Сучасне уявлення про роль мутацій протеїнів сурфактанту в формуванні інтерстиціальних захворювань легень у новонароджених і немовлят

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.