LIBRO + ANEXOS NEUMOLOGÍA PEDIÁTRICA

Libro Blanco de las ACES Pediátricas 2024 126 ❚ f. Recognize that interstitial lung disease may be a manifestation of graft versus host disease following bone marrow transplantation g. Know the most common causes of hypersensitivity pneumonitis h. Recognize which inborn errors of surfactant metabolism (mutations resulting in surfactant dysfunction) cause interstitial lung disease in neonates, infants, and children i. Know that neuroendocrine cell hyperplasia of infancy (NEHI) and pulmonary interstitial glycogenosis are forms of chronic lung disease that are unique to infants j. Know that lung growth disorders characterized by alveolar simplification are often misdiagnosed as interstitial lung disease and are the most common finding in lung biopsies done in children under 2 years of age 3. Pathophysiology a. Pathology 1. Recognize the histologic picture of bronchiolitis obliterans 2. Know the histology of desquamative interstitial pneumonitis 3. Know the histology of lymphoid interstitial pneumonia 4. Recognize the pulmonary manifestations of collagen vascular disease b. Path mechanisms and consequences 1. Know that surfactant proteins B and C and ABCA3 gene mutations can have various pathologies, including pulmonary alveolar proteinosis, desquamative interstitial pneumonia, chronic pneumonitis of infancy, nonspecific interstitial pneumonia, and usual inter 4. Diagnosis and clinical manifestations a. History 1. Know the time course of onset of radiation-induced pneumonitis 2. Recognize clinical symptoms suggestive of interstitial lung disease in infants and older children 3. Recognize the value of an environmental history in the evaluation of pediatric interstitial lung disease 4. Know the clinical presentation of acute eosinophilic pneumonia b. Physical examination 1. Recognize physical findings characteristic of interstitial lung disease (eg, tachypnea and crackles) in children c. Imaging 1. Recognize imaging findings typical of bronchiolitis obliterans 2. Recognize radiographic findings typical of unilateral or localized hyperlucent lung (Swyer-James or Macleod syndrome) 3. Recognize radiographic manifestations of interstitial lung disease 4. Recognize radiographic manifestations of pulmonary disease secondary to collagen vascular disease 5. Know the usefulness of high-resolution computed tomography (CT scan) in the evaluation of interstitial lung disease 6. Know the radiologic appearance and clinical implications of honey-comb lung d. Pulmonary function tests 1. Identify pulmonary function abnormalities associated with interstitial lung disease