A modern understanding of the surfactant protein mutations role in the formation of interstitial lung diseases in newborns and infants

Abstract

The article presents a modern understanding of the components of the surfactant, the functions of non-serum proteins SP-A, SP-B, SP-C and SP-D. The history of the discovery of protein deficiencies SP-B, SP-C and ABCA3 is described. The authors had analyzed modern epidemiology, clinical and histological features of various deficiencies of surfactant proteins. Va­rious variants of SFTPC, SFTPB and ABCA3 mutations and their influence on the course, clinical manifestations and outcome of interstitial lung disease in children are demonstrated.

Keywords

• newborns
• children
• interstitial lung diseases
• surfactant
• respiratory distress syndrome
• pulmonary alveolar proteinosis