Kendig and Wilmott’s Disorders of the Respiratory Tract in Children

10th Edition - August 21, 2023
Editors: Andrew Bush, Robin R Deterding, Albert Li, Felix Ratjen, Peter Sly, Heather Zar, Robert W. Wilmott
Language: English
Hardback ISBN:
9 7 8 - 0 - 3 2 3 - 8 2 9 1 5 - 1
eBook ISBN:
9 7 8 - 0 - 3 2 3 - 8 2 9 1 6 - 8

Extensively revised from cover to cover, Kendig and Wilmott's Disorders of the Respiratory Tract in Children, 10th Edition, continues to be your #1 choice for reliable, up-to-date… Read more

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Extensively revised from cover to cover, Kendig and Wilmott's Disorders of the Respiratory Tract in Children, 10th Edition, continues to be your #1 choice for reliable, up-to-date information on all aspects of pediatric respiratory disorders. This highly respected reference is accessible to specialists and primary care providers alike, with coverage of both common and less common respiratory problems found in the newborn and child. Detailed and thorough, this edition covers basic science and its relevance to today’s clinical issues as well as treatment, management, and outcomes information, making it an ideal resource for day-to-day practice as well as certification or recertification review and other professional examinations such as pHERMES.

Instructions for online access
Cover image
Title page
Table of Contents
Copyright
List of Contributors
Preface
Section 1. Scientific Basis of Pediatric Pulmonology
1. Molecular and Cellular Determinants of Lung Morphogenesis
Introduction
Organogenesis of the Lung
Molecular Mechanisms Directing Lung Development
Development of the Pulmonary Host Defense Systems
Gene Mutations in Lung Development and Function
Summary
2. Basic Genetics and Epigenetics of Childhood Lung Disease
INTRODUCTION
TYPES OF GENETIC VARIATION
TECHNOLOGIES TO IDENTIFY GENETIC VARIATION
INTERPRETATION OF GENETIC VARIATION
WHEN TO CONSIDER CLINICAL GENETIC TESTING
RESEARCH STUDY DESIGNS TO ATTRIBUTE GENETIC VARIATION TO DISEASE
EPIGENETICS—TERMINOLOGY AND TECHNOLOGY
“MULTI-OMICS” APPROACHES TO REFINE GENOTYPE-PHENOTYPE ASSOCIATIONS
3. Big Data and Omics in Respiratory Diseases:: An Analytical Perspective
INTRODUCTION
THE OMICS REVOLUTION IN RESPIRATORY DISEASES
BIG DATA ANALYTICS TO HARNESS MULTI-OMICS DATA
CONCLUDING REMARKS
4. The Surfactant System
SURFACTANT COMPOSITION AND METABOLISM
SURFACTANT FUNCTION
SURFACTANT DEFICIENCY
SURFACTANT TREATMENT OF SURFACTANT DEFICIENCY
5. The Structural and Physiologic Basis of Respiratory Disease
NORMAL LUNG ANATOMY AND CELL FUNCTION
AIRWAYS
ALVEOLAR REGION
PULMONARY VASCULAR SYSTEM
LYMPHATIC SYSTEM
INNERVATION OF THE LUNG
INTERSTITIUM
GROWTH AND DEVELOPMENT OF THE LUNG
THE LUNG AT BIRTH
POSTNATAL LUNG GROWTH
VENTILATION AND MECHANICS OF BREATHING
DEFINITIONS AND SYMBOLS
ELASTIC RECOIL OF THE LUNG
COMPLIANCE OF THE LUNG
ELASTIC PROPERTIES OF THE CHEST WALL
LUNG VOLUMES
REGIONAL LUNG VOLUMES
DYNAMIC (FLOW-RESISTIVE) PROPERTIES OF THE LUNG
DISTRIBUTION OF VENTILATION
PULMONARY CIRCULATION
PULMONARY VASCULAR PRESSURES
PULMONARY VASCULAR RESISTANCE
DISTRIBUTION OF BLOOD FLOW
METHODS OF EVALUATING THE PULMONARY CIRCULATION
Muscles of Respiration
GAS EXCHANGE
ALVEOLAR VENTILATION
DEAD SPACE
DIFFUSION
SHUNT AND VENTILATION-PERFUSION RELATIONSHIPS
SYSTEMIC GAS TRANSPORT
OXYGEN THERAPY
CARBON DIOXIDE TRANSPORT AND ACID-BASE BALANCE
TISSUE RESPIRATION
REGULATION OF RESPIRATION
SENSORY FEEDBACK SYSTEM
METABOLIC FUNCTIONS OF THE LUNG
Acknowledgments
6. Biology and Assessment of Airway Inflammation
INTRODUCTION
ALLERGIC INFLAMMATION
ACUTE INFLAMMATION
CHRONIC INFLAMMATION
INFLAMMATORY CELLS
STRUCTURAL CELLS AS SOURCES OF MEDIATORS
INFLAMMATORY MEDIATORS
NEURAL MECHANISMS
TRANSCRIPTION FACTORS
ANTIINFLAMMATORY MECHANISMS
DIRECT MEASUREMENTS OF AIRWAY INFLAMMATION
NONINVASIVE ASSESSMENT OF AIRWAY INFLAMMATION
IS AIRWAY HYPERRESPONSIVENESS AN INFLAMMATORY SURROGATE?
OTHER POTENTIAL INDIRECT INFLAMMATORY MARKERS
IS THERE A ROLE FOR ASSESSING INFLAMMATION IN PEDIATRIC RESPIRATORY DISEASE?
THERAPEUTIC IMPLICATIONS
CONCLUSION
7. Lung Defenses: Intrinsic, Innate, and Adaptive
INTRINSIC LUNG DEFENSES
INNATE LUNG DEFENSES
