DOI: https://doi.org/10.15574/SP.2019.99.74

Актуальные проблемы диагностики и лечения отдельных наследственных заболеваний легких у детей (лекция)

O. L. Tsimbalista, N. Ya. Mytnyk

Аннотация


В статье представлены современные взгляды на патогенетические механизмы развития, патоморфологию альвеолярного протеиноза и альвеолярного микролитиаза — одних из наиболее тяжелых наследственных заболеваний легких. Актуальность проблемы обусловлена сложностью диагностики, отсутствием эффективных методов патогенетической терапии и высокой летальностью. Уточнен возраст манифестации альвеолярного протеиноза и альвеолярного микролитиаза. Обоим заболеваниям свойственно отсутствие патогномонических клинических симптомов: в клинической картине доминируют симптомы легочной, далее — сердечной недостаточности. При альвеолярном протеинозе кратковременный терапевтический эффект обусловлен бронхоальвеолярным лаважом, гранулоцитарно-колониестимулирующим фактором, синдромальной терапией; сравнительно эффективной является трансплантация легких. При альвеолярном микролитиазе проводится только симптоматическая терапия и, в отдельных случаях, трансплантация легких.

Ключевые слова


альвеолярный протеиноз; альвеолярный микролитиаз; диагностика; терапия

Полный текст:

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Литература


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