Surfactant Protein-B Deficiency

Pulmonary surfactant is the mixture of lipids and proteins needed to reduce alveolar surface tension at the air-liquid interface and to prevent end-expiratory atelectasis. An inability to produce sufficient quantities of surfactant due to immaturity is the primary cause of the respiratory distress syndrome (RDS) observed in infants born prematurely, and diminished surfactant function contributes to the pathophysiologic condition of ARDS. 1 Farrell PM Avery MA Hyaline membrane disease.. Am Rev Respir Dis. 1975; 111: 657-688 PubMed Google Scholar , 2 Lewis JF Jobe AH Surfactant and the adult respiratory distress syndrome.. Am Rev Respir Dis. 1993; 147: 218-233 Crossref PubMed Scopus (478) Google Scholar While the phospholipid components of pulmonary surfactant are important in its surface tension-lowering properties, specific surfactant proteins have been identified that have important roles in surfactant function and metabolism. The inability to produce one of these proteins, surfactant protein-B (SP-B), results in fatal respiratory disease, indicating its fundamental role in normal lung function. 3 Nogee LM DeMello DM Dehner LP et al. Deficiency of pulmonary surfactant protein B in congenital alveolar proteinosis.. N Engl J Med. 1993; 328: 406-410 Crossref PubMed Scopus (525)