DOI: https://doi.org/10.22141/2224-0551.13.5.2018.141570

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

O.L. Logvinova, M.A. Gonchar

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

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