CB04.2
category
Disorders of surfactant metabolism
Definition
Primary interstitial lung disease specific to childhood due to pulmonary surfactant protein anomalies is a group of interstitial lung diseases (ILD) induced by genetic mutations disrupting surfactant function and gas exchange in the lung. The disorders caused by these mutations affect full-term infants and older children and exhibit considerable overlap in their clinical and histologic presentation
Also indexed as
Chronic lung disease with surfactant disorderDisorders of surfactant metabolismLate onset surfactant deficiencyNeonatal acute respiratory distress with surfactant disorderSurfactant ABCA3 gene mutationSurfactant protein B (SP-B) deficiencySurfactant protein C gene mutation
Nearby in Primary interstitial lung diseases specific to infancy or childhood
CB04.0Diffuse pulmonary developmental disordersCB04.1Pulmonary lymphatic dysplasia syndromesCB04.3Alveolar or peri-alveolar conditionsCB04.4Pulmonary capillaritisCB04.5Brain-lung-thyroid syndromeCB04.6Chronic pneumonitis of infancyCB04.7Neuroendocrine cell hyperplasia of infancy4B4YOther specified diseases of the immune system