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ГЕННАЯ ТЕРАПИЯЕще одной надеждой больных МВ и их врачей является генная терапия. При помощи генно-инженерных технологий клонирована комплементарная ДНК, кодирующая белок МВТР, и ведутся активные поиски метода доставки этого гена в эпителиальные клетки бронхов. Доказано, что 10% уровень нормального МВТР достаточен, чтобы защитить дыхательные пути от развития патологического процесса. Проведены клинические испытания с использованием вирусных векторов и невирусных носителей (катионные липосомы, полимеросомы) [144]. Клинически значимого результата пока получено не было, однако исследования продолжаются, так как в случае успеха наступит новый этап в лечении МВ - этап этиологического подхода к лечению этого заболевания. Таким образом, МВ является генетически детерминированным заболеванием, основной патогенетический механизм которого связан с увеличением вязкости секрета экзокринных желез. При этом патология легких определяет клиническую картину и тяжесть течения и является одной из наиболее частых причин смерти при МВ у взрослых. Патоморфология легких определяется наличием множественных бронхо- и бронхиолоэктазов, выраженным воспалением, приводящим к структурным изменениям легочной ткани с развитием буллезной эмфиземы. Лечение больных МВ основано на комплексной терапии с применением антибактериальных, муко- и бронхолитических препаратов в сочетании с ферменто- и кинезитерапией. Перспективы лечения связаны с дальнейшей разработкой антибактериальной терапии, методов трансплантации и генетической коррекции основного дефекта МВ.
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