CELLULAR DEFENSES: AT THE CROSSROADS OF INNATE AND ADAPTIVE IMMUNITY
ADAPTIVE LUNG DEFENSES
ROLE OF PROGRAMMED CELL DEATH IN LUNG GROWTH, INJURY, AND REPAIR
SUMMARY
Section 2. Clinical Tools of Pediatric Pulmonology
8. History, Physical Examination, and Strategies for Remote Monitoring
THE PEDIATRIC RESPIRATORY HISTORY
THE PATIENT AND THEIR FAMILY
9. Bronchoscopy and Bronchoalveolar Lavage in Pediatric Patients
THE SCOPE OF BRONCHOSCOPY
INSTRUMENTATION
INDICATIONS FOR BRONCHOSCOPY
CONTRAINDICATIONS TO BRONCHOSCOPY
TECHNIQUES FOR BRONCHOSCOPY
ANESTHESIA FOR BRONCHOSCOPY
BRONCHOALVEOLAR LAVAGE
DIAGNOSTIC TECHNIQUES OTHER THAN BRONCHOALVEOLAR LAVAGE
THERAPEUTIC BRONCHOSCOPY
THERAPEUTIC ASPIRATION
BRONCHOPLEURAL FISTULA—DIAGNOSIS AND MANAGEMENT
COMPLICATIONS OF BRONCHOSCOPY
ECONOMIC CONSIDERATIONS IN BRONCHOSCOPY
10. Diagnostic Imaging of the Respiratory Tract
PLAIN RADIOGRAPHY
SPECIFIC FEATURES OF THE CHEST RADIOGRAPH IN CHILDREN
SYSTEMATIC REVIEW OF THE CHEST RADIOGRAPH
FLUOROSCOPIC TECHNIQUES
COMPUTED TOMOGRAPHY
ISOTROPIC COMPUTED TOMOGRAPHY
DATA PROCESSING
REVIEW OF FINDINGS
PITFALLS
RADIATION DOSE
THORACIC COMPUTED TOMOGRAPHY
CONTROLLED VENTILATION TECHNIQUE
TEAM WORKING IN PAEDIATRIC RESPIRATORY CT
INTERPRETATION
ANGIOGRAPHY
MAGNETIC RESONANCE IMAGING
ENDOBRONCHIAL ULTRASONOGRAPHY
ULTRASONOGRAPHY
RADIONUCLIDE IMAGING
CONCLUSION
11. Pulmonary Function Tests in Infants and Preschool Children
INTRODUCTION
THE CHALLENGES ASSOCIATED WITH INFANT AND PRESCHOOL PULMONARY FUNCTION TESTS AND THE UNIQUE PHYSIOLOGY OF THE YOUNGEST POPULATION
INFANT PULMONARY FUNCTION TEST TECHNIQUES
INFANT PULMONARY FUNCTION TEST FINDINGS IN DISEASE
PRESCHOOL PULMONARY FUNCTION TEST TECHNIQUES
PRESCHOOL PULMONARY FUNCTION TEST FINDINGS IN DISEASE
GAPS AND FUTURE DIRECTIONS
12. Exercise, Physical Activity, and Respiratory Health and Disease in Pediatrics
THE BIOLOGICAL IMPORTANCE OF PHYSICAL ACTIVITY IN THE GROWING CHILD
LABORATORY CARDIOPULMONARY EXERCISE TESTING WITH MEASUREMENT OF GAS EXCHANGE
Nonlaboratory and Field Tests of Exercise Response and Fitness (See Table 12.2)
THE NEED FOR INTEROPERABILITY, DATA SHARING, AND STANDARDS IN PEDIATRIC CRF TESTING
ASTHMA AND EXERCISE
AIR QUALITY AND EXERCISE
PHYSICAL ACTIVITY AND EXERCISE IN CYSTIC FIBROSIS AND SICKLE CELL DISEASE
EXERCISE-INDUCED LARYNGEAL OBSTRUCTION
EXERCISE, COVID-19, AND LONG-TERM CONSEQUENCES OF SARS-CoV-2 INFECTION
TOWARD THE FUTURE: PEDIATRIC EXERCISE SCIENCE IN THE AGE OF MOLECULAR MECHANISMS, DATA SCIENCE, AND SOCIAL DETERMINANTS OF HEALTH
13. Integrating Patient-Reported Outcomes Into Research and Clinical Practice: Measures of Health-Related Quality of Life and Mental Health
DEFINITION OF A PATIENT-REPORTED OUTCOME
DEVELOPMENT AND UTILIZATION OF PATIENT-REPORTED OUTCOMES
DEVELOPMENTAL CONSIDERATIONS
USE OF PROXY RESPONDENTS
HEALTH-RELATED QUALITY-OF-LIFE MEASURES
SCREENING FOR COMMON MENTAL HEALTH CONDITIONS
PREVALENCE OF DEPRESSION AND ANXIETY IN ASTHMA
PREVALENCE OF DEPRESSION AND ANXIETY IN CYSTIC FIBROSIS
PREVALENCE OF DEPRESSION AND ANXIETY IN PRIMARY CILIARY DYSKINESIA
RECOMMENDATIONS FOR MENTAL HEALTH SCREENING MEASURES
REVIEW OF DISEASE-SPECIFIC RESPIRATORY HRQOL MEASURES FOR ASTHMA, CYSTIC FIBROSIS, AND PRIMARY CILIARY DYSKINESIA
Clinical Utility of HRQoL and Mental Health Screening
CONCLUSIONS AND FUTURE DIRECTIONS
14. Aerosol Therapy in Children
INTRODUCTION
BASIC PRINCIPLES OF AEROSOL THERAPY
AEROSOL DELIVERY DEVICES
MATCH THE DEVICE TO THE PATIENT
INHALATION INSTRUCTIONS
MONITORING ADHERENCE
MAINTENANCE AND CLEANING INSTRUCTIONS
AEROSOL THERAPY IN SPECIAL CIRCUMSTANCES
IN CONCLUSION
15. Physical Therapies in Pediatric Respiratory Disease
INTRODUCTION
AIRWAY CLEARANCE
RESPIRATORY PHYSIOTHERAPY FOR SPECIFIC CONDITIONS
EXERCISE
CONTINENCE
INHALATION THERAPIES
FREQUENCY AND DOSAGE
ADHERENCE
OTHER CONSIDERATIONS
CONDITIONS NOT GENERALLY AMENABLE TO PHYSIOTHERAPY
SUMMARY
Appendix 1
APPENDIX 2:
16. Children Dependent on Respiratory Support: Invasive and Non-Invasive Ventilation
Introduction
Epidemiology
Goals of Therapy
General Considerations for Home Care
Treatment of Chronic Respiratory Failure
Ancillary Equipment
Patient Follow-up
Outcomes
Summary
17. Microbiological Diagnosis of Respiratory Illness
