Keywords
460.1 Primary Pulmonary Hypertension
Pathophysiology
Clinical Manifestations
Diagnosis
Prognosis and Treatment
460.2 Pulmonary Vascular Disease (Eisenmenger Syndrome)
Pathophysiology
Pathology and Pathophysiology
Clinical Manifestations
Diagnosis
Treatment
Bibliography
Chapter 461: General Principles of Treatment of Congenital Heart Disease
Keywords
Postoperative Management
Interstage Management
Long-Term Management
461.1 Congenital Heart Disease in Adults
Keywords
Long-Term Medical Considerations
Specific Lesions
Left-to-Right Shunts
Atrial Septal Defects
Late Outcome Following Closure of Atrial Septal Defect
Ventricular Septal Defects
Complete Atrioventricular Canal
Patent Ductus Arteriosus
Cyanotic Heart Disease
Tetralogy of Fallot
Transposition of the Great Arteries
Pulmonary Valve Stenosis
Left-Sided Obstructive Lesions
Coarctation of the Aorta
Aortic Valve Stenosis
Endocarditis Prophylaxis
Pregnancy and Congenital Heart Disease
Contraception
Adolescent Transition
Bibliography
Bibliography
Section 4: Cardiac Arrhythmias
Chapter 462: Disturbances of Rate and Rhythm of the Heart
Keywords
462.1 Principles of Antiarrhythmic Therapy
462.2 Sinus Arrhythmias and Extrasystoles
462.3 Supraventricular Tachycardia
Clinical Manifestations
Treatment
462.4 Ventricular Tachyarrhythmias
462.5 Long QT Syndromes
462.6 Sinus Node Dysfunction
462.7 Atrioventricular Block
Bibliography
Chapter 463: Sudden Death
Keywords
Mechanism of Sudden Death
Congenital Heart Disease
Cardiomyopathy
Cardiac Arrhythmia
Miscellaneous Causes
Evaluation and Therapy for Resuscitated Patients
Medication for Attention-Deficit/Hyperactivity Disorder
Prevention of Sudden Death
Bibliography
Section 5: Acquired Heart Disease
Chapter 464: Infective Endocarditis
Keywords
Etiology
Epidemiology
Clinical Manifestations
Diagnosis
Prognosis and Complications
Treatment
Prevention
Bibliography
Chapter 465: Rheumatic Heart Disease
Keywords
Patterns of Valvular Disease
Mitral Insufficiency
Pathophysiology
Clinical Manifestations
Complications
Treatment
Mitral Stenosis
Pathophysiology
Clinical Manifestations
Treatment
Aortic Insufficiency
Clinical Manifestations
Prognosis and Treatment
Tricuspid Valve Disease
Pulmonary Valve Disease
Bibliography
Section 6: Diseases of the Myocardium and Pericardium
Chapter 466: Diseases of the Myocardium
466.1 Dilated Cardiomyopathy
Keywords
Etiology and Epidemiology
Pathogenesis
Clinical Manifestations
Laboratory Findings
Prognosis and Management
Bibliography
466.2 Hypertrophic Cardiomyopathy
Keywords
Etiology and Epidemiology
Pathogenesis
Clinical Manifestations
Diagnosis
Prognosis and Management
Bibliography
466.3 Restrictive Cardiomyopathy
Keywords
Etiology and Epidemiology
Pathogenesis
Clinical Manifestations
Diagnosis
Prognosis and Management
Bibliography
466.4 Left Ventricular Noncompaction, Arrhythmogenic Right Ventricular Cardiomyopathy, Endocardial Fibroelastosis, and Takotsubo Cardiomyopathy
Keywords
Bibliography
466.5 Myocarditis
Keywords
Etiology and Epidemiology
Viral Infections
Bacterial Infections
Pathophysiology
Clinical Manifestations
Diagnosis
Differential Diagnosis
Treatment
Prognosis
Bibliography
Chapter 467: Diseases of the Pericardium
467.1 Acute Pericarditis
Keywords
Pathogenesis
Clinical Manifestations
Diagnosis
Differential Diagnosis
Infectious Pericarditis
Noninfectious Pericarditis
467.2 Constrictive Pericarditis
Keywords
Bibliography
Chapter 468: Tumors of the Heart
Keywords
Bibliography
Section 7: Cardiac Therapeutics
Chapter 469: Heart Failure
Keywords
Pathophysiology
Clinical Manifestations
Diagnosis
Treatment
General Measures
Diet
Diuretics
Afterload Reducers, Including Angiotensin-Converting Enzyme Inhibitors and Angiotensin II Receptor Blockers
Digitalis Glycosides
Alpha- and β-Adrenergic Agonists
Phosphodiesterase Inhibitors
Chronic Treatment With β-Blockers
New Therapies
Electrophysiologic Approaches to Heart Failure Management
469.1 Cardiogenic Shock
Mechanical Circulatory Support
Bibliography
Chapter 470: Pediatric Heart and Heart-Lung Transplantation
Keywords
470.1 Pediatric Heart Transplantation
Indications
Recipient and Donor Selection
Perioperative Management
Diagnosis and Management of Acute Graft Rejection
Complications of Immunosuppression
Infection
Growth Retardation
Hypertension
Renal Function
Neurologic Complications
Tumors
Cardiac Allograft Vasculopathy
Other Complications
Rehabilitation
Bibliography
470.2 Heart-Lung and Lung Transplantation
Bibliography
Section 8: Diseases of the Peripheral Vascular System
Chapter 471: Diseases of the Blood Vessels (Aneurysms and Fistulas)
471.1 Kawasaki Disease
Keywords
471.2 Arteriovenous Fistulas
Clinical Manifestations
Treatment
471.3 Generalized Arterial Calcification of Infancy/Idiopathic Infantile Arterial Calcification
Arterial Calcifications Caused by Deficiency of CD73
471.4 Arterial Tortuosity
Bibliography
Chapter 472 - Systemic Hypertension
472 Systemic Hypertension
Keywords
Prevalence of Hypertension in Children
Definition of Hypertension
Blood Pressure Measurement in Children
Ambulatory Blood Pressure Monitoring
Etiology and Pathophysiology
Clinical Manifestations
Diagnosis
Prevention
Treatment
Bibliography
Part XX: Diseases of the Blood
Section 1: The Hematopoietic System
Chapter 473: Development of the Hematopoietic System
Keywords
Hematopoiesis in the Human Embryo and Fetus
Fetal Granulocytopoiesis
Fetal Thrombopoiesis
Fetal Erythropoiesis
Hemoglobins in the Fetus and Neonate
Embryonic Hemoglobins
Fetal Hemoglobin
Adult Hemoglobins
Alterations of the Hemoglobins by Disease
Red Cell Life Span in the Fetus and Neonate
Bibliography
Chapter 474: The Anemias
Keywords
History and Physical Examination
Laboratory Studies
Differential Diagnosis
Bibliography
Section 2: Anemias of Inadequate Production
Chapter 475: Congenital Hypoplastic Anemia (Diamond-Blackfan Anemia)
Keywords
Etiology
Epidemiology
Clinical Manifestations
Laboratory Findings
Differential Diagnosis
Treatment
Prognosis
Bibliography
Chapter 476: Pearson Syndrome
Keywords
Bibliography
Chapter 477: Acquired Pure Red Blood Cell Anemia
Keywords
Transient Erythroblastopenia of Childhood
Red Cell Aplasia Associated With Parvovirus B19 Infection
Other Red Cell Aplasias in Children
Bibliography
Chapter 478: Anemia of Chronic Disease and Renal Disease
Keywords
478.1 Anemia of Chronic Disease
Etiology
Clinical Manifestations
Laboratory Findings
Treatment
Bibliography
478.2 Anemia of Renal Disease
Etiology
Laboratory Findings
Treatment
Bibliography
Chapter 479: Congenital Dyserythropoietic Anemias
Keywords
Congenital Dyserythropoietic Anemia Type I
Pathogenesis
Clinical Manifestations
Laboratory Findings
Treatment
Congenital Dyserythropoietic Anemia Type II
Pathogenesis
Clinical Manifestations
Laboratory Findings
Treatment
Congenital Dyserythropoietic Anemia Type III
Bibliography
Chapter 480: Physiologic Anemia of Infancy
Keywords
Treatment
Bibliography
Chapter 481: Megaloblastic Anemias
Keywords
481.1 Folic Acid Deficiency
Etiology
Inadequate Folate Intake
Decreased Folate Absorption
Congenital Abnormalities in Folate Transport and Metabolism
Drug-Induced Abnormalities in Folate Metabolism
Clinical Manifestations
Laboratory Findings
Treatment
Bibliography
481.2 Vitamin B12 (Cobalamin) Deficiency
Keywords
Metabolism
Etiology
Inadequate Vitamin B12 Intake
Impaired Vitamin B12 Absorption
Absence of Vitamin B12 Transport Protein Transcobalamin
Inborn Errors of Cobalamin Metabolism
Clinical Manifestations
Laboratory Findings
Diagnosis
Treatment
Bibliography
481.3 Other Rare Megaloblastic Anemias
Keywords
Bibliography
Chapter 482: Iron-Deficiency Anemia
Keywords
Etiology
Clinical Manifestations
Laboratory Findings
Differential Diagnosis
Prevention
Treatment
482.1 Iron-Refractory Iron-Deficiency Anemia
Keywords
Etiology
Clinical Manifestations
Laboratory Findings
Differential Diagnosis
Treatment
Bibliography
Bibliography
Chapter 483: Other Microcytic Anemias
Keywords
Infantile Poikilocytosis and Hereditary Pyropoikilocytosis
Copper Deficiency
Defects of Iron Metabolism
Bibliography
Section 3: Hemolytic Anemias
Chapter 484: Definitions and Classification of Hemolytic Anemias
Keywords
Bibliography
Chapter 485: Hereditary Spherocytosis
Keywords
Etiology
Clinical Manifestations
Diagnosis
Differential Diagnosis
Treatment
General Supportive Care
Guidelines for Splenectomy
Bibliography
Chapter 486: Hereditary Elliptocytosis, Hereditary Pyropoikilocytosis, and Related Disorders
Keywords
Etiology
Clinical Manifestations
Laboratory Findings
Treatment
Bibliography
Chapter 487: Hereditary Stomatocytosis
Keywords
Hereditary Xerocytosis
Hereditary Hydrocytosis
Intermediate Syndromes and Other Variants
Cryohydrocytosis
Rh Deficiency Syndrome
Familial Deficiency of High-Density Lipoproteins
Sitosterolemia
Other Disorders Associated With Stomatocytosis
Bibliography
Chapter 488: Paroxysmal Nocturnal Hemoglobinuria and Acanthocytosis
Keywords
Paroxysmal Nocturnal Hemoglobinuria
Etiology
Clinical Manifestations
Laboratory Findings
Treatment
Acanthocytosis
Bibliography
Chapter 489: Hemoglobinopathies
Keywords
Hemoglobin Disorders
489.1 Sickle Cell Disease
Pathophysiology
Diagnosis and Epidemiology
Clinical Manifestations and Treatment of Sickle Cell Anemia (HbSS)
Fever and Bacteremia
Aplastic Crisis
Splenic Sequestration
Hepatic and Gallbladder Involvement
Sickle Cell Pain
Avascular Necrosis
Priapism
Neurologic Complications
Transcranial Doppler Ultrasonography
Pulmonary Complications
Renal Disease and Enuresis
Cognitive and Psychological Complications
Other Complications
Therapeutic Considerations
Hydroxyurea
Hematopoietic Stem Cell Transplantation
Red Blood Cell Transfusions
Iron Overload
Other Sickle Cell Syndromes
Anticipatory Guidance
Prophylactic Penicillin
Immunizations
Spleen Palpation
Transcranial Doppler Ultrasound
Hydroxyurea
Red Cell Transfusion Therapy
Pulmonary and Asthma Screening
Retinopathy
Renal Disease
Echocardiography
Additional Screening
Bibliography
489.2 Sickle Cell Trait (Hemoglobin AS)
Bibliography
489.3 Other Hemoglobinopathies
Hemoglobin C
Hemoglobin E
Hemoglobin D
489.4 Unstable Hemoglobin Disorders
489.5 Abnormal Hemoglobins With Increased Oxygen Affinity
489.6 Abnormal Hemoglobins Causing Cyanosis
489.7 Hereditary Methemoglobinemia
489.8 Hereditary Methemoglobinemia With Deficiency of NADH Cytochrome b5 Reductase
489.9 Syndromes of Hereditary Persistence of Fetal Hemoglobin
489.10 Thalassemia Syndromes
Epidemiology
Pathophysiology
Homozygous β-Thalassemia (Thalassemia Major, Cooley Anemia)
Clinical Manifestations
Laboratory Findings
Management and Treatment of Thalassemia
Transfusion Therapy
Guidelines for Transfusion Therapy.
Iron Overload Monitoring
Chelation Therapy
Hydroxyurea
Hematopoietic Stem Cell Transplantation
Splenectomy
Preventive Monitoring of Thalassemia Patients
Cardiac Disease
Endocrine Disease
Psychosocial Support
Other β-Thalassemia Syndromes
Non–Transfusion-Dependent Thalassemia: β-Thalassemia Intermedia
α-Thalassemia Syndromes
Bibliography
Chapter 490: Enzymatic Defects
490.1 Pyruvate Kinase Deficiency
Keywords
Etiology
Clinical Manifestations and Laboratory Findings
Treatment
490.2 Other Glycolytic Enzyme Deficiencies
Keywords
Deficiencies of Enzymes of Hexose Monophosphate Pathway
490.3 Glucose-6-Phosphate Dehydrogenase Deficiency and Related Deficiencies
Keywords
Episodic or Induced Acute Hemolytic Anemia
Etiology
Clinical Manifestations
Laboratory Findings
Diagnosis
Prevention and Treatment
Chronic Hemolytic Anemias Associated With Deficiency of G6PD or Related Factors
Bibliography
Chapter 491: Hemolytic Anemias Resulting from Extracellular Factors—Immune Hemolytic Anemias
Keywords
Immune Hemolytic Anemias
Autoimmune Hemolytic Anemias Associated With “Warm” Antibodies
Etiology
Clinical Manifestations
Laboratory Findings
Treatment
Course and Prognosis
Autoimmune Hemolytic Anemias Associated With “Cold” Antibodies
Cold Agglutinin Disease
Paroxysmal Cold Hemoglobinuria
Bibliography
Chapter 492: Hemolytic Anemias Secondary to Other Extracellular Factors
Keywords
Fragmentation Hemolysis
Thermal Injury
Renal Disease
Liver Disease
Toxins and Venoms
Wilson Disease
Bibliography
Section 4: Polycythemia (Erythrocytosis)
Chapter 493: Polycythemia
Keywords
Clonal (Primary) Polycythemia (Polycythemia Vera)
Pathogenesis
Clinical Manifestations
Treatment
Bibliography
Chapter 494: Nonclonal Polycythemia
Keywords
Pathogenesis
Congenital Polycythemia
Acquired Polycythemia
Diagnosis
Treatment
Bibliography
Section 5: The Pancytopenias
Chapter 495: Inherited Bone Marrow Failure Syndromes With Pancytopenia
Keywords
Fanconi Anemia
Etiology and Epidemiology
Pathology
Clinical Manifestations
Complications
Diagnosis
Treatment
Prognosis
Shwachman-Diamond Syndrome
Etiology and Epidemiology
Pathology
Clinical Manifestations
Laboratory Findings
Diagnosis
Complications
Treatment
Prognosis
Dyskeratosis Congenita
Etiology and Epidemiology
Pathology
Clinical Manifestations
Laboratory Findings
Diagnosis
Complications
Treatment
Prognosis
Congenital Amegakaryocytic Thrombocytopenia
Etiology and Epidemiology
Clinical Manifestations
Laboratory Findings
Diagnosis
Complications
Therapy and Prognosis
Other Inherited Aplastic Anemias
Reticular Dysgenesis
Cartilage-Hair Hypoplasia
Other Inherited Syndromes With Occasional Significant Bone Marrow Failure
Down Syndrome
Dubowitz Syndrome
Seckel Syndrome
Schimke Immunoosseous Dysplasia
Noonan Syndrome
Unclassified Inherited Bone Marrow Failure Syndromes
Bibliography
Chapter 496: The Acquired Pancytopenias
Keywords
Etiology and Epidemiology
Pathology and Pathogenesis
Clinical Manifestations, Laboratory Findings, and Differential Diagnosis
Treatment
Complications
Prognosis
Pancytopenia Caused by Marrow Replacement
Bibliography
Section 6: Blood Component Transfusions
Chapter 497: Red Blood Cell Transfusions and Erythropoietin Therapy
Keywords
RBC Transfusion in Children and Adolescents
RBC Transfusion in Preterm Infants and Neonates
RBC Product and Dose
Storage Age of RBC Units
Bibliography
Chapter 498: Platelet Transfusions
Keywords
Children and Adolescents
Infants and Neonates
Platelet Products and Dosing
Bibliography
Chapter 499: Neutrophil (Granulocyte) Transfusions
Keywords
Granulocyte Transfusions for Children
Granulocyte Transfusion for Neonates
Granulocyte Product
Granulocyte Product Dosing
Bibliography
Chapter 500: Plasma Transfusions
Keywords
Plasma Products and Patient Testing
Plasma Transfusion in Children
Plasma Transfusion in Neonates
Bibliography
Chapter 501: Risks of Blood Transfusions
Keywords
Infectious Risks of Transfusion
Noninfectious Risks of Transfusion
Bibliography
Section 7: Hemorrhagic and Thrombotic Diseases
Chapter 502: Hemostasis
Key Words
The Hemostatic Process
Pathology
502.1 Clinical and Laboratory Evaluation of Hemostasis
Keywords
Clinical History
Physical Examination
Laboratory Tests
Platelet Count
Prothrombin Time and Activated Partial Thromboplastin Time
Partial Thromboplastin Time
Thrombin Time
Reptilase Time
Mixing Studies
Platelet Aggregation
Testing for Thrombotic Predisposition
Tests of the Fibrinolytic System
Developmental Hemostasis
Bibliography
Chapter 503: Hereditary Clotting Factor Deficiencies (Bleeding Disorders)
Keywords
