Болезнь Рандю-Ослера-Вебера (наследственная геморрагическая телеангиэктазия) с поражением органов дыхания

Наследственная геморрагическая телеангиэктазия (НГТ) — редкое заболевание, наследуемое по аутосомно-доминантному типу, характеризуемое различными сосудистыми дефектами, включая носовые кровотечения, расширение кровеносных сосудов (телеангиэктазии) и артериовенозные мальформации (АВМ) в легких и других внутренних органах. Легочные АВМ наблюдаются у 15—50 % больных НГТ. Заболевание манифестирует в детском возрасте и нарастает при тяжелых клинических проявлениях в течение всей жизни больного. Эпонимичное название болезни Рандю—Ослера—Вебера происходит от фамилий врачей, впервые ее описавших в середине XIX века. Несмотря на определенные успехи в изучении этиологии и патогенеза этого заболевания, тактика лечения до сих пор остается недостаточно определенной.

Целью обзора является систематизация сведений об этиологии, диагностике и лечении НГТ. В статье представлены современное состояние проблемы, основные диагностические исследования и принципы медикаментозного и хирургического лечения.

Заключение. При полиорганном поражении при НГТ требуется междисциплинарный подход к ведению таких пациентов как в детском, так и взрослом возрасте. С открытием генетических основ этиологии заболевания перспективным направлением в лечении является применение патогенетической терапии с использованием гуманизированных моноклональных антител. Однако данный вид терапии требует дальнейших исследований.

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