RESPIRATORY PATHOGENS AND SYNDROMES
USE OF THE CLINICAL MICROBIOLOGY LABORATORY
MICROBIOME
FUTURE PROSPECTS
18. Transition From Pediatric to Adult Care
Introduction
Transition: A Practical Approach
Evidence for Transition
Barriers to Transition
Understanding Adolescent Development
Preparing for Transition
Meeting Needs of Adolescents During the Transition Period
Preparing Adult Clinicians to Receive Adolescents and Young Adults With Chronic Respiratory Conditions
Summary
19. Long-Term Consequences of Childhood Respiratory Disease
INTRODUCTION
HOW THE LUNG DEVELOPS IN HEALTH AND DISEASE
EARLY LIFE ORIGINS OF ADULT DISEASE
SUMMARY: WHAT HAVE WE LEARNED ABOUT THE IMPORTANCE OF ANTENATAL AND CHILDHOOD FACTORS?
LONG-TERM CONSEQUENCES OF SPECIFIC DISEASES
WHAT ARE THE PUBLIC HEALTH IMPLICATIONS?
WHEN SHOULD WE MEASURE SPIROMETRY?
SUMMARY AND CONCLUSIONS
20. Pediatric Lung Transplantation
INTRODUCTION
INDICATIONS AND TIMING
CONTRAINDICATIONS
SURGICAL TECHNIQUE
POSTTRANSPLANT MANAGEMENT
MANAGEMENT ISSUES UNIQUE TO PEDIATRICS
COMPLICATIONS
OUTCOMES
FUTURE DIRECTIONS
Section 3. Neonatal Pediatric Pulmonology
21. Congenital Lung Disease
INTRODUCTION
CLINICAL APPROACH
THE SIZE OF THE PROBLEM: EPIDEMIOLOGY OF CONGENITAL MALFORMATIONS OF THE LUNG
ANTENATAL DIAGNOSIS AND MANAGEMENT OF CONGENITAL LUNG DISEASE
ANTENATAL DIAGNOSIS OF CONGENITAL LUNG LESIONS
CONGENITAL ABNORMALITIES OF THE UPPER AIRWAY
SPECIFIC PROBLEMS: CONGENITAL BRONCHIAL ABNORMALITIES
SPECIFIC PROBLEMS: PARENCHYMAL LESIONS
FOREGUT (BRONCHOGENIC) CYSTS (CLINICALLY, CYSTIC CONGENITAL THORACIC MALFORMATIONS)
CONGENITAL LARGE HYPERLUCENT LOBE
PREVENTION OF (NON-MALIGNANT) COMPLICATIONS
OPTIMIZING LUNG GROWTH
RELATIONSHIP BETWEEN CONGENITAL THORACIC MALFORMATIONS AND MALIGNANT TRANSFORMATION
ADENOCARCINOMA AND CONGENITAL THORACIC MALFORMATIONS
SUMMARY AND CONCLUSIONS: WHAT APPROACH IS BEST FOR AN ASYMPTOMATIC CONGENITAL THORACIC MALFORMATION?
PULMONARY SEQUESTRATION
SPECIFIC PROBLEMS: OVEREXPANSION, APLASIA AND ECTOPIA
ECTOPIA
SPECIFIC PROBLEMS: ABNORMAL CONNECTIONS BETWEEN THE BRONCHIAL TREE AND OTHER STRUCTURES
OTHER ABNORMAL CONNECTIONS
SPECIFIC PROBLEMS: CONGENITAL DISEASE OF THE PULMONARY ARTERIAL TREE
SPECIFIC PROBLEMS: CONGENITAL DISEASE OF THE PULMONARY VENOUS TREE
SPECIFIC PROBLEMS: ABNORMALITIES OF THE CONNECTIONS BETWEEN THE PULMONARY ARTERIAL AND VENOUS TREES
SPECIFIC PROBLEMS: CONGENITAL DISEASE OF THE LYMPHATIC TREE
SPECIFIC PROBLEMS: CONGENITAL DISORDERS OF THE CHEST WALL AND DIAPHRAGM
SPECIFIC PROBLEMS: CONGENITAL CARDIAC DISORDERS
SPECIFIC PROBLEMS: MULTISYSTEM CONGENITAL DISORDERS THAT AFFECT THE LUNG
ROLE OF SPECIFIC INVESTIGATIONS
SUMMARY AND CONCLUSIONS
ACKNOWLEDGMENTS
22. Respiratory Disorders in the Newborn
INITIATION OF RESPIRATION AT BIRTH
FETAL LUNG FLUID
RESPIRATORY DEPRESSION AT BIRTH
DELIVERY ROOM RESUSCITATION
VENTILATORY CONTROL
RESPIRATORY DISTRESS SYNDROME
TRANSIENT TACHYPNEA OF THE NEWBORN
MECONIUM ASPIRATION SYNDROME
ACUTE RESPIRATORY DISTRESS SYNDROME
EARLY-ONSET PNEUMONIA
LATE-ONSET PNEUMONIA
ASPIRATION PNEUMONIA
INTERSTITIAL LUNG DISEASE
PERSISTENT PULMONARY HYPERTENSION OF THE NEWBORN
PULMONARY HYPERTENSION IN BRONCHOPULMONARY DYSPLASIA
PNEUMOTHORAX
PNEUMOMEDIASTINUM
PNEUMOPERICARDIUM
PULMONARY INTERSTITIAL EMPHYSEMA
SECONDARY PULMONARY HYPOPLASIA
PATENT DUCTUS ARTERIOSUS
PULMONARY EDEMA
PULMONARY HEMORRHAGE
UPPER AIRWAY OBSTRUCTION
GASTROESOPHAGEAL REFLUX
23. Bronchopulmonary Dysplasia
INTRODUCTION
DISEASE DEFINITIONS
EPIDEMIOLOGY
ETIOLOGY
PATHOGENESIS
PATHOPHYSIOLOGY
CLINICAL FEATURES
PREVENTION
TREATMENT
PROGNOSIS
SUMMARY
Section 4. Respiratory Infections
24. Acute Infections That Produce Upper Airway Obstruction
VIRAL LARYNGOTRACHEOBRONCHITIS
DIAGNOSIS AND DIFFERENTIAL DIAGNOSIS
EPIGLOTTITIS
BACTERIAL TRACHEITIS
DIPHTHERIA
RETROPHARYNGEAL ABSCESS
PERITONSILLAR ABSCESS (QUINSY)
INFECTIOUS MONONUCLEOSIS
25. Bronchiolitis
EPIDEMIOLOGY
ETIOLOGY
PATHOLOGY/PATHOGENESIS
CLINICAL FEATURES
DIAGNOSIS AND DIFFERENTIAL DIAGNOSIS
MANAGEMENT AND TREATMENT
PREVENTION
PROGNOSIS
26. Pneumonia in Children
EPIDEMIOLOGY AND ETIOLOGY
PATHOGENESIS
CLINICAL FEATURES
ETIOLOGIC DIAGNOSIS
DIAGNOSIS AND DIFFERENTIAL DIAGNOSIS
GENERAL MANAGEMENT