503.1 Factor VIII or Factor IX Deficiency (Hemophilia A or B)
Keywords
Pathophysiology
Clinical Manifestations
Laboratory Findings and Diagnosis
Differential Diagnosis
Genetics and Classification
Treatment
Prophylaxis
Supportive Care
Chronic Complications
Inhibitor Formation
Comprehensive Care
Bibliography
503.2 Factor XI Deficiency (Hemophilia C)
Keywords
Bibliography
503.3 Deficiencies of the Contact Factors (Nonbleeding Disorders)
Keywords
503.4 Factor VII Deficiency
Keywords
Bibliography
503.5 Factor X Deficiency
Keywords
Bibliography
503.6 Prothrombin (Factor II) Deficiency
Keywords
Bibliography
503.7 Factor V Deficiency
Keywords
Bibliography
503.8 Combined Deficiency of Factors V and VIII
Keywords
Bibliography
503.9 Fibrinogen (Factor I) Deficiency
Keywords
Bibliography
503.10 Factor XIII Deficiency (Fibrin-Stabilizing Factor or Transglutaminase Deficiency)
Keywords
Bibliography
503.11 Antiplasmin or Plasminogen Activator Inhibitor Deficiency
Keywords
Bibliography
Chapter 504: Von Willebrand Disease
Keywords
Pathophysiology
Classification
Laboratory Diagnosis
Treatment
Bibliography
Chapter 505: Hereditary Predisposition to Thrombosis
Keywords
Bibliography
Chapter 506: Thrombotic Disorders in Children
Keywords
Epidemiology
Clinical Manifestations
Extremity Deep Vein Thrombosis
Pulmonary Embolism
Cerebral Sinovenous Thrombosis
Renal Vein Thrombosis
Peripheral Arterial Thrombosis
Stroke
Rapidly Progressive Thrombosis (Thrombotic Storm)
Diagnosis
Laboratory Testing
Treatment
Complications
506.1 Anticoagulant and Thrombolytic Therapy
Keywords
Unfractionated Heparin
Heparin Dosing
Heparin Complications
Low-Molecular-Weight Heparin
Enoxaparin Dosing
Warfarin
Dosing
Complications
Direct Oral Anticoagulants
Thrombolytic Therapy
Dosing
Monitoring
Complications
Thromboprophylaxis
Antiplatelet Therapy
Bibliography
Chapter 507: Postneonatal Vitamin K Deficiency
Keywords
Bibliography
Chapter 508: Liver Disease
Keywords
Bibliography
Chapter 509: Acquired Inhibitors of Coagulation
Keywords
Laboratory Findings
Treatment
Bibliography
Chapter 510: Disseminated Intravascular Coagulation
Keywords
Etiology
Clinical Manifestations
Laboratory Findings
Treatment
Bibliography
Chapter 511: Platelet and Blood Vessel Disorders
Keywords
Megakaryopoiesis
Thrombocytopenia
511.1 Idiopathic (Autoimmune) Thrombocytopenic Purpura
Keywords
Epidemiology
Pathogenesis
Clinical Manifestations
Outcome
Laboratory Findings
Diagnosis and Differential Diagnosis
Treatment
Chronic Autoimmune Thrombocytopenic Purpura
Bibliography
511.2 Drug-Induced Thrombocytopenia
Keywords
Bibliography
511.3 Nonimmune Platelet Destruction
Keywords
511.4 Hemolytic-Uremic Syndrome
511.5 Thrombotic Thrombocytopenic Purpura
Keywords
Bibliography
511.6 Kasabach-Merritt Syndrome
Keywords
Bibliography
511.7 Sequestration
Keywords
511.8 Congenital Thrombocytopenic Syndromes
Keywords
Bibliography
511.9 Neonatal Thrombocytopenia
Keywords
Bibliography
511.10 Thrombocytopenia From Acquired Disorders Causing Decreased Production
Keywords
511.11 Platelet Function Disorders
Keywords
Bibliography
511.12 Acquired Disorders of Platelet Function
Keywords
Bibliography
511.13 Congenital Abnormalities of Platelet Function
Keywords
Other Hereditary Disorders of Platelet Function
Treatment of Patients With Platelet Dysfunction
Bibliography
511.14 Disorders of the Blood Vessels
Keywords
Henoch-Schönlein Purpura
Ehlers-Danlos Syndrome
Other Acquired Disorders
Bibliography
Section 8: The Spleen
Chapter 512: Anatomy and Function of the Spleen
Keywords
Anatomy
Function
Bibliography
Chapter 513: Splenomegaly
Keywords
Clinical Manifestations
Differential Diagnosis
Pseudosplenomegaly
Hypersplenism
Congestive Splenomegaly (Banti Syndrome)
Bibliography
Chapter 514: Hyposplenism, Splenic Trauma, and Splenectomy
Keywords
Hyposplenism
Splenic Trauma
Splenectomy
Bibliography
Section 9: The Lymphatic System
Chapter 515: Anatomy and Function of the Lymphatic System
Keywords
Bibliography
Chapter 516: Abnormalities of Lymphatic Vessels
Keywords
Lymphatic Malformations
Genetics
Treatment
Lymphangiectasia
Lymphedema
Lymphangioleiomyomatosis
Lymphangitis
Bibliography
Chapter 517: Lymphadenopathy
Keywords
Diagnosis
Treatment
517.1 Kikuchi-Fujimoto Disease (Histiocytic Necrotizing Lymphadenitis)
Keywords
Bibliography
517.2 Sinus Histiocytosis With Massive Lymphadenopathy (Rosai-Dorfman Disease)
Keywords
Bibliography
517.3 Castleman Disease
Keywords
Bibliography
Bibliography
Part XXI: Cancer and Benign Tumors
Chapter 518: Epidemiology of Childhood and Adolescent Cancer
Keywords
Influencing the Incidence of Cancer
Bibliography
Chapter 519: Molecular and Cellular Biology of Cancer
Keywords
Genes Involved in Oncogenesis
Syndromes Predisposing to Cancer
Other Factors Associated With Oncogenesis
Viruses
Genomic Imprinting
Telomerase
Bibliography
Chapter 520: Principles of Cancer Diagnosis
Keywords
Signs and Symptoms
Physical Examination
Age-Related Manifestations
Early Detection
Ensuring the Diagnosis
Staging
Bibliography
Chapter 521: Principles of Cancer Treatment
Keywords
Diagnosis and Staging
A Multimodal, Multidisciplinary Approach
Discussing the Treatment Plan With the Patient and Family
Treatments
Chemotherapy
Surgery
Radiation Therapy
Acute Toxic Effects and Supportive Care
Late Adverse Effects
Palliative Care
Bibliography
Chapter 522: The Leukemias
Keywords
522.1 Acute Lymphoblastic Leukemia
Epidemiology
Etiology
Cellular Classification
Clinical Manifestations
Diagnosis
Differential Diagnosis
Treatment
Treatment of Relapse
Supportive Care
Prognosis
Bibliography
522.2 Acute Myelogenous Leukemia
Keywords
Epidemiology
Cellular Classification
Clinical Manifestations
Diagnosis
Prognosis and Treatment
Bibliography
522.3 Down Syndrome and Acute Leukemia and Transient Myeloproliferative Disorder
Keywords
Bibliography
522.4 Chronic Myelogenous Leukemia
Keywords
Bibliography
522.5 Juvenile Myelomonocytic Leukemia
Keywords
Bibliography
522.6 Infant Leukemia
Keywords
Bibliography
Chapter 523: Lymphoma
523.1 Hodgkin Lymphoma
Keywords
Epidemiology
Pathogenesis
Clinical Manifestations
Diagnosis
Treatment
Relapse
Prognosis
Bibliography
523.2 Non-Hodgkin Lymphoma
Keywords
Epidemiology
Pathogenesis
Clinical Manifestations
Laboratory Findings
Treatment
Burkitt Lymphoma and Diffuse Large B-Cell Lymphoma
Lymphoblastic Lymphoma
Anaplastic Large Cell Lymphoma
Relapsed Non-Hodgkin Lymphoma
Complications
Prognosis
Bibliography
523.3 Late Effects in Children and Adolescents With Lymphoma
Keywords
Chapter 524: Brain Tumors in Childhood
Keywords
Etiology
Epidemiology
Pathogenesis
Clinical Manifestations
Diagnosis
Specific Tumors
Astrocytoma
Ependymal Tumors
Choroid Plexus Tumors
Embryonal Tumors
Pineal Parenchymal Tumors
Craniopharyngioma
Germ Cell Tumors
Tumors of the Brainstem
Metastatic Tumors
Complications and Long-Term Management
Bibliography
Chapter 525: Neuroblastoma
Keywords
Epidemiology
Pathology
Pathogenesis
Clinical Manifestations
Diagnosis
Treatment
Bibliography
Chapter 526: Neoplasms of the Kidney
526.1 Wilms Tumor
Keywords
Epidemiology
Etiology: Genetics and Molecular Biology
Clinical Presentation
Diagnosis and Differential Diagnosis
Treatment
Recurrent Disease
Prognosis
Late Effects
526.2 Other Pediatric Renal Tumors
Keywords
Mesoblastic Nephroma
Clear Cell Sarcoma of the Kidney
Rhabdoid Tumor of the Kidney
Renal Cell Carcinoma
Bibliography
Chapter 527: Soft Tissue Sarcomas
Keywords
Rhabdomyosarcoma
Epidemiology
Pathogenesis
Clinical Manifestations
Diagnosis
Treatment
Prognosis
Other Soft Tissue Sarcomas
Bibliography
Chapter 528: Neoplasms of Bone
528.1 Malignant Tumors of Bone
Keywords
Osteosarcoma
Epidemiology
Pathogenesis
Clinical Manifestations
Diagnosis
Treatment
Prognosis
Ewing Sarcoma
Epidemiology
Pathogenesis
Clinical Manifestations
Diagnosis
Treatment
Prognosis
528.2 Benign Tumors and Tumor-Like Processes of Bone
Keywords
Characteristic Lesions of the Tibia
Histiocytosis
Diagnostic Considerations
Vascular Tumors of Bone
Bibliography
Chapter 529: Retinoblastoma
Keywords
Epidemiology
Pathogenesis
Screening
Clinical Manifestations
Diagnosis
Treatment
Prognosis
Bibliography
Chapter 530: Gonadal and Germ Cell Neoplasms
Keywords
Epidemiology
Pathogenesis
Clinical Manifestations and Diagnosis
Treatment
Prognosis
Bibliography
Chapter 531: Neoplasms of the Liver
Keywords
Hepatoblastoma
Epidemiology
Pathogenesis
Clinical Manifestations
Diagnosis
Treatment
Prognosis
Hepatocellular Carcinoma
Epidemiology
Pathogenesis
Clinical Manifestations
Treatment
Bibliography
Chapter 532: Benign Vascular Tumors
532.1 Hemangiomas
Keywords
Clinical Manifestations
Treatment
Bibliography
532.2 Lymphangiomas and Cystic Hygromas
Keywords
Bibliography
Chapter 533: Rare Tumors
533.1 Thyroid Tumors
Keywords
Benign Thyroid Tumors
Malignant Thyroid Tumors
Bibliography
533.2 Nasopharyngeal Carcinoma
Keywords
Bibliography
533.3 Adenocarcinoma of the Colon and Rectum
Keywords
Bibliography
533.4 Adrenal Tumors
Keywords
Bibliography
533.5 Desmoplastic Small Round Cell Tumor
Keywords
Bibliography
Chapter 534: Histiocytosis Syndromes of Childhood
Keywords
Classification and Pathology
534.1 Langerhans Cell Histiocytosis
Keywords
Clinical Manifestations
Treatment and Prognosis
Bibliography
534.2 Hemophagocytic Lymphohistiocytosis
Keywords
Clinical Manifestations
Treatment and Prognosis
Bibliography
534.3 Other Histiocytoses
Keywords
Bibliography
Part XXII: Nephrology
Section 1: Glomerular Disease
Chapter 535: Introduction to Glomerular Diseases
Keywords
535.1 Anatomy of the Glomerulus
Bibliography
535.2 Glomerular Filtration
Bibliography
535.3 Glomerular Diseases
Pathogenesis
Pathology
Bibliography
Section 2: Conditions Particularly Associated With Hematuria
Chapter 536: Clinical Evaluation of the Child With Hematuria
Keywords
Bibliography
Chapter 537: Isolated Glomerular Diseases Associated With Recurrent Gross Hematuria
Keywords
537.1 Immunoglobulin A Nephropathy (Berger Nephropathy)
Pathology and Pathologic Diagnosis
Clinical and Laboratory Manifestations
Prognosis and Treatment
Bibliography
537.2 Alport Syndrome
Genetics
Pathology
Clinical Manifestations
Diagnosis
Prognosis and Treatment
Bibliography
537.3 Thin Basement Membrane Disease
Bibliography
537.4 Acute Poststreptococcal Glomerulonephritis
Etiology and Epidemiology
Pathology
Pathogenesis
Clinical Manifestations
Diagnosis
Complications
Prevention
Treatment
Prognosis
Bibliography
537.5 Membranous Nephropathy
Pathology
Pathogenesis
Clinical Manifestations
Diagnosis
Prognosis and Treatment
Bibliography
537.6 Membranoproliferative Glomerulonephritis
Pathology
Pathogenesis
Clinical Manifestations
Differential Diagnosis
Prognosis and Treatment
Bibliography
537.7 Rapidly Progressive (Crescentic) Glomerulonephritis
Classification
Pathology and Pathogenesis
Clinical Manifestations
Diagnosis and Differential Diagnosis
Prognosis and Treatment
Bibliography
Chapter 538: Multisystem Disease Associated With Hematuria
Keywords
538.1 Chronic Infections
Bibliography
538.2 Glomerulonephritis Associated With Systemic Lupus Erythematosus
Keywords
Pathogenesis and Pathology
Clinical Manifestations
Diagnosis
Treatment
Prognosis
Bibliography
538.3 Henoch-Schönlein Purpura Nephritis
Keywords
Pathogenesis and Pathology
Clinical and Laboratory Manifestations
Prognosis and Treatment
Bibliography
538.4 Goodpasture Disease
Keywords
Pathology
Clinical Manifestations
Diagnosis and Differential Diagnosis
Prognosis and Treatment
Bibliography
538.5 Hemolytic-Uremic Syndrome
Keywords
Etiology
Pathology
Pathogenesis
Clinical Manifestations
Diagnosis and Differential Diagnosis
Prognosis and Treatment
Bibliography
538.6 Toxic Nephropathy
Keywords
Bibliography
538.7 Cortical Necrosis
Keywords
Background
Etiology
Pathogenesis
Clinical Manifestations
Laboratory and Radiologic Findings
Treatment
Prognosis
Bibliography
538.8 Coagulopathies and Thrombocytopenia
Chapter 539: Tubulointerstitial Disease Associated With Hematuria
539.1 Pyelonephritis
539.2 Tubulointerstitial Nephritis
Keywords
Acute Tubulointerstitial Nephritis
Pathogenesis and Pathology
Clinical Manifestations
Diagnosis
Treatment and Prognosis
Chronic Tubulointerstitial Nephritis
Pathogenesis and Pathology
Clinical Manifestations
Diagnosis
Treatment and Prognosis
Bibliography
539.3 Papillary Necrosis
Keywords
Pathogenesis and Pathology
Clinical Manifestations
Diagnosis
Treatment and Prognosis
Bibliography
539.4 Acute Tubular Necrosis
Keywords
Pathogenesis and Pathology
Clinical Manifestations
Diagnosis
Treatment and Prognosis (see also Chapter 550.1)
Bibliography
Chapter 540: Vascular Diseases Associated with Hematuria
540.1 Vascular Abnormalities
Keywords
Bibliography
540.2 Renal Vein Thrombosis
Keywords
Epidemiology
Pathogenesis
Clinical Manifestations
Diagnosis
Differential Diagnosis
Treatment
Prognosis
Bibliography
540.3 Sickle Cell Nephropathy
Keywords
Etiology
Pathology
Clinical Manifestations
Treatment
Prognosis
Bibliography
540.4 Idiopathic Hypercalciuria
Keywords
Diagnosis
Treatment
Bibliography
540.5 Nephrocalcinosis
Chapter 541: Anatomic Abnormalities Associated With Hematuria
541.1 Congenital Anomalies
541.2 Autosomal Recessive Polycystic Kidney Disease
Keywords
Pathology
Pathogenesis
Clinical Manifestations
Diagnosis
Treatment
Prognosis
Bibliography
541.3 Autosomal Dominant Polycystic Kidney Disease
Keywords
Pathology
Pathogenesis
Clinical Presentation
Diagnosis
Treatment and Prognosis
Bibliography
541.4 Trauma
Keywords
Bibliography
541.5 Renal Tumors
Chapter 542: Lower Urinary Tract Causes of Hematuria
542.1 Infectious Causes of Cystitis and Urethritis
542.2 Hemorrhagic Cystitis
Keywords
Bibliography
542.3 Vigorous Exercise
Keywords
Bibliography
Section 3: Conditions Particularly Associated With Proteinuria
Chapter 543: Clinical Evaluation of the Child With Proteinuria
Normal Physiology
Pathophysiology of Proteinuria
Measurement of Urine Protein
Urine Dipstick Measurement of Protein
Urine Protein-to-Creatinine Ratio Measurement
Clinical Considerations
Bibliography
Chapter 544: Conditions Associated With Proteinuria
544.1 Transient Proteinuria
Keywords
Bibliography
544.2 Orthostatic (Postural) Proteinuria
Bibliography
544.3 Fixed Proteinuria
Keywords
Glomerular Proteinuria
Tubular Proteinuria
Bibliography
Tubular Proteinuria
Chapter 545: Nephrotic Syndrome
Keywords
Etiology
Pathogenesis
Role of the Podocyte
Role of the Immune System
Clinical Consequences of Nephrotic Syndrome
Edema
Hyperlipidemia
Increased Susceptibility to Infections
Hypercoagulability
545.1 Idiopathic Nephrotic Syndrome
Keywords
Pathology
Minimal Change Nephrotic Syndrome
Clinical Manifestations
Diagnosis
Recommendations for the Initial Evaluation of Children With Nephrotic Syndrome
Confirming the Diagnosis of Nephrotic Syndrome.
Treatment
Use of Corticosteroids to Treat Minimal Change Nephrotic Syndrome
Treatment of the Initial Episode of Nephrotic Syndrome
Managing the Clinical Sequelae of Nephrotic Syndrome
Edema.
Dyslipidemia.
Infections.
Thromboembolism.
Obesity and Growth.
Relapse of Nephrotic Syndrome.
Steroid Resistance.
Implications of Steroid-Resistant Nephrotic Syndrome.
Alternative Therapies to Corticosteroids in the Treatment of Nephrotic Syndrome.
Immunizations in Children With Nephrotic Syndrome.
