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Диагностика и лечение легочной патологии при дефиците альфа-1-антитрипсина: доклад Европейского респираторного общества

https://doi.org/10.18093/0869-0189-2018-28-3-273-295

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Аннотация

Дефицит альфа-1-антитрипсина (ААТ) – наиболее распространенное наследственное заболевание взрослых, связанное с повышенным риском развития эмфиземы легких и патологии печени. Поражение легких при дефиците ААТ тесно связано с курением, но даже у никогда не куривших больных может развиться прогрессирующее поражение легких, вызванное этим генетическим дефектом. У многих больных с дефицитом ААТ болезнь остается недиагностированной и, соответственно, такие больные не получают должного лечения.

В 2003 г. опубликован совместный доклад Американского Торакального Общества и Европейского Респираторного Общества по дефициту ААТ. В последующие годы появились новые, более точные и дешевые генетические методы диагностики, разработаны новые конечные показатели для использования в клинических исследованиях, выполнены новые наблюдательные и рандомизированные клинические исследования, представившие новые доказательства безопасности и эффективности заместительной терапии, которая сегодня остается единственным методом специфической терапии поражения легких при дефиците ААТ. Поскольку дефицит ААТ является редким заболеванием, важно создавать национальные и международные регистры для получения проспективной информации о естественном течении этого заболевания. Больные с дефицитом ААТ должны наблюдаться и лечиться в национальных экспертных центрах; следует совместно обсуждать любые разногласия в ведении таких больных в Европе.

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Для цитирования:


Диагностика и лечение легочной патологии при дефиците альфа-1-антитрипсина: доклад Европейского респираторного общества.  Пульмонология. 2018;28(3):273-295. https://doi.org/10.18093/0869-0189-2018-28-3-273-295

For citation:


Diagnosis and treatment of pulmonarydisease in α1-antitrypsin deficiency: a statement of European Respiratory Society. Russian Pulmonology. 2018;28(3):273-295. (In Russ.) https://doi.org/10.18093/0869-0189-2018-28-3-273-295

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