TREATMENT WITH ANTIMICROBIALS
SLOWLY RESOLVING PNEUMONIA
COMPLICATIONS
PREVENTION
PREVENTION BY VACCINES
PROGNOSIS
27. New and Emerging Viral Infections of the Lung; Focus on SARS-CoV-2
INTRODUCTION
SURVEILLANCE
SUMMARY OF HIGH-CONSEQUENCE INFECTIOUS DISEASES IN CHILDREN
EPIDEMIOLOGY
PATHOPHYSIOLOGY
TRANSMISSION
CLINICAL PRESENTATION AND COMPLICATIONS
DIAGNOSIS AND INVESTIGATIONS
MANAGEMENT OF COVID-19
MANAGEMENT OF MIS-C
PREVENTION AND VACCINATION
EMERGING VARIANTS OF COVID-19
THE IMPACT OF COVID-19 ON ACUTE RESPIRATORY ILLNESS IN CHILDREN
THE FUTURE OF RESPIRATORY INFECTIONS AND THE ENVIRONMENT
28. Tuberculosis
EPIDEMIOLOGY
MYCOBACTERIOLOGY
IMMUNOLOGY
PATHOPHYSIOLOGY
CLINICAL FEATURES
DIAGNOSIS
TESTS OF INFECTION
LABORATORY DIAGNOSIS
TREATMENT
CONTROL AND PREVENTION
29. Nontuberculous Mycobacterial Disease
MICROBIOLOGY
EPIDEMIOLOGY
ACQUISITION AND POTENTIAL FOR TRANSMISSION
CLINICAL MANIFESTATIONS OF NONTUBERCULOUS MYCOBACTERIA PULMONARY DISEASE
DIAGNOSIS OF NONTUBERCULOUS MYCOBACTERIA PULMONARY DISEASE
MANAGEMENT AND TREATMENT
OUTCOMES
30. The Mycoses
INTRODUCTION
ANTIFUNGAL DRUGS
ENDEMIC MYCOSES
Pulmonary Mycoses Primarily Seen in Hosts With Impaired Immunity
Acknowledgments
31. Pertussis and Other Bordetella Infections of the Respiratory Tract
Epidemiology
Etiology
Pathology/Pathogenesis
Clinical Features
Imaging, Pulmonary Function Testing, Laboratory Findings
Diagnosis and Differential Diagnosis
Management and Treatment
Prevention
Prognosis
32. Toxocariasis, Hydatid Disease of the Lung, Strongyloidiasis, and Pulmonary Paragonimiasis
Toxocariasis
Echinococcosis (Hydatid Disease)
Strongyloidiasis
Pulmonary Paragonimiasis (Lung Fluke Disease)
Section 5. Asthma and Other Wheezing Disorders
33. Epidemiology of Childhood Asthma
INTRODUCTION
ESTIMATES OF THE WORLDWIDE PREVALENCE OF ASTHMA
GEOGRAPHICAL VARIATIONS IN THE PREVALENCE OF CHILDHOOD ASTHMA
Conclusion
34. The Immunopathogenesis of Asthma
Altered Pulmonary Immunity in Asthma Inception
Cells, Molecules, and Cytokines Involved in Pediatric Allergic Asthma
School-Age Allergic Asthma: Pathology and Mechanisms
The Clinical Relevance of Eosinophilic Inflammation in School-Age Asthma
Essential Role of Structural Airway Cells in Pulmonary Immunity and Asthma Pathogenesis
Preschool Wheeze: A Unique Maturing Immune Environment
Specific Clinical Scenarios for Consideration
Summary
35. Asthma in the Preschool-Age Child
EPIDEMIOLOGY AND BURDEN
DIAGNOSIS
Management
NATURAL HISTORY
36. Wheezing in Older Children: Asthma
INTRODUCTION
PATHOLOGY
PATHOPHYSIOLOGY
NATURAL HISTORY AND PROGNOSIS
DIAGNOSIS OF ASTHMA
APPROACH TO MANAGEMENT
37. Severe Asthma
INTRODUCTION
OVERARCHING PRINCIPLES
THE PREVALENCE OF SEVERE ASTHMA
DEFINITIONS
WHY IS ASTHMA CONTROL POOR?
CLINICAL AND PATHOPHYSIOLOGICAL CHARACTERISTICS OF SEVERE ASTHMA
SEVERE ASTHMA: FURTHER ASSESSMENTS
MANAGEMENT OF SEVERE ASTHMA
LOOKING AHEAD TO ADULT LIFE
SUMMARY AND FUTURE DEVELOPMENTS
Section 6. Cystic Fibrosis and Other Causes of Chronic Airway Infection
38. Genetics and Pathophysiology of Cystic Fibrosis
INTRODUCTION
CYSTIC FIBROSIS TRANSMEMBRANE CONDUCTANCE REGULATOR GENE
Cystic Fibrosis Transmembrane Conductance Regulator Protein
Cellular Distribution and Function
Tissue Distribution
Genotype-Phenotype Correlations
PATHOPHYSIOLOGY
CHARACTERISTICS OF SYSTEMIC DISEASE
39. Diagnosis and Presentation of Cystic Fibrosis
DIAGNOSTIC CRITERIA FOR CYSTIC FIBROSIS
MAKING THE DIAGNOSIS OF CYSTIC FIBROSIS
ANTENATAL TESTING FOR CYSTIC FIBROSIS
NEONATAL SCREENING FOR CYSTIC FIBROSIS
THE PHENOTYPIC SPECTRUM OF CYSTIC FIBROSIS—INFLUENCING FACTORS
CYSTIC FIBROSIS PHENOTYPES
40. Pulmonary Disease in Cystic Fibrosis
EPIDEMIOLOGY
ETIOLOGY AND PATHOGENESIS
CLINICAL FEATURES: SYMPTOMS AND PHYSICAL FINDINGS
Monitoring Lung Disease
MANAGEMENT AND TREATMENT
AIRWAY HYDRATING AGENTS
PROGNOSIS
CONCLUSIONS
41. Nonpulmonary Manifestations of Cystic Fibrosis
PANCREATIC DISEASE
FIBROSING COLONOPATHY
PANCREATITIS
NUTRITION
HEPATOBILIARY DISEASE
GALLBLADDER DISEASE
CYSTIC FIBROSIS TRANSMEMBRANE CONDUCTANCE REGULATOR AND THE INTESTINE
GASTROESOPHAGEAL REFLUX DISEASE
GASTROPARESIS
SMALL INTESTINE DYSMOTILITY AND BACTERIAL OVERGROWTH
CONSTIPATION
RECTAL PROLAPSE