Prognosis
Bibliography
545.2 Secondary Nephrotic Syndrome
Keywords
Bibliography
545.3 Congenital Nephrotic Syndrome
Keywords
Bibliography
Bibliography
Section 4: Tubular Disorders
Chapter 546: Tubular Function
Keywords
Sodium
Potassium
Calcium
Phosphate
Magnesium
Acidification and Concentrating Mechanisms
Developmental Considerations
Bibliography
Chapter 547: Renal Tubular Acidosis
Keywords
Normal Urinary Acidification
547.1 Proximal (Type II) Renal Tubular Acidosis
Pathogenesis
Autosomal Recessive Disease
Cystinosis
Lowe Syndrome
Clinical Manifestations of Proximal Renal Tubular Acidosis and Fanconi Syndrome
547.2 Distal (Type I) Renal Tubular Acidosis
Pathogenesis
Clinical Manifestations
547.3 Hyperkalemic (Type IV) Renal Tubular Acidosis
Pathogenesis
Clinical Manifestations
Diagnostic Approach to Renal Tubular Acidosis
Treatment and Prognosis
Bibliography
547.4 Rickets Associated With Renal Tubular Acidosis
Chapter 548: Nephrogenic Diabetes Insipidus
Keywords
Pathogenesis
Clinical Manifestations
Diagnosis
Treatment and Prognosis
Bibliography
Chapter 549: Inherited Tubular Transport Abnormalities
Keywords
549.1 Bartter Syndrome
Pathogenesis
Clinical Manifestations
Diagnosis
Treatment and Prognosis
549.2 Gitelman Syndrome
Pathogenesis
Clinical Manifestations
Diagnosis
Treatment
549.3 Other Inherited Tubular Transport Abnormalities
Bibliography
Chapter 550: Renal Failure
550.1 Acute Kidney Injury
Keywords
Pathogenesis
Clinical Manifestations and Diagnosis
Laboratory Findings
Treatment
Medical Management
Dialysis
Prognosis
Bibliography
550.2 Chronic Kidney Disease
Keywords
Etiology
Pathogenesis
Clinical Manifestations
Laboratory Findings
Treatment and Management
Nutrition
CKD Mineral and Bone Disorder (CKD-MBD)
Fluid and Electrolyte Management
Linear Growth
Anemia
Hypertension and Proteinuria
Immunizations
Adjustment in Drug Dose
Progression of Disease
Bibliography
550.3 End-Stage Renal Disease
Keywords
Bibliography
Chapter 551: Renal Transplantation
Keywords
Incidence and Etiology of End-Stage Renal Disease
Indications for Renal Transplantation
Characteristics of Kidney Donors and Recipients
Evaluation and Preparing for Kidney Transplantation
Immunosuppression
Induction Therapy
T-Cell Antibodies
Interleukin-2 Receptor Antibodies
Other Induction Therapies
Maintenance Immunosuppression
Calcineurin Inhibitors
Antiproliferative Agents
Mammalian Target of Rapamycin (mTOR) Inhibitors
Corticosteroids
Other Agents
Fluid Management in Infants and Small Children Following Kidney Transplantation
Rejection of Kidney Transplant
Kidney Biopsy
Graft Survival of Kidneys
Complications of Immunosuppression
Long-Term Outcome of Kidney Transplantation
Bibliography
Part XXIII: Urologic Disorders in Infants and Children
Chapter 552: Congenital Anomalies and Dysgenesis of the Kidneys
Keywords
Embryonic and Fetal Development
Renal Agenesis
Renal Dysgenesis: Dysplasia, Hypoplasia, and Cystic Anomalies
Renal Cysts in Children
Anomalies in Shape and Position
Associated Physical Findings
Bibliography
Chapter 553: Urinary Tract Infections
Keywords
Prevalence and Etiology
Clinical Manifestations and Classification
Pyelonephritis
Cystitis
Pathogenesis and Pathology
Diagnosis
Imaging Findings
Treatment
Acute Lobar Nephronia, Renal Abscess, and Perinephric Abscess
Other Potential Treatment or Prevention Options
Imaging Studies in Children With a Febrile UTI
Prevention of Recurrences
Bibliography
Chapter 554: Vesicoureteral Reflux
Keywords
Classification
Clinical Manifestations
Diagnosis
Natural History
Treatment
Observation
Antimicrobial Prophylaxis
Surgery
Current Vesicoureteral Reflux Guidelines
Bibliography
Chapter 555: Obstruction of the Urinary Tract
Keywords
Etiology
Clinical Manifestations
Diagnosis
Physical Findings
Imaging Studies
Renal Ultrasound
Voiding Cystourethrogram
Radioisotope Studies
Magnetic Resonance Urography
Computed Tomography
Ancillary Studies
Specific Types of Urinary Tract Obstruction and Their Treatment
Hydrocalycosis
Ureteropelvic Junction Obstruction
Midureteral Obstruction
Ectopic Ureter
Ureterocele
Megaureter
Prune-Belly Syndrome
Bladder Neck Obstruction
Posterior Urethral Valves
Urethral Atresia
Urethral Hypoplasia
Urethral Stricture
Anterior Urethral Valves and Urethral Diverticula in the Male
Male Urethral Meatal Stenosis
Bibliography
Chapter 556: Anomalies of the Bladder
Keywords
Bladder Exstrophy
Clinical Manifestations
Treatment
Long-Term Prognosis
Other Exstrophy Anomalies
Bladder Diverticula
Urachal Anomalies
Bibliography
Chapter 557: Neuropathic Bladder
Keywords
Neural Tube Defects
Clinical Manifestations and Diagnosis
Renal Damage
Urinary Incontinence
Complications of Augmentation Cystoplasty
Urinary Tract Infection
Metabolic Acidosis
Spontaneous Perforation
Bladder Calculi
Malignant Neoplasm
Future Management
Associated Disorders
Constipation
Latex Allergy
Occult Spinal Dysraphism
Sacral Agenesis
Imperforate Anus
Cerebral Palsy
Bibliography
Chapter 558: Enuresis and Voiding Dysfunction
Keywords
Normal Voiding and Toilet Training
Diurnal Incontinence
Overactive Bladder (Diurnal Urge Syndrome)
Nonneurogenic Neurogenic Bladder (Hinman Syndrome)
Infrequent Voiding (Underactive Bladder)
Vaginal Voiding
Other Causes of Incontinence in Females
Voiding Disorders Without Incontinence
Nocturnal Enuresis
Epidemiology
Pathogenesis
Clinical Manifestations and Diagnosis
Treatment
Bibliography
Chapter 559: Anomalies of the Penis and Urethra
Keywords
Hypospadias
Clinical Manifestations
Treatment
Chordee Without Hypospadias
Phimosis and Paraphimosis
Circumcision
Penile Torsion
Inconspicuous Penis
Micropenis
Priapism
Other Penile Anomalies
Meatal Stenosis
Other Male Urethral Anomalies
Urethral Prolapse (Female)
Other Female Urethral Lesions
Bibliography
Chapter 560: Disorders and Anomalies of the Scrotal Contents
Keywords
Undescended Testis (Cryptorchidism)
Epidemiology
Pathogenesis
Clinical Manifestations
Treatment
Scrotal Swelling
Clinical Manifestations
Laboratory Findings and Diagnosis
Testicular (Spermatic Cord) Torsion
Etiology
Diagnosis
Treatment
Torsion of the Appendix Testis/Epididymis
Epididymitis
Varicocele
Spermatocele
Hydrocele
Etiology
Diagnosis
Treatment
Inguinal Hernia
Testicular Microlithiasis
Testicular Tumor
Bibliography
Chapter 561: Trauma to the Genitourinary Tract
Keywords
Etiology
Diagnosis
Treatment
Bibliography
Chapter 562: Urinary Lithiasis
Keywords
Stone Formation
Clinical Manifestations
Diagnosis
Metabolic Evaluation
Pathogenesis of Specific Renal Calculi
Calcium Oxalate and Calcium Phosphate Calculi
Cystine Calculi
Struvite Calculi
Uric Acid Calculi
Indinavir Calculi
Nephrocalcinosis
Treatment
Stone Prevention
Bibliography
Part XXIV: Gynecologic Problems of Childhood
Chapter 563: Gynecologic History and Physical Examination
Keywords
History
Gynecologic Examination
Neonates
Infants and Prepubertal Girls
Indications for Genital Examination
Preparation
Positioning
Documentation
Adolescents
Pelvic Examination
Bibliography
Chapter 564: Vulvovaginitis
Keywords
Etiology
Epidemiology
Clinical Manifestations
Diaper Dermatitis
Physiologic Leukorrhea
Labial Agglutination
Genital Ulcers
Dermatoses
Diagnosis and Differential Diagnosis
Treatment and Prevention
Bibliography
Chapter 565: Vaginal Bleeding in the Prepubertal Child
Keywords
Bibliography
Chapter 566: Breast Concerns
Keywords
Breast Development
Breast Evaluation
Breast Self-Awareness
Abnormal Development
Neonatal Breast Abnormalities
Precocious Puberty
Amastia
Polymastia and Polythelia
Breast Asymmetry and Hypomastia
Juvenile or Virginal Hypertrophy
Infections
Trauma and Inflammation
Nipple Discharge
Galactorrhea
Bloody Discharge
Mastalgia
Breast Masses
Peripubertal Masses
Common Adolescent Breast Masses
Malignant Masses
Imaging of Breast Masses
Recommendations for Daughters of Women With Breast Cancer
Risk Reduction
Screening Procedures
Genetic Testing in Children
Cosmetic Surgery
Bibliography
Chapter 567: Polycystic Ovary Syndrome and Hirsutism
Keywords
Polycystic Ovary Syndrome
Etiology and Definition
Pathology, Pathogenesis, and Genetics
Clinical Manifestations
Laboratory Findings, Diagnosis, and Differential Diagnosis
Complications and Long-Term Outlook
Treatment
Lifestyle Changes
Hormonal Contraceptives
Metformin
Antiandrogens
Hirsutism
Bibliography
Chapter 568: Gynecologic Neoplasms and Adolescent Prevention Methods for Human Papillomavirus
Keywords
Overview of Gynecologic Malignancies in Children and Adolescents
Impact of Cancer Therapy on Fertility
Ovarian Neoplasms
Neonatal and Pediatric Ovarian Cysts
Functional Cysts
Teratomas
Cystadenomas
Endometriomas
Pelvic Inflammatory Disease and Tuboovarian Abscess
Adnexal Torsion
Ovarian Malignancies
Uterine Malignancies
Vaginal Malignancies
Vulvar Malignancies
Cervical Malignancies and Their Prevention
Bibliography
Chapter 569: Vulvovaginal and Müllerian Anomalies
Keywords
Embryology
Epidemiology
Clinical Manifestations
Laboratory Findings
Uterine Anomalies
Septate Uterus
Bicornuate Uterus
Unicornuate Uterus and Rudimentary Horns
Uterine Didelphys
Arcuate Uterus
Treatment
Vaginal Anomalies
Abnormalities of the Hymen
Congenital Absence of the Vagina and Mayer-Rokitansky-Küster-Hauser Syndrome
Longitudinal Vaginal Septa
Transverse Vaginal Septa (Vertical Fusion Defects)
Treatment
Cervical Anomalies
Vulvar and Other Anomalies
Complete Vulvar Duplication
Labial Asymmetry and Hypertrophy
Clitoral Abnormalities
Cloacal Anomalies
Ductal Remnants
Bibliography
Chapter 570: Gynecologic Care for Girls With Special Needs
Keywords
Sexuality and Sexual Education
Abuse
Pelvic Examination
Menstruation
Treatment of Menstruation
Nonhormonal Methods
Estrogen-Containing Methods
Oral Contraceptives
Contraceptive Ring
Contraceptive Patch
Estrogen Use, Venous Thromboembolism, and Mobility Issues
Progestin-Only Methods
Intramuscular Medroxyprogesterone Acetate
Oral Progestins
Progesterone Intrauterine Device
Implants
Hormones and Antiepileptic Drugs
Surgical Methods
Contraception
Bibliography
Chapter 571: Female Genital Mutilation
Keywords
Background
Complications
Clinical Management of FGM
Bibliography
Part XXV: The Endocrine System
Section 1: Disorders of the Hypothalamus and Pituitary Gland
Chapter 572: Hormones of the Hypothalamus and Pituitary
Keywords
Anatomy
Embryology
Vascular Supply
Anterior Pituitary Cell Types
Growth Hormone
Prolactin
Thyroid-Stimulating Hormone
Adrenocorticotropic Hormone
Luteinizing Hormone and Follicle-Stimulating Hormone
Posterior Pituitary Cell Types
Antidiuretic Hormone
Oxytocin
Bibliography
Chapter 573: Hypopituitarism
Keywords
Multiple Pituitary Hormone Deficiency
Genetic Forms (Table 573.1)
PROP1
POU1F1 (PIT1)
HESX1
LHX3 and LHX4
Other Congenital Forms
Acquired Forms
Isolated Growth Hormone Deficiency and Insensitivity
Genetic Forms of Growth Hormone Deficiency
Growth Hormone–Releasing Hormone Receptor
GH1
X-Linked Isolated Growth Hormone Deficiency
Acquired Forms
Growth Hormone Insensitivity
Abnormalities of the Growth Hormone Receptor
Postreceptor Forms of Growth Hormone Insensitivity
IGF-1 Gene Abnormalities
Insulin-Like Growth Factor–Binding Protein Abnormalities
IGF-1 Receptor Gene Abnormalities
Clinical Manifestations
Congenital Hypopituitarism
Acquired Hypopituitarism
Laboratory Findings
Radiologic Findings
Differential Diagnosis
Constitutional Growth Delay
Treatment
Complications and Adverse Effects of Growth Hormone Treatment
Bibliography
Chapter 574: Diabetes Insipidus
Keywords
Physiology of Water Balance
Approach to the Patient With Polyuria, Polydipsia, and Hypernatremia
Causes of Hypernatremia
Central Diabetes Insipidus
Nephrogenic Diabetes Insipidus
Treatment of Central Diabetes Insipidus
Fluid Therapy
Vasopressin Analogs
Aqueous Vasopressin
Treatment of Nephrogenic Diabetes Insipidus
Bibliography
Chapter 575: Other Abnormalities of Arginine Vasopressin Metabolism and Action
Keywords
Causes of Hyponatremia
Syndrome of Inappropriate Antidiuretic Hormone Secretion
Nephrogenic Syndrome of Inappropriate Antidiuresis
Systemic Dehydration
Primary Salt Loss
Decreased Effective Plasma Volume
Primary Polydipsia (Increased Water Ingestion)
Decreased Free Water Clearance
Cerebral Salt Wasting
Runner’s Hyponatremia
Pseudohyponatremia and Other Causes of Hyponatremia
Treatment
Emergency Treatment of Hyponatremia
Treatment of Syndrome of Inappropriate Antidiuretic Hormone
Treatment of Cerebral Salt Wasting
Bibliography
Chapter 576: Hyperpituitarism, Tall Stature, and Overgrowth Syndromes
Keywords
Hyperpituitarism
Tall Stature
Differential Diagnosis of Tall Stature
Overgrowth in the Fetus and Neonate
Overgrowth in Childhood or Adolescence
Sotos Syndrome (Cerebral Gigantism)
Treatment of Normal Variant Tall Stature
Excess Growth Hormone Secretion and Pituitary Gigantism
Diagnosis
Treatment
Hypersecretion of Other Pituitary Hormones
Prolactinoma
Corticotropinoma
Bibliography
Chapter 577: Physiology of Puberty
Keywords
Bibliography
Chapter 578: Disorders of Pubertal Development
578.1 Central Precocious Puberty
Keywords
Clinical Manifestations
Laboratory Findings
Differential Diagnosis
Treatment
Bibliography
578.2 Precocious Puberty Resulting from Organic Brain Lesions
Keywords
Etiology
Clinical Manifestations
Treatment
Bibliography
578.3 Precocious Puberty Following Irradiation of the Brain
Keywords
Treatment
Bibliography
578.4 Syndrome of Precocious Puberty and Hypothyroidism
Keywords
578.5 Chorionic Gonadotropin-Secreting Tumors
Keywords
Hepatic Tumors
Intracranial Tumors
Tumors in Other Locations
Peripheral Precocious Puberty
Bibliography
578.6 McCune-Albright Syndrome
Keywords
Extragonadal Manifestations
Bibliography
578.7 Familial Male Gonadotropin-Independent Precocious Puberty
Keywords
Treatment
Bibliography
578.8 Incomplete (Partial) Precocious Development
Keywords
Premature Thelarche
Premature Pubarche (Adrenarche)
Premature Menarche
Bibliography
578.9 Medicational Precocity
Keywords
578.10 Delayed or Absent Puberty*
Keywords
Differential Diagnosis
Constitutional Delay of Growth and Puberty
Hypogonadotropic Hypogonadism
Kallmann Syndrome
Hypergonadotropic Hypogonadism: Males
Klinefelter Syndrome
Hypergonadotropic Hypogonadism: Females
Turner Syndrome
Females with Delayed or Absent Adrenarche
Diagnostic Approach to Delayed Puberty
Treatment of Delayed Puberty
Bibliography
Section 2: Disorders of the Thyroid Gland
Chapter 579: Thyroid Development and Physiology
Keywords
Fetal Development
Thyroid Physiology
Thyroid Regulation
579.1 Thyroid Hormone Studies
Serum Thyroid Hormones
Fetal and Newborn Thyroid
Serum Thyroxine-Binding Globulin
Radionuclide Studies
Thyroid Ultrasonography
Bibliography
Bibliography
Chapter 580: Disorders of Thyroxine-Binding Globulin
Keywords
Bibliography
Chapter 581: Hypothyroidism
Keywords
Congenital Hypothyroidism
Epidemiology
Etiology
Primary Hypothyroidism
Thyroid Dysgenesis.
Defects in Thyroid Hormone Synthesis (Dyshormonogenesis).
Defective Iodide Transport.
Defects of Iodine Organification.
Defects of Thyroglobulin Synthesis.
Defects in Deiodination.
Defects in Thyroid Hormone Transport.
Thyrotropin Receptor–Blocking Antibodies.
Radioiodine Administration.
Iodine Exposure.
Iodine Deficiency (Endemic Goiter).
Central (Secondary) Hypothyroidism
Thyrotropin and Thyrotropin-Releasing Hormone Deficiency.
