Diseases of Pulmonary Surfactant Homeostasis

Pulmonary surfactant is required for adaptation to air breathing after birth, reducing surface tension at the air-liquid interface in the alveolus to maintain lung volumes during the respiratory cycle. Disorders of pulmonary surfactant homeostasis are associated with acute and chronic respiratory disease in infants and adults. Mutations in the surfactant protein genes encoding SP-B and SP-C cause severe respiratory failure in infancy and chronic interstitial lung disease in older individuals.