GASTROINTESTINAL CANCERS
INFERTILITY
CYSTIC FIBROSIS-RELATED DIABETES
CYSTIC FIBROSIS BONE DISEASE
CONCLUSION
42. Modern Molecular Therapies for Cystic Fibrosis
INTRODUCTION
CFTR MODULATORS
POTENTIATOR MONOTHERAPY
MODULATOR COMBINATION THERAPIES
OTHER DRUGS RESTORING CFTR FUNCTION: THE TRIALS PIPELINE
NON-CFTR ION CHANNELS AS DRUG TARGETS
CFTR GENE REPLACEMENT
Gene Editing
mRNA Editing
Other RNA-Based Technologies
REMAINING CHALLENGES AND OPPORTUNITIES IN THE DEVELOPMENT OF MOLECULAR THERAPIES FOR CYSTIC FIBROSIS
CONCLUSIONS
43. Primary Ciliary Dyskinesia and Other Ciliopathies
INTRODUCTION
STRUCTURE AND FUNCTION OF CILIA
CLINICAL FEATURES
DIAGNOSIS
GENETICS OF PRIMARY CILIARY DYSKINESIA
ULTRASTRUCTURAL CILIARY DEFECTS
NASAL NITRIC OXIDE MEASUREMENTS
ASSESSMENT OF CILIARY FUNCTION
MANAGEMENT AND TREATMENT
PROGNOSIS
SUMMARY
44. Bronchiectasis and Chronic Suppurative Lung Disease
INTRODUCTION
BRONCHIECTASIS AND CHRONIC SUPPURATIVE LUNG DISEASE
PROTRACTED BACTERIAL BRONCHITIS
SUMMARY
ACKNOWLEDGMENT
Section 7. Diffuse Lung Disease
45. New Concepts in Children’s Interstitial and Diffuse Lung Disease
CONCEPTS, TERMINOLOGY, AND CLASSIFICATION
GENERAL DIAGNOSTIC APPROACH
TREATMENT
RESOURCES FOR FAMILIES AND PHYSICIANS
46. Rare and Newly Recognized Childhood Lung Disorders
INTRODUCTION
PULMONARY ALVEOLAR MICROLITHIASIS
ATAXIA TELANGIECTASIA
GAUCHER DISEASE
NIEMANN-PICK DISEASE
NEUROFIBROMATOSIS
DYSKERATOSIS CONGENITA
HERMANSKY-PUDLAK SYNDROME
LUNG DISEASE IN TBX4 GENETIC VARIANTS
SUMMARY
47. Childhood Interstitial Lung Disease More Prevalent in Infancy
OVERVIEW
Developmental Lung Disorders
ALVEOLAR GROWTH ABNORMALITIES PRESENTING AS CHILDHOOD INTERSTITIAL LUNG DISEASE
PULMONARY INTERSTITIAL GLYCOGENOSIS
NEUROENDOCRINE CELL HYPERPLASIA OF INFANCY
SUMMARY
48. Lung Diseases Associated With Disruption of Pulmonary Surfactant Homeostasis
OVERVIEW OF SURFACTANT COMPOSITION AND METABOLISM
ETIOLOGY
PATHOLOGY/PATHOGENESIS
CLINICAL FEATURES OF SURFACTANT PRODUCTION DISORDERS
DIAGNOSIS AND DIFFERENTIAL DIAGNOSIS
MANAGEMENT, TREATMENT, AND PROGNOSIS
SUMMARY
49. Pulmonary Involvement in the Systemic Inflammatory Diseases of Childhood
OVERVIEW OF CHAPTER
JUVENILE IDIOPATHIC ARTHRITIS
SYSTEMIC LUPUS ERYTHEMATOSUS
JUVENILE DERMATOMYOSITIS
SYSTEMIC SCLEROSIS (SCLERODERMA)
MIXED CONNECTIVE TISSUE DISEASE
SARCOIDOSIS
CHILDHOOD VASCULITIDES
GRANULOMATOSIS WITH POLYANGIITIS
OTHER SYSTEMIC INFLAMMATORY DISEASES WITH SIGNIFICANT PULMONARY INVOLVEMENT
CLINICAL APPROACH TO DIAGNOSIS AND MANAGEMENT OF PULMONARY INVOLVEMENT IN THE SYSTEMIC INFLAMMATORY DISEASES OF CHILDHOOD
50. Lung Injury Caused by Pharmacologic Agents
CYTOTOXIC DRUGS USED IN CANCER THERAPY
IMMUNOMODULATORY AGENTS USED IN CANCER THERAPY AND AFTER TRANSPLANTATION
NONCYTOTOXIC DRUGS
OTHER AGENTS
CONCLUSIONS
51. Diffuse Alveolar Hemorrhage in Children
ETIOLOGY OF DIFFUSE ALVEOLAR HEMORRHAGE
TREATMENT OF ANTINEUTROPHIL CYTOPLASMIC ANTIBODY-ASSOCIATED VASCULITIS
ANTI-GLOMERULAR BASEMENT MEMBRANE DISEASE
ISOLATED PULMONARY CAPILLARITIS
COATOMER PROTEIN COMPLEX SUBUNIT ALPHA SYNDROME
SAVI
NONIMMUNE-MEDIATED ALVEOLAR HEMORRHAGE
APPROACH TO THE CHILD WITH PULMONARY HEMORRHAGE
SUMMARY
52. Hypersensitivity Pneumonitis and Eosinophilic Lung Diseases
HYPERSENSITIVITY PNEUMONITIS
LANGERHANS HISTIOCYTOSIS X (FORMERLY EOSINOPHILIC GRANULOMA)
Section 8. Respiratory Complications in the Immunocompromised Child
53. Primary Immunodeficiency and Other Diseases With Immune Dysregulation
DEFECTS OF THE INNATE IMMUNE SYSTEM
DEFECTS OF THE ADAPTIVE IMMUNE SYSTEM
54. Pulmonary Disease in the Immunosuppressed Pediatric Patient
INTRODUCTION
PULMONARY INFECTIONS IN THE IMMUNOCOMPROMISED PEDIATRIC HOST
CLINICAL PRESENTATION OF PULMONARY INFECTION IN THE IMMUNOCOMPROMISED CHILD
INFECTIOUS RISKS SHARED BY MALIGNANCY, SOLID-ORGAN TRANSPLANTATION, AND STEM CELL TRANSPLANTATION
COMMON PULMONARY INFECTIOUS AGENTS IN THE IMMUNOCOMPROMISED PEDIATRIC HOST
RARER FUNGAL PNEUMONIAS
NONINFECTIOUS PULMONARY COMPLICATIONS FOLLOWING SOLID-ORGAN TRANSPLANTATION (EXCLUDING THOSE UNIQUE TO LUNG TRANSPLANTATION)
PULMONARY TOXICITY OF IMMUNOSUPPRESSIVE MEDICATIONS
PULMONARY COMPLICATIONS OF CHILDHOOD TUMORS AND THEIR TREATMENT