Thyroid Function in Preterm and Low Birthweight Infants
Clinical Manifestations
Laboratory Findings
Treatment
Prognosis
Acquired Hypothyroidism
Epidemiology
Etiology
Clinical Manifestations
Diagnostic Studies
Treatment and Prognosis
Bibliography
Congenital Hypothyroidism
Acquired Hypothyroidism
Chapter 582: Thyroiditis
Keywords
Thyroiditis With Pain
Thyroiditis Without Pain
Chronic Lymphocytic Thyroiditis (Hashimoto Thyroiditis, Autoimmune Thyroiditis)
Etiology
Clinical Manifestations
Laboratory Findings
Treatment
Other Causes of Thyroiditis
Bibliography
Chapter 583: Goiter
583.1 Congenital Goiter
Keywords
Bibliography
583.2 Intratracheal Goiter
Keywords
583.3 Endemic Goiter and Cretinism
Keywords
Etiology
Clinical Manifestations
Pathogenesis
Treatment
Bibliography
583.4 Acquired Goiter
Keywords
Iodide Goiter
Iodine-Deficiency Goiter
Goitrogens
Simple Goiter (Colloid Goiter)
Multinodular Goiter
Toxic Goiter (Hyperthyroidism)
Bibliography
Chapter 584: Thyrotoxicosis
Keywords
584.1 Graves Disease
Keywords
Epidemiology
Etiology
Clinical Manifestations
Laboratory Findings
Differential Diagnosis
Treatment
Antithyroid Drugs
Definitive Therapy
Bibliography
584.2 Congenital Hyperthyroidism
Keywords
Etiology and Pathogenesis
Clinical Manifestations
Treatment
Prognosis
Resistance to Thyroid Hormone
Bibliography
Chapter 585: Carcinoma of the Thyroid
Keywords
Epidemiology
Pathogenesis
Clinical Manifestations
Diagnosis
Treatment
Prognosis
Medullary Thyroid Carcinoma
585.1 Thyroid Nodules
Bibliography
Bibliography
Chapter 586: Autoimmune Polyglandular Syndromes
Keywords
Monogenic Autoimmune Polyglandular Syndromes
Autoimmune Polyglandular Syndrome Type 1
Immune Dysregulation- Polyendocrinopathy-Enteropathy X-Linked
Other Monogenic Immune Dysregulation Disorders
Polygenic Autoimmune Polyglandular Syndrome (APS-2)
Chromosomal Abnormalities Associated With Autoimmune Polyglandular Syndrome
Nongenetic Autoimmune Causes of Multiple Endocrinopathy
Bibliography
Chapter 587: Multiple Endocrine Neoplasia Syndromes
Keywords
Multiple Endocrine Neoplasia Type 1
Multiple Endocrine Neoplasia Type 2
Multiple Endocrine Neoplasia Type 2A
Multiple Endocrine Neoplasia Type 2B
Familial Medullary Thyroid Carcinoma
Management of Multiple Endocrine Neoplasia Type 2
Bibliography
Section 3: Disorders of the Parathyroid Gland
Chapter 588: Hormones and Peptides of Calcium Homeostasis and Bone Metabolism
Keywords
Parathyroid Hormone
Parathyroid Hormone–Related Peptide
Vitamin D
Calcitonin
Bibliography
Chapter 589: Hypoparathyroidism
Keywords
Etiology
Aplasia or Hypoplasia of the Parathyroid Glands
X-Linked Recessive Hypoparathyroidism
Autosomal Recessive Hypoparathyroidism With Dysmorphic Features
Hypoparathyroidism, Sensorineural Deafness, and Renal Anomaly Syndrome
Suppression of Neonatal Parathyroid Hormone Secretion Because of Maternal Hyperparathyroidism
Autosomal Dominant Hypoparathyroidism
Hypoparathyroidism Associated With Mitochondrial Disorders
Surgical Hypoparathyroidism
Autoimmune Hypoparathyroidism
Idiopathic Hypoparathyroidism
Clinical Manifestations
Laboratory Findings
Treatment
Differential Diagnosis
Bibliography
Chapter 590: Pseudohypoparathyroidism
Keywords
Type Ia
Type Ia With Precocious Puberty
Type Ib
Acrodysostosis With Hormone Resistance
Bibliography
Chapter 591: Hyperparathyroidism
Keywords
Etiology
Clinical Manifestations
Laboratory Findings
Differential Diagnosis
Treatment
Prognosis
591.1 Other Causes of Hypercalcemia
Familial Hypocalciuric Hypercalcemia (Familial Benign Hypercalcemia)
Granulomatous Diseases
Hypercalcemia of Malignancy
Miscellaneous Causes of Hypercalcemia
Bibliography
Section 4: Disorders of the Adrenal Gland
Chapter 592: Physiology of the Adrenal Gland
592.1 Histology and Embryology
Keywords
Bibliography
592.2 Adrenal Steroid Biosynthesis
Keywords
Zona Glomerulosa
Zona Fasciculata
Zona Reticularis
Fetoplacental Unit
Bibliography
592.3 Regulation of the Adrenal Cortex
Keywords
Regulation of Cortisol Secretion
Regulation of Aldosterone Secretion
Regulation of Adrenal Androgen Secretion
Bibliography
592.4 Adrenal Steroid Hormone Actions
Keywords
Actions of Glucocorticoids
Metabolic Effects
Circulatory and Renal Effects
Growth
Immunologic Effects
Effects on Skin, Bone, and Calcium
Central Nervous System Effects
Actions of Mineralocorticoids
Actions of the Adrenal Androgens
Synthetic Corticosteroids
Bibliography
592.5 Adrenal Medulla
Keywords
Bibliography
Chapter 593: Adrenocortical Insufficiency
Keywords
593.1 Primary Adrenal Insufficiency
Keywords
Inherited Etiologies
Inborn Defects of Steroidogenesis
Adrenal Hypoplasia Congenita
Other Genetic Causes of Adrenal Hypoplasia
Adrenoleukodystrophy
Familial Glucocorticoid Deficiency
Type I Autoimmune Polyendocrinopathy Syndrome
Disorders of Cholesterol Synthesis and Metabolism
Corticosteroid-Binding Globulin Deficiency and Decreased Cortisol-Binding Affinity
Acquired Etiologies
Autoimmune Addison Disease
Infection
Drugs
Hemorrhage Into Adrenal Glands
Clinical Manifestations
Laboratory Findings
Differential Diagnosis
Treatment
Bibliography
593.2 Secondary and Tertiary Adrenal Insufficiency
Keywords
Etiology
Abrupt Cessation of Administration of Corticosteroids
Corticotropin (Adrenocorticotropic Hormone) Deficiency
Clinical Presentation
Laboratory Findings
Treatment
Bibliography
593.3 Adrenal Insufficiency in the Critical Care Setting
Keywords
Etiology
Clinical Manifestations
Laboratory Findings
Treatment
Bibliography
593.4 Altered End-Organ Sensitivity to Corticosteroids
Keywords
Generalized Glucocorticoid Resistance
Etiology
Clinical Manifestations
Laboratory Findings
Differential Diagnosis
Treatment
Cortisone Reductase Deficiency
Etiology
Clinical Manifestations
Laboratory Findings
Differential Diagnosis
Treatment
Altered End-Organ Sensitivity to Mineralocorticoids
Pseudohypoaldosteronism
Etiology
Clinical Manifestations
Laboratory Findings
Differential Diagnosis
Treatment
Apparent Mineralocorticoid Excess
Etiology
Clinical Manifestations
Laboratory Findings
Differential Diagnosis
Treatment
Liddle Syndrome
Etiology
Clinical Manifestations, Laboratory Findings, and Differential Diagnosis
Treatment
Bibliography
Generalized Glucocorticoid Resistance
Cortisone Reductase Deficiency
Altered End-Organ Sensitivity to Mineralocorticoids
Apparent Mineralocorticoid Excess
Liddle Syndrome
Chapter 594: Congenital Adrenal Hyperplasia and Related Disorders
594.1 Congenital Adrenal Hyperplasia Caused by 21-Hydroxylase Deficiency
Keywords
Etiology
Epidemiology
Genetics
Pathogenesis and Clinical Manifestations
Aldosterone and Cortisol Deficiency
Prenatal Androgen Excess
Postnatal Androgen Excess
Adrenomedullary Dysfunction
Laboratory Findings
Differential Diagnosis
Prenatal Diagnosis
Newborn Screening
Treatment
Glucocorticoid Replacement
Mineralocorticoid Replacement
Surgical Management of Ambiguous Genitals
Prenatal Treatment
Bibliography
594.2 Congenital Adrenal Hyperplasia Caused by 11β-Hydroxylase Deficiency
Keywords
Etiology
Epidemiology
Clinical Manifestations
Laboratory Findings
Treatment
Bibliography
594.3 Congenital Adrenal Hyperplasia Caused by 3β-Hydroxysteroid Dehydrogenase Deficiency
Keywords
Etiology
Clinical Manifestations
Laboratory Findings
Differential Diagnosis
Treatment
Bibliography
594.4 Congenital Adrenal Hyperplasia Caused by 17-Hydroxylase Deficiency
Keywords
Etiology
Clinical Manifestations and Laboratory Findings
Treatment
Bibliography
594.5 Lipoid Adrenal Hyperplasia
Keywords
Etiology
Clinical Manifestations
Laboratory Findings
Treatment
Bibliography
594.6 Deficiency of P450 Oxidoreductase (Antley-Bixler Syndrome)
Keywords
Etiology, Pathogenesis, and Clinical Manifestations
Epidemiology
Laboratory Findings
Differential Diagnosis
Bibliography
594.7 Aldosterone Synthase Deficiency
Keywords
Etiology
Clinical Manifestations
Laboratory Findings
Differential Diagnosis
Treatment
Bibliography
594.8 Glucocorticoid-Suppressible Hyperaldosteronism
Keywords
Etiology
Clinical Manifestations
Laboratory Findings
Differential Diagnosis
Treatment
Genetic Counseling
Bibliography
Chapter 595: Adrenocortical Tumors and Masses
Epidemiology
595.1 Adrenocortical Carcinoma
Keywords
Etiology
Clinical Manifestations
Pathologic Findings
Differential Diagnosis
Treatment
595.2 Adrenal Incidentaloma
595.3 Adrenal Calcification
Keywords
Bibliography
Chapter 596: Virilizing and Feminizing Adrenal Tumors
Keywords
Clinical Manifestations
Laboratory Findings
Differential Diagnosis
Treatment
Chapter 597: Cushing Syndrome
Keywords
Etiology
Clinical Manifestations
Laboratory Findings
Differential Diagnosis
Treatment
Bibliography
Chapter 598: Primary Aldosteronism
Keywords
Etiology
Epidemiology
Clinical Manifestations
Laboratory Findings
Differential Diagnosis
Treatment
Bibliography
Chapter 599: Pheochromocytoma
Keywords
Etiology
Clinical Manifestations
Laboratory Findings
Differential Diagnosis
Treatment
Bibliography
Section 5: Disorders of the Gonads
Chapter 600: Development and Function of the Gonads
Keywords
Genetic Control of Embryonic Gonadal Differentiation
Function of the Testes
Function of the Ovaries
Diagnostic Testing
Therapeutic Use of Sex Steroids
Bibliography
Chapter 601: Hypofunction of the Testes
601.1 Hypergonadotropic Hypogonadism in the Male (Primary Hypogonadism)
Keywords
Etiology
Congenital Anorchia or Testicular Regression Syndrome
Chemotherapy and Radiation-Induced Hypogonadism
Sertoli Cell–Only Syndrome
Other Causes of Testicular Hypofunction
Testicular Dysgenesis Syndrome
Clinical Manifestations
Diagnosis
Noonan Syndrome
Etiology
Clinical Manifestations
Treatment
Klinefelter Syndrome
Etiology
Clinical Manifestations
Klinefelter Variants and Other Poly-X Syndromes
Laboratory Findings
Management
XX Males
45,X Males
47,XXX Males
Bibliography
601.2 Hypogonadotropic Hypogonadism in the Male (Secondary Hypogonadism)
Keywords
Etiology
Isolated Gonadotropin Deficiency
Hypogonadotropic Hypogonadism Without Anosmia
Other Disorders With Hypogonadotropic Hypogonadism
Hypogonadotropic Hypogonadism Associated With Other Pituitary Hormone Deficiencies
Diagnosis
Treatment
Bibliography
Chapter 602: Pseudoprecocity Resulting From Tumors of the Testes
Keywords
Bibliography
Chapter 603: Gynecomastia
Keywords
Physiologic Forms of Gynecomastia
Neonatal Gynecomastia
Pubertal Gynecomastia
Pathologic Gynecomastia
Evaluation of Gynecomastia
Treatment
Bibliography
Chapter 604: Hypofunction of the Ovaries
604.1 Hypergonadotropic Hypogonadism in the Female (Primary Hypogonadism)
Keywords
Turner Syndrome
Pathogenesis
Clinical Manifestations
Laboratory Findings
Treatment
XX Gonadal Dysgenesis
45,X/46,XY Gonadal Dysgenesis
XXX, XXXX, and XXXXX Females
XXX Females
XXXX and XXXXX Females
Noonan Syndrome
Other Ovarian Defects
Bibliography
604.2 Hypogonadotropic Hypogonadism in the Female (Secondary Hypogonadism)
Keywords
Etiology
Hypopituitarism
Isolated Deficiency of Gonadotropins
Diagnosis
Bibliography
Chapter 605: Pseudoprecocity Resulting From Lesions of the Ovary
Keywords
Estrogenic Lesions of the Ovary
Juvenile Granulosa Cell Tumor
Clinical Manifestations and Diagnosis
Treatment and Prognosis
Follicular Cyst
Androgenic Lesions of the Ovary
Bibliography
Chapter 606: Disorders of Sex Development
Sex Differentiation
Diagnostic Approach to the Patient With Atypical or Ambiguous Genitalia
606.1 46,XX DSD
Keywords
Congenital Adrenal Hyperplasia
Aromatase Deficiency
Cortisol Resistance Due to Glucocorticoid Receptor Gene Mutation
P450 Oxidoreductase Deficiency
Virilizing Maternal Tumors
Exposure to Androgenic Drugs by Women During Pregnancy
SF-1 Mutations
46,XX Testicular DSD
46,XX Gonadal Dysgenesis
Undetermined/Unknown
Bibliography
606.2 46,XY DSD
Keywords
Defects in Testicular Differentiation
Wilms Tumor Suppressor Gene (WT1) Mutations: Denys-Drash, Frasier, and WAGR Syndromes
Campomelic Syndrome
SF-1 (Also Known as Ad4BP or NR5A1) Defects and 46,XY DSD
XY Pure Gonadal Dysgenesis (Swyer Syndrome)
XY Gonadal Agenesis Syndrome (Embryonic Testicular Regression Syndrome)
Defiency of Testicular Hormone Production
Leydig Cell Aplasia
Lipoid Adrenal Hyperplasia
3β-Hydroxysteroid Dehydrogenase Deficiency
Deficiency of 17-Hydroxylase/17,20-Lyase
Deficiency of 17-Ketosteroid Reductase
Persistent Müllerian Duct Syndrome
Defects in Androgen Action
Dihydrotestosterone Deficiency
Androgen Insensitivity Syndromes
Clinical Manifestations
Diagnosis
Treatment and Prognosis
Undetermined Causes
Bibliography
606.3 Ovotesticular DSD
Keywords
Diagnosis and Management of Disorders of Sex Development
Bibliography
Bibliography
Section 6: Diabetes Mellitus in Children
Chapter 607: Diabetes Mellitus
607.1 Classification of Diabetes Mellitus
Keywords
Type 1 Diabetes Mellitus
Type 2 Diabetes Mellitus
Other Specific Types of Diabetes
Monogenic Diabetes
Other Etiologies of Diabetes
Prediabetes
607.2 Type 1 Diabetes Mellitus (Immune Mediated)
Keywords
Epidemiology
Genetics
Monogenic Type 1 Diabetes Mellitus
Genes Altering the Risk of Autoimmune Type 1 Diabetes Mellitus
Major Histocompatibility Complex/Human Leukocyte Antigen Encoded Susceptibility to Type 1 Diabetes Mellitus
Role of Aspartate at Position 57 in DQB1
Role of Human Leukocyte Antigen Class I
Non-MHC/HLA Genes Associated With T1DM Risk
Environmental Factors
Viral Infections
Congenital Rubella Syndrome
Enteroviruses
Mumps Virus
The Hygiene Hypothesis: Possible Protective Role of Infections
Gastrointestinal Microbiome
Diet
Psychologic Stress
Pathogenesis and Natural History of Type 1 Diabetes Mellitus
Initiation of Autoimmunity
Preclinical Autoimmunity With Progressive Loss of β-Cell Function
Role of Autoantibodies
Role of Genetics in Disease Progression
Role of Environmental Factors
Onset of Clinical Disease
Prediction and Prevention
Pathophysiology
Clinical Manifestations
Diagnosis
Treatment
Treatment of Diabetic Ketoacidosis
Hyperglycemia and Dehydration
Catabolic Losses
Ketoacid Accumulation
Diabetic Ketoacidosis Protocol
Cerebral Edema
Nonketotic Hyperosmolar Coma
Initiation of Subcutaneous Insulin Therapy
Insulin Therapy
Insulin Pump Therapy
Continuous Glucose Monitoring Systems
Closed-Loop Systems
Adjunct Pharmacotherapy
Basic and Advanced Diabetes Education
Nutritional Management
Monitoring
Glycosylated Hemoglobin (HbA1c)
Exercise
Benefits of Improved Glycemic Control
Hypoglycemic Reactions
Dawn Phenomenon and Somogyi Phenomenon
Behavioral/Psychologic Aspects and Eating Disorders
Cognitive Function
Coping Styles
Nonadherence
Anxiety and Depression
Fear of Self-Injecting and Self-Testing
Eating Disorders
Management During Infections
Management During Surgery
Long-Term Complications: Relation to Glycemic Control
Diabetic Retinopathy
Diabetic Nephropathy
Diabetic Neuropathy
Skeletal Effects of Type 1 Diabetes Mellitus
Other Complications
Prognosis
Pancreas and Islet Transplantation and Regeneration
607.3 Type 2 Diabetes Mellitus
Natural History
Epidemiology
Genetics
Epigenetics and Fetal Programming
Environmental and Lifestyle-Related Risk Factors
Clinical Features
Treatment
Complications
Prevention
607.4 Other Specific Types of Diabetes
Keywords
Genetic Defects of β-Cell Function
Transient Neonatal Diabetes Mellitus
Permanent Neonatal Diabetes Mellitus
Maturity-Onset Diabetes of Youth
MODY2
MODY3
Less Common Forms of Monogenic Diabetes
Mitochondrial Gene Defects
Abnormalities of the Insulin Gene
Genetic Defects of Insulin Action
Donohue Syndrome
Rabson-Mendenhall Syndrome
Lipoatrophic Diabetes
Stiff-Person Syndrome
Systemic Lupus Erythematosus
Cystic Fibrosis–Related Diabetes
Endocrinopathies
Drugs
Genetic Syndromes Associated With Diabetes Mellitus
Autoimmune Diseases Associated With T1DM
IPEX Syndrome
Autoimmune Polyendocrine Syndromes
Bibliography
Epidemiology, Etiology, Pathology, Classification, and Prevention
Genetics
Diabetic Ketoacidosis
Management of Type 1 Diabetes in Children
Long-Term Outcome of Childhood Diabetes: Relation of Control to Development of Complications
Type 2 Diabetes
Other Diseases and Syndromes Associated With Diabetes
Diabetes of the Newborn
Hypoglycemia and Diabetes
Pancreas and Islet Transplantation
Part XXVI: The Nervous System
Chapter 608: Neurologic Evaluation
Keywords
History
Neurologic Examination
Mental State
Head
Cranial Nerves
Olfactory Nerve (Cranial Nerve I)
Optic Nerve (Cranial Nerve II; see also Part XXVIII)
Vision
Oculomotor (Cranial Nerve III), Trochlear (Cranial Nerve IV), and Abducens (Cranial Nerve VI) Nerves
Trigeminal Nerve (Cranial Nerve V)
Facial Nerve (Cranial Nerve VII)
Vestibulocochlear Nerve (Cranial Nerve VIII)
Glossopharyngeal Nerve (Cranial Nerve IX)
Vagus Nerve (Cranial Nerve X)
Accessory Nerve (Cranial Nerve XI)
Hypoglossal Nerve (Cranial Nerve XII)
Motor Examination
Bulk
Tone
Strength
Involuntary Movements
Sensory Examination
Reflexes
Deep Tendon Reflexes and the Plantar Response
