КЛИНИКО-ГЕНЕТИЧЕСКИЕ ХАРАКТЕРИСТИКИ СИНДРОМА МНОЖЕСТВЕННЫХ ЭНДОКРИННЫХ НЕОПЛАЗИЙ ТИПА 1 И ПРИНЦИПЫ ЕГО ЛЕЧЕНИЯ


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Аннотация

Представлены современные представления об эпидемиологии, патогенезе и клинической картине синдрома множественных эндокринных неоплазий типа 1 (МЭН-1). МЭН-1 - это редкий врожденный аутосомно-доминантный синдром, вызванный мутацией гена MEN1, локализованного на хромосоме 11q13. Для МЭН-1 характерно сочетание опухолей околощитовидных желез, гипофиза и поджелудочной железы. Рассматривается алгоритм диагностики синдрома МЭН-1, обсуждаются возможность его лечения, в т. ч. с использованием аналогов соматостатина. Подчеркивается, что на основе точного диагноза и современных методов хирургического и медикаментозного лечения пациентов с МЭН-1 в последние годы значительно улучшился прогноз и увеличилась продолжительность жизни пациентов с разнообразными проявлениями этого синдрома.

Об авторах

Л Г Ростомян

ФГУ Эндокринологический научный центр Росмедтехнологий, Москва

ФГУ Эндокринологический научный центр Росмедтехнологий, Москва

Л Я Рожинская

ФГУ Эндокринологический научный центр Росмедтехнологий, Москва

ФГУ Эндокринологический научный центр Росмедтехнологий, Москва

А Н Тюльпаков

ФГУ Эндокринологический научный центр Росмедтехнологий, Москва

ФГУ Эндокринологический научный центр Росмедтехнологий, Москва

L Rostomyan

L Rozhinskaya

A Tyulpakov

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