PULMONARY COMPLICATIONS OF HEMATOPOIETIC STEM CELL TRANSPLANTATION
APPROACH TO PULMONARY DISEASE IN THE IMMUNOCOMPROMISED PEDIATRIC HOST
NONINVASIVE DIAGNOSTIC STUDIES
INVASIVE DIAGNOSTIC STUDIES
PREVENTION STRATEGIES
55. Respiratory Disorders in Children and Adolescents Living With HIV
SPECTRUM OF RESPIRATORY DISEASE
ACUTE LUNG DISEASE
CHRONIC LUNG DISEASE
SUMMARY
Section 9. Pulmonary Vascular Disease
56. Pulmonary Embolism and Thromboembolic Diseases
EPIDEMIOLOGY
ETIOLOGY
CENTRAL VENOUS CATHETERS AND AGE-SPECIFIC ETIOLOGIES
INHERITED AND ACQUIRED THROMBOPHILIA
PATHOLOGY AND PATHOGENESIS OF PTE
CLINICAL FEATURES
IMAGING AND LABORATORY TESTS
MANAGEMENT
PREVENTION
57. Childhood Pulmonary Arterial Hypertension
INTRODUCTION
DEFINITION AND CLASSIFICATION
EPIDEMIOLOGY AND ETIOLOGY
NEONATAL PULMONARY HYPERTENSION
ASSOCIATED PULMONARY ARTERIAL HYPERTENSION WITH CONGENITAL HEART DISEASE
RISK STRATIFICATION
MANAGEMENT
ADDITIONAL PHARMACOTHERAPY: CARDIAC GLYCOSIDES, DIURETICS, ANTIARRHYTHMIC THERAPY, INOTROPIC AGENTS, AND NITRATES
ATRIAL SEPTOSTOMY
LUNG TRANSPLANTATION
CONCLUSIONS
58. Pulmonary Arteriovenous Malformations
INTRODUCTION
GENETICS AND PATHOPHYSIOLOGY
CLINICAL MANIFESTATIONS AND DIAGNOSTIC CRITERIA
SCREENING AND TREATMENT
SUMMARY
59. Pulmonary Lymphatic Disease
Introduction
Other Conditions
Section 10. The Aerodigestive Model
60. The Aerodigestive Model: Improving Health Care for Complex Patients
HISTORICAL PERSPECTIVES
WHICH PATIENTS BENEFIT FROM AERODIGESTIVE CARE?
ESTABLISHING A TEAM
OUTCOMES AND RESEARCH
SUMMARY
61. Aspiration, Feeding, and Swallowing Disorders
INTRODUCTION
EMBRYOLOGY/DEVELOPMENT OF THE UPPER AERODIGESTIVE TRACT
ANATOMY, PHYSIOLOGY, AND DEVELOPMENT
FEEDING SKILL DEVELOPMENT
PHASES OF SWALLOWING
EPIDEMIOLOGY OF PEDIATRIC FEEDING AND SWALLOWING DISORDERS
ETIOLOGY/PATHOGENESIS
CONDITIONS ASSOCIATED WITH FEEDING, SWALLOWING, AND AIRWAY PROTECTION PROBLEMS
SPECIFIC AIRWAY CONDITIONS AND THE EFFECT ON FEEDING AND SWALLOWING
EVALUATION OF FEEDING AND SWALLOWING
DIAGNOSIS AND EVALUATION OF ASPIRATION
ASPIRATION OF GASTROESOPHAGEAL REFLUX
EVALUATION OF SALIVARY ASPIRATION
LUNG INJURY DUE TO ASPIRATION
BIOMARKERS OF ASPIRATION
MULTIDISCIPLINARY APPROACH TO CHRONIC ASPIRATION
TREATMENT STRATEGIES FOR FEEDING AND SWALLOWING DISORDERS
TREATMENT CONSIDERATIONS FOR REFLUX ASPIRATION
TREATMENT OF SALIVARY ASPIRATION
AIRWAY SURGICAL INTERVENTIONS FOR ASPIRATION
CONCLUSION
62. Gastroesophageal Reflux Disease and Eosinophilic Esophagitis in Children With Complex Airway Disease
GASTROESOPHAGEAL REFLUX DISEASE
DISORDERS OF MOTILITY
EOSINOPHILIC ESOPHAGITIS
63. Laryngeal and Tracheal Airway Disorders
INTRODUCTION
OPERATIVE ASSESSMENT
MITIGATING FACTORS
OPTIMIZATION
MANAGEMENT OF AIRWAY PATHOLOGY
CONCLUSION
Section 11. Disorders of Paediatric Sleep and Control of Breathing
64. Basic Physiological Principles of Sleep and Respiratory Control in Infants and Children
NEURAL CIRCUITRY OF SLEEP AND WAKING
MATURATION OF SLEEP CYCLES
SLEEP AND BREATHING DURING DEVELOPMENT
CENTRAL CHEMORECEPTORS AND THEIR DEVELOPMENT
MULTIPLICITY OF CENTRAL CHEMOSENSITIVE CENTERS
PERIPHERAL CHEMOREFLEXES
65. Testing for Sleep-Disordered Breathing
INTRODUCTION
PEDIATRIC SLEEP QUESTIONNAIRES
PHYSICAL EXAMINATION
DIAGNOSTIC SLEEP STUDY
PHYSIOLOGIC ENDOTYPING
ENDOSCOPY
IMAGING APPROACHES
BIOMARKERS
SLEEP TECHNOLOGY
CONCLUSION
66. Obstructive Sleep Apnea in Children
INTRODUCTION
EPIDEMIOLOGY
OBSTRUCTIVE SLEEP APNEA–RELATED CARDIAC AND VASCULAR DYSFUNCTION
67. Complex Sleep and Hypoventilation Disorders in Children
CENTRAL HYPOVENTILATION
CONGENITAL CENTRAL HYPOVENTILATION SYNDROME
ROHHAD SYNDROME
OTHER HYPOVENTILATION DISORDERS
CENTRAL SLEEP APNEA
68. Sudden Infant Death Syndrome, Brief Resolved Unexplained Events and Apparent Life-Threatening Events, and Apnea of Prematurity
BRIEF RESOLVED UNEXPLAINED EVENTS AND APPARENT LIFE-THREATENING EVENTS
APNEA IN PREMATURE INFANTS
SUDDEN INFANT DEATH SYNDROME
ACKNOWLEDGMENTS
Section 12. The Critically Ill Child
69. Principles of Mechanical Ventilation
HISTORY
INDICATIONS FOR MECHANICAL VENTILATION
APPLIED RESPIRATORY PHYSIOLOGY
COMPOSITION OF A MECHANICAL VENTILATOR
MODES OF VENTILATION
HIGH-FREQUENCY VENTILATION
NONINVASIVE VENTILATION