Primitive Reflexes
Coordination
Station and Gait
General Examination
Special Diagnostic Procedures
Lumbar Puncture and Cerebrospinal Fluid Examination
Neuroradiologic Procedures
Electroencephalography
Evoked Potentials
Specific and General Genetic and Metabolic Testing
Bibliography
Chapter 609: Congenital Anomalies of the Central Nervous System
Keywords
609.1 Neural Tube Defects
Hydrocephalus
609.2 Spina Bifida Occulta (Occult Spinal Dysraphism)
609.3 Meningocele
609.4 Myelomeningocele
Etiology
Prevention
Clinical Manifestations
Treatment
Prognosis
Bibliography
609.5 Encephalocele
Bibliography
609.6 Anencephaly
609.7 Disorders of Neuronal Migration
Lissencephaly
Schizencephaly
Neuronal Heterotopias
Polymicrogyrias
Focal Cortical Dysplasias
Porencephaly
Bibliography
609.8 Agenesis of the Corpus Callosum
Holoprosencephaly
Bibliography
609.9 Agenesis of the Cranial Nerves and Dysgenesis of the Posterior Fossa
Congenital Cranial Dysinnervation Disorders
Brainstem and Cerebellar Disorders
Bibliography
609.10 Microcephaly
Etiology
Clinical Manifestations and Diagnosis
Treatment
Bibliography
609.11 Hydrocephalus
Physiology
Pathophysiology and Etiology
Clinical Manifestations
Diagnosis and Differential Diagnosis
Megalencephaly
Hydranencephaly
Treatment
Prognosis
Bibliography
609.12 Craniosynostosis
Development and Etiology
Clinical Manifestations and Treatment
Bibliography
Bibliography
Chapter 610: Deformational Plagiocephaly
Keywords
Epidemiology and Etiology
Incidence
Risk Factors
Causes
Examination and Differentiating Between Deformational Plagiocephaly and Craniosynostosis
History and Physical Examination
Treatment
Prevention
Treatment Options
Outcomes
Bibliography
Chapter 611: Seizures in Childhood
Keywords
Evaluation of the First Seizure
611.1 Febrile Seizures
Keywords
Genetic and Other Factors Leading to Febrile Seizures
Evaluation
Lumbar Puncture
Electroencephalogram
Blood Studies
Neuroimaging
Treatment
Bibliography
611.2 Unprovoked Seizures
Keywords
History and Examination
Differential Diagnosis
Long-Term Approach to the Patient and Additional Testing
Bibliography
611.3 Focal Seizures and Related Epilepsy Syndromes
Keywords
Focal Seizures With Preserved Awareness
Focal Seizures With Impaired Awareness
Focal to Bilateral Tonic-Clonic Seizures
Benign Epilepsy Syndromes With Focal Seizures
Severe Epilepsy Syndromes With Focal Seizures
Bibliography
611.4 Generalized Seizures and Related Epilepsy Syndromes
Keywords
Absence Seizures
Generalized Motor Seizures
Benign Generalized Epilepsies
Severe Generalized Epilepsies
611.5 Mechanisms of Seizures
Keywords
Bibliography
611.6 Treatment of Seizures and Epilepsy
Keywords
Deciding on Long-Term Therapy
Counseling
Mechanisms of Action of Antiepileptic Drugs
Choice of Drug According to Seizure Type and Epilepsy Syndrome
Choice of Drug: Other Considerations
Initiating and Monitoring Therapy
Titration
Monitoring
Side Effects
Additional Treatments
Approach to Epilepsy Surgery
Discontinuation of Therapy
Sudden Unexpected Death in Epilepsy (SUDEP)
Bibliography
611.7 Neonatal Seizures
Keywords
Pathophysiology
Types of Neonatal Seizures
Subtle Seizures
Clonic Seizures
Tonic Seizures
Spasms
Myoclonic Seizures
Seizures Versus Jitteriness
Etiology
Hypoxic-Ischemic Encephalopathy
Vascular Events
Intracranial Infections
Brain Malformations
Metabolic Disturbances
Drug Withdrawal
Neonatal Seizure Syndromes
Miscellaneous Conditions
Diagnosis
Prognosis
Treatment
Lorazepam and Other Benzodiazepines
Phenobarbital
Phenytoin and Fosphenytoin
Other Medications
Duration of Therapy
Bibliography
611.8 Status Epilepticus
Keywords
Etiology
Mechanisms
Therapy
Bibliography
611.9 Reflex Seizures (Stimulus-Precipitated Seizures)
Keyword
Bibliography
611.10 Nodding Syndrome
Keywords
Bibliography
Chapter 612: Conditions That Mimic Seizures
Keywords
Syncope and Other Generalized Paroxysms
Apnea
Breath-Holding Spells
Compulsive Valsalva-Like Maneuver
Neurally Mediated Syncope
Postural Tachycardia Syndrome
Cardiac Syncope
Migraine and Migraine Variants
Psychological Disorders
Paroxysmal Extreme Pain Disorder
Autonomic Storms
Movement Disorders and Other Paroxysmal Movements and Postures
Neonatal Jitteriness and Clonus
Hyperekplexia (Stiff Baby Syndrome) and Pathologic Startles
Benign Paroxysmal Torticollis of Infancy
Sandifer Syndrome and Rumination
Alternating Hemiplegia of Childhood
Paroxysmal Dyskinesias and Other Movement Disorders
Motor Tics
Episodic Ataxias
Benign Motoric Paroxysms in Infancy
Brainstem Dysfunction
Psychological Disorders
Oculomotor and Visual Abnormalities
Paroxysmal Tonic Upgaze of Childhood
Oculomotor Apraxia and Saccadic Intrusions
Spasmus Nutans
Opsoclonus–Myoclonus Syndrome
Daydreaming and Behavioral Staring
Visual Hallucinations
Sleep-Related Disorders
Benign Sleep Myoclonus and Neonatal Sleep Myoclonus
Non-Rapid Eye Movement Partial Arousal Disorders
Rapid Eye Movement Sleep Disorders
Sleep Transition Disorders
Narcolepsy–Cataplexy Syndrome
Bibliography
Chapter 613: Headaches
Keywords
613.1 Migraine
Epidemiology
Classification and Clinical Manifestations
Migraine Without Aura
Migraine With Aura
Diagnosis and Differential Diagnosis
Treatment
Acute Treatment
Emergency Department Treatments for Intractable Headaches
Antidopaminergic Drugs: Prochlorperazine and Metoclopramide
Nonsteroidal Antiinflammatory Drugs: Ketorolac
Antiepileptic Drugs: Sodium Valproate
Triptans
Dihydroergotamine (DHE)
Inpatient Management of Intractable Migraine and Status Migrainosus
Dihydroergotamine
Sodium Valproate
Other Inpatient Therapies
Preventive Therapy
Biobehavioral Therapy
Young Adults and the Transition of Headache Care From a Pediatric to an Adult Provider
Bibliography
613.2 Secondary Headaches
Bibliography
613.3 Tension-Type Headaches
Bibliography
Chapter 614: Neurocutaneous Syndromes
Keywords
614.1 Neurofibromatosis
Clinical Manifestations and Diagnosis
Management
Genetic Counseling
Bibliography
614.2 Tuberous Sclerosis
Clinical Manifestations and Diagnosis
Skin Lesions
Other Organ Involvement
Management
Bibliography
614.3 Sturge-Weber Syndrome
Etiology
Clinical Manifestations
Diagnosis
Management
Bibliography
614.4 Von Hippel-Lindau Disease
Bibliography
614.5 Linear Nevus Sebaceous Syndrome
Bibliography
614.6 PHACE Syndrome
Bibliography
614.7 Incontinentia Pigmenti
Clinical Manifestations and Diagnosis
Management
Bibliography
Chapter 615: Movement Disorders
Keywords
615.1 Ataxias
Bibliography
615.2 Chorea, Athetosis, Tremor
Bibliography
615.3 Myoclonus
Bibliography
615.4 Dystonia
Keywords
Inherited Primary Dystonias
Drug-Induced Dystonias
Cerebral Palsy
Metabolic Disorders
Other Disorders
Treatment
Bibliography
Bibliography
Chapter 616: Encephalopathies
Keywords
616.1 Cerebral Palsy
Epidemiology and Etiology
Clinical Manifestations
Diagnosis
Treatment
Bibliography
616.2 Mitochondrial Encephalomyopathies
Mitochondrial Myopathy, Encephalopathy, Lactic Acidosis, and Stroke-Like Episodes
Myoclonic Epilepsy and Ragged Red Fibers
Neuropathy, Ataxia, and Retinitis Pigmentosa (NARP) Syndrome
Leber Hereditary Optic Neuropathy (LHON)
Kearns-Sayre Syndrome (KSS)
Reversible Infantile Cytochrome C Oxidase Deficiency Myopathy
Leigh Disease (Subacute Necrotizing Encephalomyopathy)
Mitochondrial DNA Depletion Syndrome
Reye Syndrome
Bibliography
616.3 Other Encephalopathies
Human Immunodeficiency Virus (HIV) Encephalopathy
Lead Encephalopathy
Burn Encephalopathy
Hypertensive Encephalopathy
Radiation Encephalopathy
Acute Necrotizing Encephalopathy
Cystic Leukoencephalopathy
Bibliography
616.4 Autoimmune Encephalitis
Keywords
Anti-N-Methyl-D-Aspartate Receptor Encephalitis
Other Types of Encephalitis Associated With Antibodies Against Neuronal Cell Surface Antigens
Acquired Demyelinating Syndromes With Encephalopathy
Hashimoto Encephalopathy
Opsoclonus–Myoclonus and Other Types of Brainstem–Cerebellar Encephalitis
Bickerstaff Encephalitis
Chronic Lymphocytic Inflammation With Pontine Perivascular Enhancement Responsive to Steroids
Autoimmune Encephalopathies Associated With Epilepsy and Status Epilepticus
Other Suspected Types of Autoimmune Encephalitis
Bibliography
Chapter 617: Neurodegenerative Disorders of Childhood
617.1 Sphingolipidoses
Keywords
Gangliosidoses
GM1 Gangliosidoses
GM2 Gangliosidoses
Krabbe Disease (Globoid Cell Leukodystrophy)
Metachromatic Leukodystrophy
Bibliography
617.2 Neuronal Ceroid Lipofuscinoses
Keywords
Bibliography
617.3 Adrenoleukodystrophy
617.4 Sialidosis
Keywords
Bibliography
617.5 Miscellaneous Neurodegenerative Disorders
Keywords
Pelizaeus-Merzbacher Disease
Alexander Disease
Canavan Spongy Degeneration
Other Leukodystrophies
Menkes Disease
Rett Syndrome
Neurodegeneration With Brain Iron Accumulation
Bibliography
Chapter 618: Demyelinating Disorders of the Central Nervous System
Keywords
618.1 Acute Disseminated Encephalomyelitis
Epidemiology
Pathogenesis
Clinical Manifestations
Neuroimaging
Laboratory Findings
Differential Diagnosis
Treatment
Prognosis
Bibliography
618.2 Optic Neuritis
Epidemiology and Clinical Presentation
Diagnostic Evaluation
Treatment
Prognosis
Bibliography
618.3 Transverse Myelitis
Epidemiology
Clinical Manifestations
Diagnostic Evaluation
Treatment
Prognosis
Bibliography
618.4 Multiple Sclerosis
Epidemiology and Risk Factors
Pathogenesis
Clinical Manifestations
Imaging and Laboratory Findings
Diagnosis and Differential Diagnosis
Treatment
Prognosis
Bibliography
618.5 Neuromyelitis Optica Spectrum Disorders
Epidemiology
Pathogenesis
Clinical Manifestations
Imaging and Laboratory Findings
Diagnosis and Differential Diagnosis
Treatment
Prognosis
Bibliography
618.6 Myelin Oligodendrocyte Glycoprotein–Associated Disorders
Clinical Presentation
Imaging and Laboratory Findings
Treatment
Prognosis
Bibliography
Bibliography
Chapter 619: Pediatric Stroke
Keywords
619.1 Arterial Ischemic Stroke
Perinatal Arterial Ischemic Stroke
Bibliography
619.2 Cerebral Sinovenous Thrombosis
Keywords
Bibliography
619.3 Spinal Cord Lesions Associated With Vascular Processes
Keywords
Vasculitic Processes: Systemic Lupus Erythematosus
Spinal Cord Embolism
Clinical Manifestations
Investigations
Treatment
Bibliography
619.4 Hemorrhagic Stroke
Keywords
Bibliography
619.5 Differential Diagnosis of Stroke-Like Events
Migraine
Seizure
Infection
Demyelination
Hypoglycemia
Global Hypoxic-Ischemic Encephalopathy
Hypertensive Encephalopathy
Inborn Errors of Metabolism
Vestibulopathy and Ataxia
Channelopathies
Alternating Hemiplegia of Childhood
Chapter 620: Central Nervous System Vasculitis
Keywords
Epidemiology
Clinical Manifestations
Diagnosis
Treatment
Prognosis
Bibliography
Chapter 621: Central Nervous System Infections
Keywords
621.1 Acute Bacterial Meningitis Beyond the Neonatal Period
Epidemiology
Streptococcus pneumoniae
Neisseria meningitidis
Haemophilus influenzae Type b
Pathology and Pathophysiology
Pathogenesis
Clinical Manifestations
Diagnosis
Lumbar Puncture
Differential Diagnosis
Treatment
Initial Antibiotic Therapy
Duration of Antibiotic Therapy
Corticosteroids
Complications
Prognosis
Prevention
Neisseria meningitidis
Haemophilus influenzae Type B
Streptococcus pneumoniae
Bibliography
621.2 Viral Meningoencephalitis
Etiology
Epidemiology
Pathogenesis and Pathology
Clinical Manifestations
Diagnosis
Differential Diagnosis
Laboratory Findings
Treatment
Prognosis
Prevention
Bibliography
621.3 Eosinophilic Meningitis
Etiology
Epidemiology
Clinical Manifestations
Diagnosis
Treatment
Prognosis
Bibliography
Chapter 622: Brain Abscess
Keywords
Pathology
Etiology
Clinical Manifestations
Diagnosis
Treatment
Prognosis
Bibliography
Chapter 623: Idiopathic Intracranial Hypertension (Pseudotumor Cerebri)
Keywords
Etiology
Clinical Manifestations
Treatment
Bibliography
Chapter 624: Spinal Cord Disorders
Keywords
624.1 Tethered Cord
Clinical Manifestations
Diagnostic Evaluation
Treatment
Outcome
Bibliography
624.2 Diastematomyelia (Split-Cord Malformation)
Clinical Manifestations
Diagnostic Evaluation
Treatment
Bibliography
624.3 Syringomyelia
Clinical Manifestations
Diagnostic Evaluation
Treatment
Bibliography
624.4 Spinal Cord Tumors
Clinical Manifestations
Diagnostic Evaluation
Treatment
Outcome
Bibliography
624.5 Spinal Arteriovenous Malformations
Diagnostic Evaluation
Treatment
Bibliography
Part XXVII: Neuromuscular Disorders
Chapter 625: Evaluation and Investigation of Neuromuscular Disorders
Genetic Testing
Clinical Manifestations
Laboratory Findings
Serum Enzymes
Molecular Genetic Markers
Nerve Conduction Velocity
Electromyography
Imaging of Muscles and the Central Nervous System
Muscle Biopsy
Nerve Biopsy
Cardiac Assessment
Bibliography
Chapter 626: Developmental Disorders of Muscle
Myogenic Regulatory Genes and Genetic Loci of Inherited Diseases of Muscle
Treatment of Congenital Myopathies
626.1 Myotubular Myopathy (Centronuclear Myopathy)
Pathogenesis
Clinical Manifestations
Laboratory Findings
Diagnosis
Genetics
Treatment
Prognosis
Bibliography
626.2 Congenital Muscle Fiber–Type Disproportion
Pathogenesis
Clinical Manifestations
Laboratory Findings
Diagnosis
Genetics
Treatment
Bibliography
626.3 Nemaline Rod Myopathy
Clinical Manifestations
Laboratory Findings
Genetics
Treatment and Prognosis
Bibliography
626.4 Core Myopathies
Clinical Manifestations
Central Core Disease
Multiminicore Disease
Laboratory Findings
Genetics
Treatment and Prognosis
Bibliography
626.5 Myofibrillar Myopathies
Clinical Manifestations and MFM Subtypes According to Genetic Background
Laboratory Findings
Treatment
Bibliography
626.6 Brain Malformations and Muscle Development
Bibliography
626.7 Amyoplasia
Bibliography
626.8 Muscular Dysgenesis (Proteus Syndrome Myopathy)
Bibliography
626.9 Benign Congenital Hypotonia
Bibliography
626.10 Arthrogryposis
Fetal Movement and the Link to Arthrogryposis
Basic Categories, Etiologies, and Classifications
Diagnostic Approach and Laboratory Evaluation
Differential Diagnosis
Genetic Counseling and Prenatal Diagnosis
Management
Bibliography
Bibliography
Chapter 627: Muscular Dystrophies
Keywords
627.1 Duchenne and Becker Muscular Dystrophies
Clinical Manifestations
Laboratory Findings
Diagnosis
Genetic Etiology and Pathogenesis
Treatment
Bibliography
627.2 Emery-Dreifuss Muscular Dystrophy/Laminopathies
Genetics
Diagnostics
Bibliography
627.3 Myotonic Muscular Dystrophy
Clinical Manifestations
Laboratory Findings
Diagnosis
Genetics
Treatment
Other Myotonic Syndromes
Bibliography
627.4 Limb–Girdle Muscular Dystrophies
Bibliography
627.5 Facioscapulohumeral Muscular Dystrophy
Clinical Manifestations
Laboratory Findings
Diagnosis and Differential Diagnosis
Treatment
Bibliography
627.6 Congenital Muscular Dystrophies
Clinical Manifestations
Laboratory Findings
Diagnosis
Treatment
Bibliography
Chapter 628: Endocrine and Toxic Myopathies
Thyroid Myopathies
Steroid-Hormone Induced Myopathy
Statin-Induced Rhabdomyolysis With Myoglobinuria
Mitochondrial Dysfunction in Toxic Myopathies
Critical Illness Myopathy
Bibliography
Chapter 629: Metabolic Myopathies and Channelopathies
629.1 Periodic Paralyses and Other Muscle Channelopathies
Treatment
Other Muscle Channelopathies
Bibliography
629.2 Malignant Hyperthermia
Bibliography
629.3 Glycogenoses
Bibliography
629.4 Mitochondrial Myopathies
Investigations
Treatment
Bibliography
629.5 Lipid Myopathies
Bibliography
629.6 Vitamin E Deficiency Myopathy
Bibliography
Chapter 630: Disorders of Neuromuscular Transmission and of Motor Neurons
630.1 Myasthenia Gravis
Autoimmune Myasthenia Gravis
Clinical Manifestations
Congenital Myasthenic Syndromes
Rare Other Causes of Myasthenia
Laboratory Findings and Diagnosis
Recommendations on the Use of Cholinesterase Inhibitors as a Diagnostic Test for MG in Infants and Children
Children Two Years of Age and Older
Children Younger Than Two Years of Age
Treatment
Complications
Prognosis
Other Causes of Neuromuscular Blockade
Bibliography
630.2 Spinal Muscular Atrophies
Etiology
Clinical Manifestations and Course
Laboratory Findings
Diagnosis
Genetics
Management
Therapeutic Advances
Bibliography
630.3 Other Motor Neuron Diseases
Bibliography
Chapter 631: Hereditary Motor-Sensory Neuropathies
631.1 Peroneal Muscular Atrophy (Charcot-Marie-Tooth Disease, HMSN Type IIa)
Clinical Manifestations
Laboratory Findings and Diagnosis
Treatment
Bibliography
631.2 Peroneal Muscular Atrophy (Axonal Type)
Bibliography
631.3 Congenital Hypomyelinating Neuropathy and Déjèrine-Sottas Disease (HMSN Type III)
Bibliography
631.4 Roussy-Lévy Syndrome
631.5 Refsum Disease (HMSN Type IV) and Infantile Refsum Disease
Bibliography
631.6 Fabry Disease
Clinical Manifestations
Laboratory Findings
Treatment
Bibliography
631.7 Giant Axonal Neuropathy
Bibliography
631.8 Hypermyelinating (Tomaculous) Neuropathy; Hereditary Neuropathy With Liability to Pressure Palsies
Bibliography
