CB04.2 Disorders of surfactant metabolism
International Classification of Diseases for Mortality and Morbidity Statistics, 11th Revision, v2024-01
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
synonyms
- Disorders of surfactant metabolism
- Chronic lung disease with surfactant disorder
- Late onset surfactant deficiency
- Neonatal acute respiratory distress with surfactant disorder
- Surfactant protein B (SP-B) deficiency
- Surfactant ABCA3 gene mutation
- Surfactant protein C gene mutation
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