SPECIAL CONSIDERATIONS OF MECHANICAL VENTILATION
MONITORING
COMPLICATIONS OF MECHANICAL VENTILATION
WEANING AND EXTUBATION
MECHANICAL VENTILATION IN SPECIFIC CONDITIONS
CONCLUSION
ACKNOWLEDGMENT
70. Pediatric Acute Respiratory Distress Syndrome
DEFINITION AND DIAGNOSIS
EPIDEMIOLOGY
HISTOLOGY
PATHOPHYSIOLOGY
ETIOLOGY
OUTCOMES: MORBIDITY AND MORTALITY, PREDICTORS OF OUTCOME, AND RISK STRATIFICATION
MANAGEMENT
SUMMARY/CONCLUSION
71. Pulmonary Edema
ANATOMIC CONSIDERATIONS
FACTORS RESPONSIBLE FOR FLUID MOVEMENT
MECHANISMS THAT CAUSE PULMONARY EDEMA
CLEARANCE OF PULMONARY EDEMA FLUID
PATHOPHYSIOLOGIC CONSEQUENCES OF EDEMA
CLINICAL PRESENTATION
CLINICAL DISORDERS CAUSING PULMONARY EDEMA
THERAPY
72. Lung Injury From Hydrocarbon Aspiration and Smoke Inhalation
HYDROCARBON ASPIRATION
RESPIRATORY COMPLICATIONS OF SMOKE INHALATION
73. Drowning
DEFINITION
PULMONARY INJURY
NON-PULMONARY SEQUELAE
MANAGEMENT
OUTCOME OF PULMONARY INJURY
OUTCOME PREDICTION OF NEUROLOGICAL INJURY
PREVENTION
74. Trauma of the Thorax
INTRODUCTION
EPIDEMIOLOGY
MECHANISMS OF INJURY
PATHOPHYSIOLOGY
PNEUMOTHORAX
CARDIOVASCULAR INJURY
INDICATIONS FOR SURGERY
OUTCOMES
PREVENTION
SUMMARY
75. Respiratory Complications of Intensive Care
HISTORY OF PEDIATRIC INTENSIVE CARE
CONSIDERATIONS PRIOR TO INTUBATION AND MECHANICAL VENTILATION
VENTILATION STRATEGIES
COMPLICATIONS OF MECHANICAL VENTILATION
CONCLUSION
Section 13. Miscellaneous Pulmonary Disorders
76. Pulmonary Disease Associated With Congenital Heart Disease
INTRODUCTION
AIRWAY COMPRESSION DUE TO VASCULAR RINGS AND SLINGS
Pulmonary Hypertension and Postoperative Pulmonary Hypertensive Crisis
PULMONARY VEIN STENOSIS
REPERFUSION PULMONARY EDEMA IN CHILDREN UNDERGOING PROCEDURES FOR TETRALOGY OF FALLOT, PULMONARY ATRESIA, AND MULTIPLE AORTOPULMONARY COLLATERAL ARTERIES
Pulmonary Disease Associated With the Fontan Repair of Single Ventricle Physiology
PULMONARY COMPLICATIONS RELATED TO CARDIAC SURGERY AND CARDIOPULMONARY BYPASS
CONCLUSION
77. The Lung in Sickle Cell Disease
EPIDEMIOLOGY
ETIOLOGY
PATHOGENESIS
CLINICAL FEATURES
DIAGNOSIS
MANAGEMENT
PREVENTION
PROGNOSIS
SUMMARY
78. Respiratory Complications of Down Syndrome and Other Genetic Syndromes
INTRODUCTION
UPPER AND LOWER AIRWAY DISEASE
LUNG PARENCHYMAL DISEASE
SLEEP-RELATED BREATHING DISORDERS
CARDIORESPIRATORY INTERACTIONS AND PULMONARY VASCULATURE
AERODIGESTIVE DISORDERS IN DOWN SYNDROME
OBESITY AND ITS IMPACT ON THE LUNG IN DOWN SYNDROME
THE APPROACH TO ORGANIZING CARE OF AND TREATING RESPIRATORY COMPLICATIONS OF DOWN SYNDROME
COVID-19 IN CHILDREN WITH DOWN SYNDROME
CONCLUSIONS
OTHER GENETIC ANOMALIES
79. Tumors of the Chest
EPIDEMIOLOGY
ETIOLOGY
BENIGN PRIMARY PULMONARY LESIONS
MALIGNANT PRIMARY PULMONARY LESIONS
CHEST WALL TUMORS
MEDIASTINAL TUMORS
PRIMARY CARDIAC AND PERICARDIAL TUMORS
TUMORS OF THE DIAPHRAGM
GENERAL APPROACH TO EVALUATION OF CHILDREN WITH SUSPECTED TUMORS OF THE CHEST
MANAGEMENT AND TREATMENT OF THORACIC TUMORS
ACKNOWLEDGMENTS
80. Respiratory Aspects of Neuromuscular Disorders
INTRODUCTION
PHYSIOLOGY
ASSESSMENT AND MONITORING
RESPIRATORY INTERVENTIONS
COMPLICATIONS
DISEASE MODIFYING TREATMENTS
END OF LIFE MANAGEMENT
SUMMARY
81. Air and Fluid in the Pleural Space, and Atelectasis
INTRODUCTION
EMBRYOLOGY AND ANATOMY
PHYSIOLOGY
AIR IN THE PLEURAL SPACE: PNEUMOTHORAX
FLUID IN THE PLEURAL SPACE
ATELECTASIS
CONCLUSION
Section 14. Emerging Threats to Children’s Lung Health
82. Environmental Contributions to Respiratory Disease in Children
WHAT HAS CHANGED SINCE THE 9TH EDITION?
VULNERABILITY OF CHILDREN TO ADVERSE ENVIRONMENTAL EXPOSURES
What Can Be Done to Reduce Environmental Impacts on Respiratory Disease?
Environmental Contributions to Acute Respiratory Illness and Pneumonia
Environmental Contributions to Asthma
Environmental Contributions to Cystic Fibrosis
Impact of Climate and Environmental Change on Increased Risk of Respiratory Disease
Summary
83. Combustible and Electronic Cigarettes: Tobacco and Marijuana Use in Children and Adolescents
INTRODUCTION
TOBACCO AND NICOTINE USE IN YOUTHS
MARIJUANA USE IN YOUTHS
ELECTRONIC NICOTINE DELIVERY SYSTEMS
TREATMENT OF TOBACCO AND NICOTINE DEPENDENCE
PROMOTION AND PREVENTION OF TOBACCO USE
SUMMARY
INDEX