631.9 Leukodystrophies
Bibliography
Chapter 632: Toxic Neuropathies
Keywords
Bibliography
Chapter 633: Autonomic Neuropathies
Keywords
633.1 Familial Dysautonomia
Pathology
Clinical Manifestations
Laboratory Findings
Diagnosis
Treatment
Prognosis
Bibliography
633.2 Other Autonomic Neuropathies
Congenital Insensitivity to Pain and Anhidrosis
Allgrove Syndrome (Triple a Syndrome)
Bibliography
Chapter 634: Guillain-Barré Syndrome
Keywords
Clinical Manifestations
Laboratory Findings and Diagnosis
Treatment
Prognosis
Bibliography
Chapter 635: Bell Palsy
Keywords
Clinical Manifestations
Treatment
Prognosis
Facial Palsy at Birth
Bibliography
Part XXVIII: Disorders of the Eye
Chapter 636: Growth and Development of the Eye
Keywords
Bibliography
Chapter 637: Examination of the Eye
Keywords
Visual Acuity
Visual Field Assessment
Color Vision Testing
Pupillary Examination
Ocular Motility
Binocular Vision
External Examination
Biomicroscopy (Slit-Lamp Examination)
Fundus Examination (Ophthalmoscopy)
Refraction
Tonometry
Bibliography
Chapter 638: Abnormalities of Refraction and Accommodation
Keywords
Hyperopia
Myopia
Astigmatism
Anisometropia
Accommodation
Bibliography
Chapter 639: Disorders of Vision
Keywords
Amblyopia
Diplopia
Suppression
Amaurosis
Nyctalopia
Psychogenic Disturbances
Dyslexia
Bibliography
Chapter 640: Abnormalities of Pupil and Iris
Keywords
Aniridia
Coloboma of the Iris
Microcoria
Congenital Mydriasis
Dyscoria and Corectopia
Anisocoria
Dilated Fixed Pupil
Tonic Pupil
Marcus Gunn Pupil
Horner Syndrome
Paradoxical Pupil Reaction
Persistent Pupillary Membrane
Heterochromia
Other Iris Lesions
Leukocoria
Bibliography
Chapter 641: Disorders of Eye Movement and Alignment
Keywords
Strabismus
Diagnosis
Clinical Manifestations and Treatment
Comitant Strabismus
Noncomitant Strabismus
3rd Nerve Palsy
4th Nerve Palsy
6th Nerve Palsy
Strabismus Syndromes
Monocular Elevation Deficiency
Duane Syndrome
Möbius Syndrome
Brown Syndrome
Parinaud Syndrome
Congenital Ocular Motor Apraxia
Nystagmus
Other Abnormal Eye Movements
Opsoclonus
Ocular Motor Dysmetria
Flutter-Like Oscillations
Bibliography
Chapter 642: Abnormalities of the Lids
Keywords
Ptosis
Epicanthal Folds
Lagophthalmos
Lid Retraction
Ectropion, Entropion, and Epiblepharon
Blepharospasm
Blepharitis
Hordeolum (Stye)
Chalazion
Coloboma of the Eyelid
Tumors of the Lid
Bibliography
Chapter 643: Disorders of the Lacrimal System
Keywords
The Tear Film
Dacryostenosis
Alacrima and “Dry Eye”
Bibliography
Chapter 644: Disorders of the Conjunctiva
Keywords
Conjunctivitis
Ophthalmia Neonatorum
Epidemiology
Clinical Manifestations
Diagnosis
Treatment
Prognosis and Prevention
Acute Purulent Conjunctivitis
Viral Conjunctivitis
Epidemic Keratoconjunctivitis
Membranous and Pseudomembranous Conjunctivitis
Allergic Conjunctivitis
Vernal Conjunctivitis
Parinaud Oculoglandular Syndrome
Chemical Conjunctivitis
Other Conjunctival Disorders
Bibliography
Chapter 645: Abnormalities of the Cornea
Keywords
Megalocornea
Microcornea
Keratoconus
Neonatal Corneal Opacities
Sclerocornea
Peters Anomaly
Corneal Dystrophies
Dermoids
Dendritic Keratitis
Corneal Ulcers
Phlyctenules
Interstitial Keratitis
Corneal Manifestations of Systemic Disease
Bibliography
Chapter 646: Abnormalities of the Lens
Keywords
Cataracts
Differential Diagnosis
Developmental Variants
Prematurity
Mendelian Inheritance
Congenital Infection Syndrome
Metabolic Disorders
Chromosomal Defects
Drugs, Toxic Agents, and Trauma
Miscellaneous Disorders
Treatment
Prognosis
Ectopia Lentis
Differential Diagnosis
Treatment and Prognosis
Other Disorders of the Lens
Microspherophakia
Anterior Lenticonus
Posterior Lenticonus
Bibliography
Chapter 647: Disorders of the Uveal Tract
Keywords
Uveitis (Iritis, Cyclitis, Chorioretinitis)
Treatment
Bibliography
Chapter 648: Disorders of the Retina and Vitreous
Keywords
Retinopathy of Prematurity
Pathogenesis
Classification
Clinical Manifestations and Prognosis
Diagnosis
Treatment
Prevention
Persistent Fetal Vasculature
Pathogenesis
Clinical Manifestations
Treatment
Retinoblastoma
Clinical Manifestations
Diagnosis
Treatment
Retinitis Pigmentosa
Stargardt Disease (Fundus Flavimaculatus)
Best Vitelliform Degeneration
Cherry-Red Spot
Phakomas
Retinoschisis
Retinal Detachment
Coats Disease
Familial Exudative Vitreoretinopathy
Hypertensive Retinopathy
Diabetic Retinopathy
Treatment
Subacute Bacterial Endocarditis
Blood Disorders
Trauma-Related Retinopathy
Myelinated Nerve Fibers
Coloboma of the Fundus
Bibliography
Chapter 649: Abnormalities of the Optic Nerve
Keywords
Optic Nerve Aplasia
Optic Nerve Hypoplasia
Optic Nerve Coloboma
Morning Glory Disc Anomaly
Tilted Disc
Drusen of the Optic Nerve
Papilledema
Optic Neuritis
Leber Optic Neuropathy
Optic Atrophy
Optic Nerve Glioma
Traumatic Optic Neuropathies
Bibliography
Chapter 650: Childhood Glaucoma
Keywords
Clinical Manifestations
Diagnosis and Treatment
Bibliography
Chapter 651: Orbital Abnormalities
Keywords
Hypertelorism and Hypotelorism
Exophthalmos and Enophthalmos
Orbital Inflammation
Tumors of the Orbit
Bibliography
Chapter 652: Orbital Infections
Keywords
Dacryoadenitis
Dacryocystitis
Preseptal Cellulitis
Orbital Cellulitis
Bibliography
Chapter 653: Injuries to the Eye
Keywords
Ecchymosis and Swelling of the Eyelids
Lacerations of the Eyelids
Superficial Abrasions of the Cornea
Foreign Body Involving the Ocular Surface
Hyphema
Open Globe
Optic Nerve Trauma
Chemical Injuries
Orbital Fractures
Penetrating Wounds of the Orbit
Child Abuse
Fireworks-Related Injuries
Sports-Related Ocular Injuries and Their Prevention
Handheld Laser Retinal Injury
Bibliography
Part XXIX: The Ear
Chapter 654: General Considerations and Evaluation of the Ear
Keywords
Clinical Manifestations
Facial Paralysis
Physical Examination
Bibliography
Chapter 655: Hearing Loss
Keywords
Incidence and Prevalence
Types of Hearing Loss
Etiology
Infectious Causes
Genetic Causes
Physical Causes
Effects of Hearing Impairment
Hearing Screening
Identification of Hearing Impairment
Clinical Audiologic Evaluation
Audiometry
Speech-Recognition Threshold
Play Audiometry
Visual Reinforcement Audiometry
Behavioral Observation Audiometry
Acoustic Immittance Testing
Tympanometry
Tympanometry in Otitis Media With Effusion
Acoustic Reflex Threshold Test
Auditory Brainstem Response
Otoacoustic Emissions
Treatment
Genetic Counseling
Bibliography
Chapter 656: Congenital Malformations of the Ear
Keywords
Pinna Malformations
Congenital Stenosis or Atresia of the External Auditory Canal
Congenital Middle-Ear Malformations
Congenital Inner Ear Malformations
Congenital Cholesteatoma
Bibliography
Chapter 657: External Otitis (Otitis Externa)
Keywords
Etiology
Clinical Manifestations
Diagnosis
Treatment
Prevention
Other Diseases of the External Ear
Furunculosis
Acute Cellulitis
Perichondritis and Chondritis
Dermatoses
Herpes Simplex Virus
Bullous Myringitis
Exostoses and Osteomas
Bibliography
Chapter 658: Otitis Media
Keywords
Epidemiology
Age
Gender
Race
Genetic Background
Socioeconomic Status
Breast Milk Compared With Formula Feeding
Exposure to Tobacco Smoke
Exposure to Other Children
Season
Congenital Anomalies
Other Factors
Etiology
Acute Otitis Media
Otitis Media With Effusion
Pathogenesis
Anatomic Factors
Host Factors
Viral Pathogens
Allergy
Clinical Manifestations
Examination of the Tympanic Membrane
Otoscopy
Clearing the External Auditory Canal
Tympanic Membrane Findings
Diagnosis
Conjunctivitis-Associated Otitis Media
Asymptomatic Purulent Otitis Media
Tympanometry
Prevention
Immunoprophylaxis and Vaccination Status
Treatment
Management of Acute Otitis Media
Bacterial Resistance
First-Line Antimicrobial Treatment
Duration of Treatment
Follow-Up
Unsatisfactory Response to First-Line Treatment
Second-Line Treatment
Antimicrobial Prophylaxis
Myringotomy and Tympanocentesis
Early Recurrence After Treatment
Myringotomy and Insertion of Tympanostomy Tubes
Tube Otorrhea
Management of Otitis Media With Effusion
Variables Influencing Otitis Media With Effusion Management Decisions
Medical Treatment
Myringotomy and Insertion of Tympanostomy Tubes
Adenoidectomy
Complications of Acute Otitis Media
Intratemporal Complications
Infectious Dermatitis
Tympanic Membrane Perforation
Chronic Suppurative Otitis Media
Mastoiditis
Facial Paralysis
Cholesteatoma
Labyrinthitis
Intracranial Complications
Physical Sequelae
Possible Developmental Sequelae
Bibliography
Chapter 659: Acute Mastoiditis
Keywords
Anatomy
Epidemiology
Microbiology
Clinical Manifestations
Imaging
Management
Special Situations
Bibliography
Chapter 660: The Inner Ear and Diseases of the Bony Labyrinth
Keywords
Other Diseases of the Inner Ear
Bibliography
Chapter 661: Traumatic Injuries of the Ear and Temporal Bone
Keywords
Auricle and External Auditory Canal
Tympanic Membrane and Middle Ear
Temporal Bone Fractures
Acoustic Trauma
Bibliography
Chapter 662: Tumors of the Ear and Temporal Bone
Keywords
Bibliography
Part XXX: The Skin
Chapter 663: Morphology of the Skin
Keywords
Epidermis
Dermis
Subcutaneous Tissue
Appendageal Structures
Hair Follicles
Sebaceous Glands
Apocrine Glands
Eccrine Sweat Glands
Nails
Bibliography
Chapter 664: Dermatologic Evaluation of the Patient
Keywords
History and Physical Examination
Biopsy of Skin
Wood Lamp
Potassium Hydroxide Preparation
Tzanck Smear
Immunofluorescence Studies
664.1 Cutaneous Manifestations of Systemic Diseases
Connective Tissue Diseases
Lupus Erythematosus
Systemic Lupus Erythematosus
Neonatal Lupus Erythematosus
Discoid Lupus Erythematosus
Juvenile Dermatomyositis
Systemic Sclerosis
Morphea
Vasculitides
Henoch-Schönlein Purpura (Immunoglobulin A Vasculitis)
Kawasaki Disease
Behçet Disease
Gastrointestinal Diseases
Inflammatory Bowel Disease
Cutaneous Manifestations of Malignancy
Sweet Syndrome
Langerhans Cell Histiocytosis
Necrolytic Migratory Erythema (Glucagonoma Syndrome)
Erythromelalgia
Cutaneous Reactions in the Setting of Immunosuppression
Medication Reactions
Graft Versus Host Disease
664.2 Multisystem Medication Reactions
Drug Rash With Eosinophilia and Systemic Symptoms (DRESS Syndrome)
Serum Sickness–Like Reaction
Fixed-Drug Eruption
Acute Generalized Exanthematous Pustulosis
Bibliography
Chapter 665: Principles of Dermatologic Therapy
Keywords
Wet Dressings
Bath Oils, Colloids, Soaps
Lubricants
Shampoos
Shake Lotions
Powders
Pastes
Keratolytic Agents
Tar Compounds
Antifungal Agents
Topical Antibiotics
Topical Corticosteroids
Topical Nonsteroidal Antiinflammatory Agents
Sunscreens
Laser Therapy
Bibliography
Chapter 666: Dermatologic Diseases of the Neonate
Keywords
Sebaceous Hyperplasia
Milia
Sucking Blisters
Cutis Marmorata
Harlequin Color Change
Nevus Simplex (Salmon Patch)
Dermal Melanocytosis (Mongolian Spots)
Erythema Toxicum
Transient Neonatal Pustular Melanosis
Infantile Acropustulosis
Eosinophilic Pustular Folliculitis
Bibliography
Chapter 667: Cutaneous Defects
Keywords
Skin Dimples
Redundant Skin
Amniotic Constriction Bands
Preauricular Sinuses and Pits
Accessory Tragi
Branchial Cleft and Thyroglossal Cysts and Sinuses
Supernumerary Nipples
Aplasia Cutis Congenita (Congenital Absence of Skin)
Focal Facial Dermal Dysplasias
Focal Dermal Hypoplasia (Goltz-Gorlin Syndrome)
Dyskeratosis Congenita (Zinsser-Engman-Cole Syndrome)
Cutis Verticis Gyrata
Bibliography
Chapter 668: Ectodermal Dysplasias
Keywords
Hypohidrotic Ectodermal Dysplasia
Hidrotic Ectodermal Dysplasia (Clouston Syndrome)
Bibliography
Chapter 669: Vascular Disorders
Keywords
Vascular Malformations
Capillary Malformation (Port-Wine Stain)
Venous Malformation
Lymphatic Malformations
Arteriovenous Malformation
Klippel-Trenaunay and Parkes-Weber Syndromes
Angiokeratoma Circumscriptum
Cutis Marmorata Telangiectatica Congenita
Blue Rubber Bleb Nevus Syndrome
Phakomatosis Pigmentovascularis
Nevus Anemicus
Vascular Tumors
Infantile Hemangioma
Multifocal Infantile Hemangioma
Congenital Hemangioma
Kaposiform Hemangioendothelioma
Kasabach-Merritt Phenomenon
Pyogenic Granuloma (Lobular Capillary Hemangioma)
Angiokeratoma of Mibelli
Spider Angioma
Maffucci Syndrome
Hereditary Hemorrhagic Telangiectasia (Osler-Weber-Rendu Disease)
Ataxia-Telangiectasia
Angiokeratoma Corporis Diffusum (Fabry Disease)
Bibliography
Chapter 670: Cutaneous Nevi
Keywords
Acquired Melanocytic Nevus
Epidemiology
Clinical Manifestations
Prognosis and Treatment
Atypical Melanocytic Nevus
Congenital Melanocytic Nevus
Melanoma
Halo Nevus
Spitz Nevus (Spindle and Epithelioid Cell Nevus)
Zosteriform Lentiginous Nevus (Agminated Lentigines)
Nevus Spilus (Speckled Lentiginous Nevus)
Nevus of Ota and Nevus of Ito
Blue Nevi
Nevus Depigmentosus (Achromic Nevus)
Epidermal Nevi
Nevus Sebaceus (Jadassohn)
Becker Nevus (Becker Melanosis)
Nevus Comedonicus
Connective Tissue Nevus
Smooth Muscle Hamartoma
Bibliography
Chapter 671: Hyperpigmented Lesions
Keywords
Disorders of Pigment
Ephelides (Freckles)
Lentigines
Café-Au-Lait Spots
Neurofibromatosis Type 1 (Von Recklinghausen Disease)
Incontinentia Pigmenti (Bloch-Sulzberger Disease)
PostInflammatory Pigmentary Changes
Bibliography
Chapter 672: Hypopigmented Lesions
Keywords
Albinism
Oculocutaneous Albinism With Melanosomal Abnormalities
Melanoblast Migration Abnormalities
Piebaldism
Waardenburg Syndrome
Tuberous Sclerosis Complex (TSC1, TSC2 Genes)
Hypomelanosis of Ito
Vitiligo
Epidemiology and Etiology
Clinical Manifestations
Treatment
Bibliography
Chapter 673: Vesiculobullous Disorders
Keywords
673.1 Erythema Multiforme
Keywords
Etiology
Clinical Manifestations
Pathogenesis
Pathology
Differential Diagnosis
Treatment
Bibliography
673.2 Stevens-Johnson Syndrome
Keywords
Etiology
Clinical Manifestations
Pathogenesis
Differential Diagnosis
Treatment
Bibliography
673.3 Toxic Epidermal Necrolysis
Keywords
Epidemiology and Etiology
Clinical Manifestations
Treatment
Bibliography
673.4 Mechanobullous Disorders
Keywords
Epidermolysis Bullosa
Epidermolysis Bullosa Simplex
Junctional Epidermolysis Bullosa
Dystrophic Epidermolysis Bullosa
Kindler Syndrome
Bibliography
673.5 Pemphigus
Keywords
Pemphigus Vulgaris
Etiology/Pathogenesis
Clinical Manifestations
Pathology
Differential Diagnosis
Treatment
Pemphigus Foliaceus
Etiology/Pathogenesis
Clinical Manifestations
Pathology
Differential Diagnosis
Bullous Pemphigoid
Etiology/Pathogenesis
Clinical Manifestations
Pathology
Diagnosis and Differential Diagnoses
Treatment
Bibliography
673.6 Dermatitis Herpetiformis
Keywords
Etiology/Pathogenesis
Clinical Manifestations
Pathology
Differential Diagnosis
Treatment
Bibliography
673.7 Linear Immunoglobulin A Dermatosis (Chronic Bullous Dermatosis of Childhood)
Keywords
Etiology/Pathogenesis
Clinical Manifestations
Pathology
Differential Diagnosis
Treatment
Bibliography
Chapter 674: Eczematous Disorders
Keywords
674.1 Contact Dermatitis
Irritant Contact Dermatitis
Diaper Dermatitis
Allergic Contact Dermatitis
Bibliography
674.2 Nummular Eczema
Bibliography
674.3 Pityriasis Alba
Bibliography
674.4 Lichen Simplex Chronicus
674.5 Acute Palmoplantar Eczema (Dyshidrotic Eczema, Dyshidrosis, Pompholyx)
Bibliography
674.6 Seborrheic Dermatitis
Etiology
Clinical Manifestations
Differential Diagnosis
Treatment
Bibliography
Chapter 675: Photosensitivity
Keywords
Acute Sunburn Reaction
Photosensitive Reactions
Porphyrias
Colloid Milium
Hydroa Vacciniforme
Solar Urticaria
Polymorphous Light Eruption
Actinic Prurigo
Cockayne Syndrome
Xeroderma Pigmentosum
Rothmund-Thomson Syndrome
Bloom Syndrome
Hartnup Disease
Bibliography
Chapter 676: Diseases of the Epidermis
Keywords
676.1 Psoriasis
Etiology/Pathogenesis
Clinical Manifestations
Differential Diagnosis
Pathology
Treatment
Prognosis
Bibliography
676.2 Pityriasis Lichenoides
Etiology/Pathogenesis
Clinical Manifestations
Pathology
Differential Diagnosis
Treatment
Bibliography
676.3 Keratosis Pilaris
676.4 Lichen Spinulosus
676.5 Pityriasis Rosea
Etiology/Pathogenesis
Clinical Manifestations
Differential Diagnosis
Treatment
Bibliography
676.6 Pityriasis Rubra Pilaris
Etiology/Pathogenesis
Clinical Manifestations
Differential Diagnosis
Histology
Treatment
Bibliography
676.7 Darier Disease (Keratosis Follicularis)
Etiology/Pathogenesis
Clinical Manifestations
Histology
Differential Diagnosis
Treatment
Bibliography
676.8 Lichen Nitidus
Etiology/Pathogenesis
Clinical Manifestations
Differential Diagnosis
Histology
Treatment
Bibliography
676.9 Lichen Striatus
Etiology/Pathogenesis
Clinical Manifestations
Differential Diagnosis
Treatment
Bibliography
676.10 Lichen Planus
Etiology/Pathogenesis
Clinical Manifestations
Histology
Treatment
Bibliography
676.11 Porokeratosis
Etiology/Pathogenesis
Clinical Manifestations
Histology
Differential Diagnosis
Treatment
Prognosis
Bibliography
676.12 Gianotti-Crosti Syndrome (Papular Acrodermatitis)
Etiology/Pathogenesis
Clinical Manifestations
Histology
