Врожденная дисфункция коры надпочечников у детей вследствие дефицита фермента 21-гидроксилазы: клинические проявления, осложнения и подходы к их коррекции
DOI:
https://doi.org/10.15574/SP.2015.68.123Аннотация
При хронической гормональной декомпенсации пациентов с врожденной дисфункцией коры надпочечников (ВДКН) возможно развитие осложнений в виде прогрессирования вирилизации с ранним закрытием зон роста, развитием бесплодия у пациентов обоих полов, появленим опухолей яичек у мальчиков, которые имеют обратимый характер при нормализации гормонального статуса. Среди девочек большинство (35%) имеют III степень вирилизации по Прадеру. Корригирующая клиторопластика в Украине проведена лишь 43% девочек с ВДКН. Подход к патогенетическому лечению пациентов с ВДКН с каріотипом 46ХХ, которые по разным причинам воспитываются в мужском гражданском поле должен быть персонифицированным.
Ключевые слова: врожденная дисфункция коры надпочечников, дети, вирилизация, опухоли яичек.
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