Andrew Bush

Andrew Bush is Professor of Pediatric Respirology, Imperial College, Consultant Pediatric Chest Physician, Royal Brompton Hospital, London, United Kingdom

Affiliations and expertise

Professor of Pediatric Respirology, Imperial College, Consultant Pediatric Chest Physician, Royal Brompton Hospital, London, United Kingdom

Robin R Deterding

Robin Deterding is Professor of Pediatrics, Director, Breathing Institute, Children’s Hospital Colorado, University of Colorado, Aurora, Colorado

Affiliations and expertise

Professor of Pediatrics, Director, Breathing Institute, Children’s Hospital Colorado, University of Colorado, Aurora, Colorado

Albert Li

Albert Li, MD, Professor of Paediatrics and Person-in-charge of the Respiratory Service in the Department of Paediatrics at the Chinese University of Hong Kong

Affiliations and expertise

Professor of Paediatrics and Person-in-charge of the Respiratory Service in the Department of Paediatrics at the Chinese University of Hong Kong.

Felix Ratjen

Felix Ratjen is Head, Division of Respiratory Medicine, Sellers Chair of Cystic Fibrosis, Professor, University of Toronto, Hospital for Sick Children, Toronto, Ontario, Canada

Affiliations and expertise

Head, Division of Respiratory Medicine, Sellers Chair of Cystic Fibrosis, Professor, University of Toronto, Hospital for Sick Children, Toronto, Ontario, Canada

Peter Sly

Peter Sly, MD is Director, Children's Health and Environment Program and Director, WHO Collaborating Centre for Children's Health and Environment. Centre for Child Health Research, South Brisbane, Australia

Affiliations and expertise

Director, University of Queensland, Department of Children's Lung Environment and Asthma Research Group, Brisbane Austrailia

Heather Zar

Heather Zar, MD, Professor & Head of the Department of Paediatrics and Child Health and Director of the School of Child and Adolescent Health at the University of Cape Town (UCT), South Africa.

Affiliations and expertise

Professor & Head of the Department of Paediatrics and Child Health and Director of the School of Child and Adolescent Health at the University of Cape Town (UCT), South Africa.

Robert W. Wilmott

Robert Wilmott is IMMUNO Professor and Chair, Department of Pediatrics, St. Louis University; Chief Pediatrician, Cardinal Glennon Children’s Hospital, St. Louis, Missouri

Affiliations and expertise

IMMUNO Professor and Chair, Department of Pediatrics, St. Louis University; Chief Pediatrician, Cardinal Glennon Children’s Hospital, St. Louis, Missouri

Life sciences

Physical sciences & Engineering

Social sciences & Humanities

Health

Kendig and Wilmott’s Disorders of the Respiratory Tract in Children

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Andrew Bush

Robin R Deterding

Albert Li

Felix Ratjen

Peter Sly

Heather Zar

Robert W. Wilmott