Differential Diagnosis
Treatment
Bibliography
676.13 Acanthosis Nigricans
Etiology/Pathogenesis
Clinical Manifestations
Histology
Treatment
Bibliography
Chapter 677: Disorders of Keratinization
Keywords
Disorders of Cornification
Collodion Baby
Nonsyndromic Ichthyoses
Ichthyosis Vulgaris
Etiology/Pathogenesis
Clinical Manifestations
Treatment
X-Linked Ichthyosis
Etiology/Pathogenesis
Clinical Manifestations
Treatment
Autosomal Recessive Congenital Ichthyoses
Harlequin Ichthyosis
Etiology/Pathogenesis
Clinical Manifestations
Treatment
Lamellar Ichthyosis and Congenital Ichthyosiform Erythroderma (Nonbullous Congenital Ichthyosiform Erythroderma)
Etiology/Pathogenesis
Clinical Manifestations
Treatment
Keratinopathic Ichthyoses
Epidermolytic Ichthyosis (Bullous Congenital Ichthyosiform Erythroderma; Epidermolytic Hyperkeratosis)
Etiology/Pathogenesis
Clinical Manifestations
Histopathology
Treatment
Other Nonsyndromic Ichthyoses
Erythrokeratoderma Variabilis
Etiology/Pathogenesis
Clinical Manifestations
Treatment
Symmetric Progressive Erythrokeratoderma
Etiology/Pathogenesis
Clinical Manifestations
Treatment
Syndromic Ichthyoses
Sjögren-Larsson Syndrome
Etiology/Pathogenesis
Clinical Manifestations
Treatment
Netherton Syndrome
Etiology/Pathogenesis
Clinical Manifestations
Treatment
Refsum Syndrome
Etiology/Pathogenesis
Clinical Manifestations
Treatment
Chondrodysplasia Punctata
Etiology/Pathogenesis
Clinical Manifestations
Other Syndromes With Ichthyosis
Palmoplantar Keratodermas
Diffuse Hyperkeratosis of Palms and Soles (Unna-Thost, Vorner)
Mal De Meleda (SLURP-1 Gene)
Vohwinkel Palmoplantar Keratoderma (Mutilating Keratoderma)
Papillon-Lefèvre Syndrome (Cathepsin C Gene)
Other Syndromes
Treatment
Bibliography
Chapter 678: Diseases of the Dermis
Keywords
Keloid
Etiology and Pathogenesis
Clinical Manifestations
Histology
Differential Diagnosis
Treatment
Striae Cutis Distensae (Stretch Marks)
Etiology and Pathogenesis
Clinical Manifestations
Differential Diagnosis
Treatment
Corticosteroid-Induced Atrophy
Etiology and Pathogenesis
Clinical Manifestations
Histology
Treatment
Granuloma Annulare
Etiology and Pathogenesis
Clinical Manifestations
Differential Diagnosis
Histology
Treatment
Necrobiosis Lipoidica
Etiology and Pathogenesis
Clinical Manifestations
Histology
Differential Diagnosis
Treatment
Lichen Sclerosus
Etiology and Pathogenesis
Clinical Manifestations
Differential Diagnosis
Histology
Treatment
Morphea
Etiology and Pathogenesis
Clinical Manifestations
Differential Diagnosis
Histology
Treatment
Scleredema (Scleredema Adultorum, Scleredema of Buschke)
Etiology and Pathogenesis
Clinical Manifestations
Differential Diagnosis
Histology
Treatment
Lipoid Proteinosis (Urbach-Wiethe Disease, Hyalinosis Cutis Et Mucosae)
Etiology and Pathogenesis
Clinical Manifestations
Histology
Treatment
Macular Atrophy (Anetoderma)
Etiology and Pathogenesis
Clinical Manifestations
Histology
Differential Diagnosis
Treatment
Cutis Laxa (Dermatomegaly, Generalized Elastolysis)
Etiology and Pathogenesis
Clinical Manifestations
Histology
Treatment
Pseudoxanthoma Elasticum
Etiology and Pathogenesis
Clinical Manifestations
Pathology
Treatment
Elastosis Perforans Serpiginosa
Etiology and Pathogenesis
Clinical Manifestations
Histology
Differential Diagnosis
Treatment
Reactive Perforating Collagenosis
Etiology and Pathogenesis
Clinical Manifestations
Histology
Differential Diagnosis
Treatment
Xanthomas
Fabry Disease
Mucopolysaccharidoses
Mastocytosis
Etiology and Pathogenesis
Clinical Manifestations
Differential Diagnosis
Prognosis
Treatment
678.1 Mast Cell Activation Syndrome
Keywords
Bibliography
Bibliography
Chapter 679: Ehlers-Danlos Syndrome
Keywords
Classification of the 6 Most Common Subtypes of Ehlers-Danlos Syndrome
Classic (Genes: COL5a1, COL5a2, COL1a1; Previously EDS Type I—Gravis, EDS Type II—Mitis)
Hypermobile (Cause Unknown, Previously EDS Type III)
Vascular (vEDS) (Gene: COL3a1; Previously EDS Type IV)
Kyphoscoliosis (Gene: PLOD [Lysyl Hydroxylase Deficiency]; Previously EDS Type VI)
Arthrochalasia (Gene: COL1a1, COL1a2; Previously EDS Types VIIA and B)
Dermatosparaxis (Type 1 Collagen N-Peptidase; Previously EDS Type VIIC)
Differential Diagnosis
General Approach to Management
Bibliography
Chapter 680: Diseases of Subcutaneous Tissue
Keywords
Corticosteroid-Induced Atrophy
680.1 Panniculitis and Erythema Nodosum
Erythema Nodosum
Etiology and Pathogenesis
Clinical Manifestations
Histology
Treatment
Post-Steroid Panniculitis
Etiology and Pathogenesis
Clinical Manifestations
Histology
Treatment
Lupus Erythematosus Profundus (Lupus Erythematosus Panniculitis)
Etiology and Pathogenesis
Clinical Manifestations
Histology
Treatment
Alpha1-Antitrypsin Deficiency
Etiology and Pathogenesis
Clinical Manifestations
Histology
Treatment
Pancreatic Panniculitis
Etiology and Pathogenesis
Clinical Manifestations
Histology
Treatment
Subcutaneous Fat Necrosis
Etiology and Pathogenesis
Clinical Manifestations
Histology
Differential Diagnosis
Treatment
Sclerema Neonatorum
Etiology and Pathogenesis
Clinical Manifestations
Histology
Treatment
Cold Panniculitis
Etiology and Pathogenesis
Clinical Manifestations
Histology
Differential Diagnosis
Treatment
Chilblains (Pernio)
Etiology and Pathogenesis
Clinical Manifestations
Histology
Differential Diagnosis
Treatment
Factitial Panniculitis
Etiology and Pathogenesis
Clinical Manifestations
Histology
Treatment
Bibliography
680.2 Lipodystrophy
Partial Lipodystrophy
Generalized Lipodystrophy
Bibliography
Chapter 681: Disorders of the Sweat Glands
Keywords
Anhidrosis
Hyperhidrosis
Etiology and Pathogenesis
Clinical Manifestations
Treatment
Miliaria
Etiology and Pathogenesis
Clinical Manifestations
Histology
Differential Diagnosis
Treatment
Bromhidrosis
Hidradenitis Suppurativa
Etiology and Pathogenesis
Clinical Manifestations
Histology
Differential Diagnosis
Treatment
Fox-Fordyce Disease
Etiology and Pathogenesis
Clinical Manifestations
Histology
Treatment
Bibliography
Chapter 682: Disorders of Hair
Keywords
Hypertrichosis
Hypotrichosis and Alopecia
Traumatic Alopecia (Traction Alopecia, Hair Pulling, Trichotillomania)
Traction Alopecia
Hair Pulling
Trichotillomania
Etiology and Pathogenesis.
Clinical Manifestations.
Differential Diagnosis.
Histology.
Treatment.
Alopecia Areata
Etiology and Pathogenesis
Clinical Manifestations
Differential Diagnosis
Histology
Treatment
Acquired Diffuse Hair Loss
Telogen Effluvium
Toxic Alopecia (Anagen Effluvium)
Congenital Diffuse Hair Loss
Structural Defects of Hair
Trichorrhexis Nodosa
Acquired Trichorrhexis Nodosa
Pili Torti
Menkes Kinky Hair Syndrome (Trichopoliodystrophy)
Monilethrix
Trichothiodystrophy
Trichorrhexis Invaginata (Bamboo Hair)
Pili Annulati
Woolly Hair Disease
Uncombable Hair Syndrome (Spun-Glass Hair)
Bibliography
Chapter 683: Disorders of the Nails
Keywords
Abnormalities in Nail Shape or Size
Changes in Nail Color
Nail Separation
Nail Changes Associated With Skin Disease
Trachyonychia (20-Nail Dystrophy)
Nail Infection
Paronychial Inflammation
Paronychial Tumors
Bibliography
Chapter 684: Disorders of the Mucous Membranes
Keywords
Angular Cheilitis
Aphthous Stomatitis (Canker Sores)
Fordyce Spots
Epstein Pearls (Gingival Cysts of the Newborn)
Mucocele
Fissured Tongue
Geographic Tongue (Benign Migratory Glossitis)
Black Hairy Tongue
Oral Hairy Leukoplakia
Acute Necrotizing Ulcerative Gingivitis (Vincent Stomatitis, Fusospirochetal Gingivitis, Trench Mouth)
Noma
Cowden Syndrome (Multiple Hamartoma Syndrome)
Bibliography
Chapter 685: Cutaneous Bacterial Infections
Keywords
685.1 Impetigo
Etiology/Pathogenesis
Clinical Manifestations
Nonbullous Impetigo
Bullous Impetigo
Differential Diagnosis
Complications
Treatment
Bibliography
685.2 Subcutaneous Tissue Infections
Cellulitis
Etiology
Clinical Manifestations
Diagnosis
Treatment
Necrotizing Fasciitis
Etiology
Clinical Manifestations
Diagnosis
Treatment
Prognosis
Bibliography
685.3 Staphylococcal Scalded Skin Syndrome (Ritter Disease)
Etiology and Pathogenesis
Clinical Manifestations
Differential Diagnosis
Histology
Treatment
Bibliography
685.4 Ecthyma
Bibliography
685.5 Other Cutaneous Bacterial Infections
Blastomycosis-Like Pyoderma (Pyoderma Vegetans)
Blistering Distal Dactylitis
Perianal Infectious Dermatitis
Erysipelas
Folliculitis
Abscesses and Furuncles
Etiology
Clinical Manifestations
Treatment
Pitted Keratolysis
Erythrasma
Erysipeloid
Tuberculosis of the Skin
Bibliography
Chapter 686: Cutaneous Fungal Infections
Keywords
Tinea Versicolor
Etiology
Clinical Manifestations
Differential Diagnosis
Treatment
Dermatophytoses
Etiology
Epidemiology
Tinea Capitis
Clinical Manifestations
Differential Diagnosis
Treatment
Tinea Corporis
Clinical Manifestations
Differential Diagnosis
Treatment
Tinea Cruris
Clinical Manifestations
Differential Diagnosis
Treatment
Tinea Pedis
Clinical Manifestations
Differential Diagnosis
Treatment
Tinea Unguium
Clinical Manifestations
Differential Diagnosis
Treatment
Tinea Nigra Palmaris
Candidal Infections (Candidosis, Candidiasis, and Moniliasis)
Oral Candidiasis (Thrush)
Vaginal Candidiasis
Congenital Cutaneous Candidiasis
Candidal Diaper Dermatitis
Intertriginous Candidiasis
Perianal Candidiasis
Candidal Paronychia and Onychia
Candidal Granuloma
Bibliography
Chapter 687: Cutaneous Viral Infections
Keywords
Wart (Verruca)
Etiology
Clinical Manifestations
Histology
Differential Diagnosis
Treatment
Molluscum Contagiosum
Etiology
Clinical Manifestations
Differential Diagnosis
Histology
Treatment
Bibliography
Chapter 688: Arthropod Bites and Infestations
Keywords
688.1 Arthropod Bites
Clinical Manifestations
Treatment
Bibliography
Arthropod Bites
688.2 Scabies
Etiology and Pathogenesis
Clinical Manifestations
Differential Diagnosis
Treatment
Norwegian (Crusted) Scabies
Canine Scabies
Other Types of Scabies
Bibliography
Scabies
688.3 Pediculosis
Bibliography
Pediculosis
688.4 Seabather’s Eruption
Chapter 689: Acne
Keywords
Acne Vulgaris
Pathogenesis
Clinical Manifestations
Treatment
Diet
Climate
Cleansing
Topical Therapy
Retinoids.
Benzoyl Peroxide.
Topical Antibiotics.
Azelaic Acid.
Systemic Therapy
Surgical Therapy
Drug-Induced Acne
Halogen Acne
Chloracne
Neonatal Acne
Infantile Acne
Mid-Childhood Acne
Tropical Acne
Acne Conglobata
Acne Fulminans (Acute Febrile Ulcerative Acne)
Bibliography
Chapter 690: Tumors of the Skin
Keywords
Epidermal Inclusion Cyst (Epidermoid Cyst)
Milium
Fibrofolliculomas
Pilar Cyst (Trichilemmal Cyst)
Pilomatricoma (Pilomatrixoma)
Trichoepithelioma
Eruptive Vellus Hair Cysts
Steatocystoma Multiplex
Syringoma
Infantile Digital Fibroma
Dermatofibroma (Histiocytoma)
Juvenile Xanthogranuloma
Lipoma
Basal Cell Carcinoma
Nevoid Basal Cell Carcinoma Syndrome (Basal Cell Nevus Syndrome, Gorlin Syndrome)
Melanoma
Mucosal Neuroma Syndrome (Multiple Endocrine Neoplasia Type Iib)
Bibliography
Chapter 691: Nutritional Dermatoses
Keywords
Acrodermatitis Enteropathica
Essential Fatty Acid Deficiency
Kwashiorkor
Cystic Fibrosis
Pellagra
Scurvy (Vitamin C or Ascorbic Acid Deficiency)
Vitamin a Deficiency
Bibliography
Part XXXI: Bone and Joint Disorders
Section 1: Orthopedic Problems
Chapter 692: Growth and Development
Keywords
In Utero Positioning
Growth and Development
Centers of Ossification
Anatomic Locations: Descriptive Terms
Important Growth and Developmental Milestones
Growth Patterns in Upper and Lower Extremities
Gait/Functional Maturation
Bibliography
Chapter 693: Orthopedic Evaluation of the Child
Keywords
History
Physical Examination
Inspection
Palpation
Range of Motion
Gait Assessment
Limping
Back Pain
Neurologic Evaluation
Radiographic Assessment
Plain Radiographs
Ultrasonography
Magnetic Resonance Imaging
Magnetic Resonance Angiography
Computed Tomography
Nuclear Medicine Imaging
Laboratory Studies
Bibliography
Chapter 694: The Foot and Toes
Keywords
694.1 Metatarsus Adductus
Clinical Manifestations
Radiographic Evaluation
Treatment
Bibliography
694.2 Calcaneovalgus Feet
Clinical Manifestations
Radiographic Evaluation
Treatment
Bibliography
694.3 Talipes Equinovarus (Clubfoot)
Clinical Manifestations
Radiographic Evaluation
Treatment
Bibliography
694.4 Congenital Vertical Talus
Clinical Manifestations
Radiographic Evaluation
Treatment
Bibliography
694.5 Hypermobile Pes Planus (Flexible Flatfeet)
Clinical Manifestations
Radiographic Evaluation
Treatment
Bibliography
694.6 Tarsal Coalition
Clinical Manifestations
Radiographic Evaluation
Treatment
Bibliography
694.7 Cavus Feet
Treatment
Bibliography
694.8 Osteochondroses/Apophysitis
Bibliography
694.9 Puncture Wounds of the Foot
Bibliography
694.10 Toe Deformities
Juvenile Hallux Valgus (Bunion)
Clinical Manifestations
Radiographic Evaluation
Treatment
Curly Toes
Overlapping 5Th Toe
Polydactyly
Syndactyly
Hammer Toe
Mallet Toe
Claw Toe
Annular Bands
Macrodactyly
Subungual Exostosis
Ingrown Toenail
Bibliography
694.11 Painful Foot
694.12 Shoes
Bibliography
Chapter 695: Torsional and Angular Deformities of the Limb
695.1 Normal Limb Development
Keywords
Bibliography
695.2 Evaluation
Keywords
Foot Progression Angle
Femoral Anteversion
Tibial Rotation
Foot Shape and Position
Bibliography
695.3 Torsional Deformities
Keywords
Femoral Anteversion
Medial Tibial Torsion
External Femoral Torsion
Lateral Tibial Torsion
Metatarsus Adductus
Bibliography
695.4 Coronal Plane Deformities
Keywords
Genu Varum
Tibia Vara
Genu Valgum (Knock-Knees)
Bibliography
695.5 Congenital Angular Deformities of the Tibia and Fibula
Keywords
Posteromedial Tibial Bowing
Anteromedial Tibial Bowing (Postaxial Hemimelia)
Anterolateral Tibial Bowing
Tibial Longitudinal Deficiency
Bibliography
Chapter 696: Leg-Length Discrepancy
Keywords
Diagnosis and Clinical Findings
Radiographic Evaluation
Treatment
Bibliography
Chapter 697: The Knee
Keywords
Normal Development of the Knee
Anatomy and Range of Motion
697.1 Discoid Lateral Meniscus
Clinical Manifestations and Diagnosis
Treatment
Bibliography
697.2 Popliteal Cysts (Baker Cysts)
Clinical Manifestations and Diagnosis
Treatment
Bibliography
697.3 Juvenile Osteochondritis Dissecans
Clinical Manifestations and Diagnosis
Treatment
Bibliography
697.4 Osgood-Schlatter Disease and Sinding-Larsen-Johansson Syndrome
Clinical Manifestations and Diagnosis
Treatment
Bibliography
697.5 Patellofemoral Pain Syndrome
Clinical Manifestations and Diagnosis
Treatment
Bibliography
697.6 Patellofemoral Instability
Clinical Manifestations and Diagnosis
Treatment
Bibliography
697.7 Anterior Cruciate Ligament Rupture
Clinical Manifestations and Diagnosis
Treatment
Bibliography
Chapter 698: The Hip
Keywords
Growth and Development
Vascular Supply
698.1 Developmental Dysplasia of the Hip
Keywords
Classification
Etiology and Risk Factors
Epidemiology
Pathoanatomy
Clinical Findings
The Neonate
The Infant
The Walking Child
Diagnostic Testing
Ultrasonography
Radiography
Treatment
Newborns and Infants Younger Than 6 Months
Children 6 Months to 2 Years of Age
Children Older Than 2 Years
Complications
Bibliography
698.2 Transient Monoarticular Synovitis (Toxic Synovitis)
Keywords
Etiology
Clinical Manifestations
Diagnosis
Treatment
Bibliography
698.3 Legg-Calvé-Perthes Disease
Keywords
Etiology
Epidemiology
Pathogenesis
Clinical Manifestations
Diagnosis
Classification
Natural History and Prognosis
Treatment
Bibliography
698.4 Slipped Capital Femoral Epiphysis
Keywords
Classification
Etiology and Pathogenesis
Epidemiology
Clinical Manifestations
Diagnostic Studies
Treatment
Complications
Bibliography
Chapter 699: The Spine
Keywords
Normal Spinal Curvatures
699.1 Idiopathic Scoliosis
Keywords
Definition
Etiology
Epidemiology
Classification of Idiopathic Scoliosis
Clinical Presentation of Idiopathic Scoliosis
Physical Examination of Idiopathic Scoliosis
Radiographic Evaluation of Idiopathic Scoliosis
Natural History of Idiopathic Scoliosis
Treatment of Idiopathic Scoliosis
Bibliography
699.2 Congenital Scoliosis
Keywords
Definition
Etiology
Associated Conditions
Classification of Congenital Scoliosis
Natural History of Congenital Scoliosis
Treatment of Congenital Scoliosis
Special Circumstance: Thoracic Insufficiency Syndrome
Bibliography
699.3 Neuromuscular Scoliosis, Genetic Syndromes, and Compensatory Scoliosis
Keywords
Neuromuscular Scoliosis
Syndromes and Genetic Disorders
Compensatory Scoliosis
Bibliography
699.4 Kyphosis
Keywords
Flexible Kyphosis (Postural Kyphosis)
Structural Kyphosis
Scheuermann Kyphosis
Physical Exam and Clinical Manifestations
Radiographic Evaluation
Natural History
Treatment
Congenital Kyphosis
Bibliography
699.5 Back Pain in Children
Keywords
Clinical Evaluation
Medical Decision Making
Radiographic and Laboratory Evaluation
Bibliography
699.6 Spondylolysis and Spondylolisthesis
Keywords
Clinical Manifestations
Physical Exam
Radiographic Evaluation
Treatment
Bibliography
