SOVREMENNAYA PEDIATRIYA.2019.3(99):74-80; doi 10.15574/SP.2019.99.74

Tsimbalista O. L., Mytnyk N. Ya.
State higher educational institution ВНЗ «Ivano–Frankivsk National Medical University», Ukraine
Ivano–Frankivsk Oblast Children's Clinical Hospital, Ukraine

The article highlights contemporary views on the pathogenetic mechanisms of development, pathomorphology of alveolar proteinosis and alveolar microlithiasis: two of the most severe hereditary pulmonary diseases. The relevance of the problem is due to the complexity of diagnosis, the lack of effective methods of pathogenetic therapy and high mortality rate. The age of alveolar proteinosis and alveolar microlithiasis manifestation is determined. Both diseases are characterized by the absence of pathognomonic clinical symptoms: the symptoms of pulmonary failure and further heart heart failure dominate in the clinical picture. With alveolar proteinosis, a short-term therapeutic effect is determined by bronchoalveolar lavage, granulocyte-colony stimulating factor, posyndromic therapy; lung transplantation is relatively effective. In alveolar microlithiasis, only symptomatic therapy and, in some cases, lung transplantation is performed.

Key words: alveolar proteinosis, alveolar microlithiasis, diagnostics, therapy.

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Article received: Dec 11, 2018. Accepted for publication: Mar 24, 2019.