Актуальные проблемы диагностики и лечения отдельных наследственных заболеваний легких у детей (лекция)
DOI:
https://doi.org/10.15574/SP.2019.99.74Ключевые слова:
альвеолярный протеиноз, альвеолярный микролитиаз, диагностика, терапияАннотация
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.Библиографические ссылки
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