699.7 Spine Infection
Keywords
Clinical Manifestations
Radiographic Evaluation
Treatment
Bibliography
699.8 Intervertebral Disc Herniation/Slipped Vertebral Apophysis
Keywords
Apophysis
Etiology
Clinical Manifestations
Radiographic Evaluation
Treatment
Bibliography
699.9 Tumors
Keywords
Bibliography
Chapter 700: The Neck
700.1 Torticollis
Keywords
Congenital Muscular Torticollis
Other Causes of Torticollis
Atlantoaxial Rotatory Displacement
Bibliography
700.2 Klippel-Feil Syndrome
Keywords
Etiology and Classification
Clinical Presentation
Physical Examination
Radiologic Investigation
Treatment
Bibliography
700.3 Cervical Anomalies and Instabilities
Keywords
OS Odontoideum
Down Syndrome
22Q11.2 Deletion Syndrome
Bibliography
Chapter 701: The Upper Limb
Keywords
Shoulder
Sprengel Deformity
Treatment
Congenital Pseudoarthrosis of the Clavicle
Treatment
Elbow
Panner Disease
Treatment
Radial Longitudinal Deficiency
Treatment
Nursemaid Elbow
Treatment
Wrist
Madelung Deformity
Treatment
Gymnast’s Wrist
Treatment
Ganglion
Treatment
Hand
Camptodactyly
Treatment
Clinodactyly
Treatment
Polydactyly
Treatment
Thumb Hypoplasia
Treatment
Syndactyly
Treatment
Fingertip Injuries
Trigger Thumb and Fingers
Treatment
Bibliography
Chapter 702: Arthrogryposis
Keywords
Etiology
Neurologic Abnormalities
Muscular Abnormalities
Limited Intrauterine Spacing
Connective Tissue Abnormalities
Maternal Diseases
Intrauterine Vascular Compromise
Classification
Management of Orthopedic Problems of Arthrogryposis
Foot Problems
Knee Problems
Hip Problems
Ambulation
Upper-Extremity Problems
Surgery of the Upper Extremity
Shoulder
Elbow
Wrist
Scoliosis
Surgical Staging
Bibliography
Chapter 703: Common Fractures
Keywords
703.1 Unique Characteristics of Pediatric Fractures
Keywords
Fracture Remodeling
Overgrowth
Progressive Deformity
Rapid Healing
Bibliography
703.2 Pediatric Fracture Patterns
Keywords
Plastic Deformation
Buckle or Torus Fracture
Greenstick Fracture
Complete Fractures
Epiphyseal Fractures
Child Abuse
Bibliography
703.3 Upper Extremity Fractures
Keywords
Phalangeal Fractures
Forearm Fractures
Distal Humeral Fractures
Proximal Humerus Fractures
Clavicular Fractures
Bibliography
703.4 Lower Extremity Fractures
Keywords
Hip Fracture
Femoral Shaft Fractures
Proximal Tibia Fractures
Tibia and Fibula Shaft Fractures
Toddler Fracture
Triplane and Tillaux Fractures
Metatarsal Fractures
Toe Phalangeal Fractures
Bibliography
703.5 Operative Treatment of Fractures
Keywords
Surgical Techniques
Bibliography
703.6 Complications of Fractures in Children
Keywords
Complications Resulting From Injury
Complications of Treatment
Late Complications of Trauma
Chapter 704: Osteomyelitis
Keywords
Etiology
Epidemiology
Pathogenesis
Clinical Manifestations
Diagnosis
Radiographic Evaluation
Plain Radiographs
Magnetic Resonance Imaging and Computed Tomography
Radionuclide Studies
Differential Diagnosis
Treatment
Antimicrobial Therapy
Surgical Therapy
Physical Therapy
Prognosis
Bibliography
Chapter 705: Septic Arthritis
Keywords
Etiology
Epidemiology
Pathogenesis
Clinical Manifestations
Diagnosis
Radiographic Evaluation
Plain Radiographs
Ultrasonography
Magnetic Resonance Imaging and Computed Tomography
Radionuclide Imaging
Differential Diagnosis
Treatment
Antimicrobial Therapy
Surgical Therapy
Prognosis
Bibliography
Section 2: Sports Medicine
Chapter 706: Epidemiology and Prevention of Injuries
Keywords
Preparticipation Sports Examination
History and Physical Examination
Bibliography
Chapter 707: Management of Musculoskeletal Injury
Keywords
Mechanism of Injury
Acute Injuries
Overuse Injuries
Initial Evaluation of the Injured Extremity
Transition From Immediate Management to Return to Play
Phase 1
Phase 2
Phase 3
Phase 4
Relative Rest and Return-to-Play Guidelines
Imaging
Differential Diagnoses of Musculoskeletal Pain
707.1 Growth Plate Injuries
Bibliography
707.2 Shoulder Injuries
Clavicle Fractures
Acromioclavicular Joint Separation
Anterior Glenohumeral Dislocation
Rotator Cuff Injury
Bibliography
707.3 Elbow Injuries
Acute Injuries
Chronic Injuries
Bibliography
707.4 Low Back Injuries
Spondylolysis, Spondylolisthesis, and Facet Syndrome
Spondylolysis
Spondylolisthesis and Facet Syndrome
Lumbar Disk Herniation, Strain, and Contusion
Other Causes
Bibliography
707.5 Hip and Pelvis Injuries
Bibliography
707.6 Knee Injuries
Initial Treatment of Acute Knee Injuries
Chronic Injuries
Patellofemoral Stress Syndrome
Osgood-Schlatter Disease
Other Chronic Injuries
Bibliography
707.7 Lower Leg Exertional Pain: Shin Splints, Stress Fractures, and Chronic Compartment Syndrome
Bibliography
707.8 Ankle Injuries
Examination and Injury Grading Scale
Radiographs
Initial Treatment of Ankle Sprains
Rehabilitation
Bibliography
707.9 Foot Injuries
Bibliography
Bibliography
Chapter 708: Sports-Related Traumatic Brain Injury (Concussion)
Keywords
Epidemiology
Pathophysiology
Assessment of the Injured Player
Management and Treatment
Initial Phase
Returning to Sport
Prevention
Bibliography
Chapter 709: Cervical Spine Injuries
Keywords
Soft Tissue Injury
Spear Tackler’s Spine
Cervical Fractures
Stingers (Burners)
Transient Quadriparesis
Congenital Spinal Stenosis
Spinal Cord Injury
Bibliography
Chapter 710: Heat Injuries
Keywords
Bibliography
Chapter 711: Female Athletes
Keywords
Bibliography
Chapter 712: Performance-Enhancing Aids
Keywords
Bibliography
Chapter 713: Specific Sports and Associated Injuries
Keywords
Sports Participation, Early Specialization, Injury Risk and Burnout
Football
Baseball/Softball
Basketball/Volleyball
Tennis
Lacrosse
Swimming/Diving
Soccer
Ice Hockey
Field Hockey
Skiing and Snowboarding
Skateboarding
Cycling and Motocross
Wrestling
Running
Cheerleading
Gymnastics
Dance
Adaptive Sports
Bibliography
Sports Participation, Early Specialization, Injury Risk and Burnout
Football
Baseball/Softball
Basketball/Volleyball
Tennis
Lacrosse
Swimming/Diving
Soccer
Ice Hockey
Field Hockey
Skiing and Snowboarding
Skateboarding
Cycling and Motocross
Wrestling
Running
Cheerleading
Gymnastics
Dance
Adaptive Sports
Section 3: The Skeletal Dysplasias
Chapter 714: General Considerations in Skeletal Dysplasias
Keywords
Clinical Manifestations
Growth
Non-Growth-Related Manifestations
Family and Reproductive History
Radiographic Features
Diagnosis
Molecular Genetics
Pathophysiology
Treatment
Bibliography
Chapter 715: Disorders Involving Cartilage Matrix Proteins
Keywords
Spondyloepiphyseal Dysplasias/Type 2 Collagenopathies
Lethal Spondyloepiphyseal Dysplasias
Spondyloepiphyseal Dysplasia Congenita
Kniest Dysplasia
Late-Onset Spondyloepiphyseal Dysplasia
Aggrecan-Related Spondyloepiphyseal Dysplasias
Stickler Syndrome/Dysplasia (Hereditary Osteoarthroophthalmopathy)
Schmid Metaphyseal Dysplasia
Pseudoachondroplasia and Multiple Epiphyseal Dysplasia
Bibliography
Chapter 716: Disorders Involving Transmembrane Receptors
Keywords
Achondroplasia Group
Thanatophoric Dysplasia
Achondroplasia
Diagnosis
Clinical Manifestations
Genetics
Hypochondroplasia
Jansen Metaphyseal Dysplasia
Bibliography
Chapter 717: Disorders Involving Ion Transporters
Keywords
Autosomal Recessive Multiple Epiphyseal Dysplasia
Diastrophic Dysplasia
Achondrogenesis Type 1B and Atelosteogenesis Type 2
Bibliography
Chapter 718: Disorders Involving Transcription Factors
Keywords
Campomelic Dysplasia
Cleidocranial Dysplasia
Nail-Patella Syndrome
Bibliography
Chapter 719: Disorders Involving Defective Bone Resorption
Keywords
Osteopetrosis
Clinical Manifestations
Treatment
Pyknodysostosis
Bibliography
Chapter 720: Other Inherited Disorders of Skeletal Development
Keywords
Ellis–Van Creveld Syndrome
Asphyxiating Thoracic Dystrophy (See Also Chapter 445.3)
Short-Rib Polydactyly Syndromes
Cartilage-Hair Hypoplasia–Anauxetic Spectrum Disorders
Cartilage-Hair Hypoplasia
Metatropic Dysplasia
Spondylometaphyseal Dysplasia, Kozlowski Type
Disorders Involving Filamins
Juvenile Osteochondroses
Caffey Disease (Infantile Cortical Hyperostosis)
Fibrodysplasia Ossificans Progressiva
Bibliography
Chapter 721: Osteogenesis Imperfecta
Keywords
Etiology
Epidemiology
Pathology
Pathogenesis
Clinical Manifestations
Osteogenesis Imperfecta Type I (Mild)
Osteogenesis Imperfecta Type II (Perinatal Lethal)
Osteogenesis Imperfecta Type III (Progressive Deforming)
Osteogenesis Imperfecta Type IV (Moderately Severe)
Osteogenesis Imperfecta Type V (Hyperplastic Callus) and Type VI Hyperosteoidosis (Mineralization Defect)
Osteogenesis Imperfecta Types VII, VIII, and IX (Autosomal Recessive)
Osteogenesis Imperfecta Types X and XI (Autosomal Recessive)
High Bone Mass Osteogenesis Imperfecta (Cleavage of the Procollagen C-Propeptide)
Defects in Osteoblast Differentiation (Types XIII-XVIII OI)
Other Genes for Osteogenesis Imperfecta
Laboratory Findings
Complications
Treatment
Prognosis
Genetic Counseling
Bibliography
Chapter 722: Marfan Syndrome
Keywords
Epidemiology
Pathogenesis
Clinical Manifestations
Skeletal System
Cardiovascular System
Ocular System
Other Systems
Diagnosis
Differential Diagnosis
Aortic Aneurysm Syndromes
Ectopia Lentis Syndromes
Syndromes With Systemic Manifestations of MFS
Laboratory Findings
Management
Current Therapies
Activity Restrictions
Aortic Surgery
Pregnancy
Endocarditis Prophylaxis
Current Pharmacological Approaches
Emerging Therapeutic Strategies
Angiotensin II Receptor Type 1 Blockers
ACE Inhibitors
ERK Inhibitors
Prognosis
Genetic Counseling
Bibliography
Section 4: Metabolic Bone Disease
Chapter 723: Bone Structure, Growth, and Hormonal Regulation
Keywords
Bibliography
Chapter 724: Hypophosphatasia
Keywords
Bibliography
Chapter 725: Hyperphosphatasia
Keywords
Bibliography
Chapter 726: Osteoporosis
Keywords
Bibliography
Part XXXII: Rehabilitation Medicine
Chapter 727: Evaluation of the Child for Rehabilitative Services
Keywords
Child Characteristics
Family Characteristics
The Physical Environment
The Previously Healthy Child
Chapter 728: Rehabilitation for Severe Traumatic Brain Injury
Keywords
Pathophysiology
Severity
Medical Complications
Disorders of Consciousness
Cognitive-Behavioral Disorders
Agitation
Posttraumatic Seizure
Paroxysmal Sympathetic Hyperactivity
Neuroendocrine Disorders
Spasticity
Outcomes Associated With Severe Traumatic Brain Injury
Bibliography
Chapter 729: Spinal Cord Injury and Autonomic Dysreflexia Management
Keywords
Clinical Manifestations
Prognosis
Bibliography
Chapter 730: Spasticity
Keywords
Oral Medications
GABAergic Medications
α2-Adrenergic Agents
Peripherally Acting Calcium Blockers
Surgical Management
Bibliography
Chapter 731: Birth Brachial Plexus Palsy
Keywords
Physical Examination
Evaluation
Treatment
Bibliography
Chapter 732: Meningomyelocele (Spina Bifida)
Keywords
Etiology
Prevention
Prenatal Screening
Clinical Implications
Adolescence and Transition to Adulthood
Bibliography
Chapter 733: Ambulation Assistance
Keywords
Orthoses
Prostheses
Assistive Mobility Devices
Wheelchair
Bibliography
Chapter 734: Health and Wellness for Children With Disabilities
Keywords
Health Promotion Definitions and Background for Disability
Anticipatory Guidance, Counseling, and Preventive Care
Physical Activity and Exercise
Nutrition and Obesity
Emotional Health and Leisure Activities
Dental Care
Role of Healthcare Providers
734.1 Home Mechanical Ventilation and Technology Dependence
Keywords
Preparation
Community Resources
Subacute Care
Emergency and Acute Care
Quality of Life
Bibliography
Bibliography
Part XXXIII: Environmental Health
Chapter 735: Overview of Environmental Health and Children
Keywords
Global Climate Change
Localized Environmental Hazards
Toxins Versus Toxicants
Mycotoxins
Pediatric Conditions Linked to Mycotoxin Exposures
Neural Tube Defects
Infant Pulmonary Hemorrhage
Food-Borne Diseases Caused by Environmental Exposures
Aflatoxins
Ochratoxin A
Fumonisins
Deoxynivalenol
Bibliography
Chapter 736: Biologic Effects of Ionizing Radiation on Children
Keywords
Basic Principles
Biologic Effects of Radiation
Radiation Exposure in Diagnostic Imaging of Children
Decreasing Unnecessary Diagnostic Radiation in Children While Still Obtaining Diagnostic Images
Reducing Radiation From the CT Examination
Radiation Therapy—Acute and Late Effects
Whole-Body Irradiation
Uncontrolled Large- or Small-Scale Exposure to Radiation
Clinical Manifestations
Treatment
Localized Irradiation
Clinical Manifestations
Treatment
Internal Contamination
Epidemiology
Clinical Manifestations
Treatment
External Contamination
Bibliography
Chapter 737: Chemical Pollutants
Keywords
Synthetic Chemicals and Human Health
Children’s Unique Susceptibility to Synthetic Chemicals
Safety Testing of Synthetic Chemicals
Synthetic Chemicals and Disease in Children
Chemical Pollutants of Major Concern
Air Pollutants
Health Hazards of Unconventional Natural Gas Development (Fracking)
Lead
Mercury
Asbestos
Pesticides
Polychlorinated Biphenyls, DDT, Dioxins, Brominated Flame Retardants, and Other Halogenated Hydrocarbons
Endocrine Disruptors
Environmental Carcinogens
Routes of Exposure
Transplacental.
Water.
Air.
Food.
Work Clothes.
School.
Child Labor.
The Physician’s Role
737.1 Tobacco
Keywords
Composition of Second-Hand Smoke and Toxicities
Tobacco Use and Exposure Is A Health Risk Disparity
Maternal Smoking During Pregnancy and Tobacco Smoke Exposure During Pregnancy
Maternal Smoking During Pregnancy and Lung Development
Postnatal Second-Hand Smoke Exposure—Effects on the Child
Treatment for Second-Hand Tobacco Smoke Exposure
Bibliography
Bibliography
Chapter 738: Heavy Metal Intoxication
Keywords
Arsenic
Epidemiology
Pharmacokinetics
Pathophysiology
Clinical Manifestations
Laboratory Findings
Mercury
Epidemiology
Pharmacokinetics
Pathophysiology
Clinical Manifestations
Laboratory Findings
Treatment of Arsenic and Mercury Intoxication
Bibliography
Chapter 739: Lead Poisoning
Keywords
Public Health History
Sources of Exposure
Metabolism
Clinical Effects
Clinical Symptoms
Diagnosis
Screening
Other Tools for Assessment
Treatment
Bibliography
Chapter 740: Nonbacterial Food Poisoning
740.1 Mushroom Poisoning
Keywords
Gastrointestinal: Delayed Onset
Amanita Poisoning
Treatment
Monomethylhydrazine Intoxication
Treatment
Renal: Delayed Onset
Orellanine Poisoning
Treatment
Autonomic Nervous System: Rapid Onset
Muscarine Poisoning
Treatment
Coprine Ingestion
Central Nervous System: Rapid Onset
Isoxazole Intoxication
Indole Intoxication
Gastrointestinal: Rapid Onset
Bibliography
740.2 Solanine Poisoning
Keywords
Bibliography
740.3 Seafood Poisoning
Keywords
Ciguatera Fish Poisoning
Treatment
Scombroid (Pseudoallergic) Fish Poisoning
Treatment
Paralytic Shellfish Poisoning
Treatment
Neurotoxic Shellfish Poisoning
Treatment
Diarrhetic Shellfish Poisoning
Amnesic Shellfish Poisoning
Pufferfish Poisoning
Azaspiracid Poisoning
Bibliography
Ciguatera Fish Poisoning
Scombroid Fish Poisoning
Shellfish Poisoning
Fish Poisoning
740.4 Melamine Poisoning
Keywords
Bibliography
Chapter 741: Biologic and Chemical Terrorism*
Keywords
Etiology
Epidemiology and Pediatric-Specific Concerns
Clinical Manifestations
Sudden-Onset Neuromuscular Syndrome: Nerve Agents
Delayed-Onset Neuromuscular Syndrome: Botulism
Sudden-Onset Respiratory Syndrome: Chlorine, Phosgene, and Cyanide
Delayed-Onset Respiratory Syndrome: Anthrax, Plague, Tularemia, and Ricin
Intermediate-Onset Dermatologic Syndrome: Mustard and Lewisite
Delayed-Onset Dermatologic Syndrome: Smallpox
Diagnosis
Prevention
Treatment
Bibliography
Chapter 742: Mass Psychogenic Illness
Keywords
Clinical Features and Diagnosis
Treatment Strategies
Bibliography
Chapter 743: Animal and Human Bites
Keywords
Epidemiology
Clinical Manifestations
Diagnosis
Complications
Treatment
Prevention
Bibliography
Chapter 744: Rat Bite Fever
Keywords
Etiology
Clinical Course
Diagnosis
Treatment
Bibliography
Chapter 745: Monkeypox
Keywords
Etiology
Clinical Course
Treatment
Bibliography
Chapter 746: Envenomations
Keywords
General Approach to the Envenomated Child
General Management
General Wound Care
Snake Bites
Venoms and Effects
Management
Disposition
Spider Bites
Latrodectus Spiders
Venoms and Effects
Management
Loxosceles Spiders
Venoms and Effects
Management
Disposition
Scorpion Stings
Venoms and Effects
Management
Disposition
Hymenoptera Stings
Venoms and Effects
Management
Disposition
Marine Envenomation
Venoms and Effects
Management
Disposition
Bibliography
Part XXXIV: Laboratory Medicine
Chapter 747: Laboratory Testing in Infants and Children
Accuracy and Precision of Laboratory Tests
Sensitivity, Accuracy, and Analytic Testing
Predictive Value of Laboratory Tests
Neonatal Screening Tests
Testing in Refining a Differential Diagnosis
Serologic Testing
Laboratory Screening
Bibliography
Chapter 748: Reference Intervals for Laboratory Tests and Procedures
Bibliography (for Table 748.5)