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"id": 53,
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},
"code": "Q56",
"name": "Неопределенность пола и псевдогермафродитизм",
"icd_name": "Неопределенность пола и псевдогермафродитизм",
"gender": 0,
"age_min": 0,
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"slug": "q56_neopredelennost_pola_i_psevdogermafroditizm",
"lead": "патологическое состояние, при котором половые железы и хромосомный набор не соответствует некоторым (или даже большинству) внутренних и наружных половых органов",
"description": "",
"etiology": "<p>Мужской псевдогермафродитизм наблюдается при различных эндокринных расстройствах (дефектах синтеза тестостерона, его метаболизма и эффектов на клетки-мишени).</p>\r\n<p>Этиология женского псевдогермафродитизма до конца не изучена. Установлена связь с гиперплазией коры надпочечников, при которой наблюдается нарушение синтеза кортизола и его прогестероновых предшественников. Имеет значение и прием лекарственных средств, содержащих андрогены и прогестины. Также существуют данные, свидетельствующие в пользу наличия наследственной передачи аутосомно-рецессивным путем.</p>",
"pathogenesis": "<p>В результате ослабления воздействия тестостерона на клетки-мишени ведущую роль в развитии половых органов берут на себя эстрогены, вырабатываемые в надпочечниках. Таким образом, фенотип становится женским.</p>\r\n<p>При женском псевдогермафродитизме отмечается <a href=\"/symptom/povyshennaya_chuvstvitelnost/\" title=\"Перейти на страницу симптома Повышенная чувствительность\">повышенная чувствительность</a> XX-плода к воздействию андрогенов во время критического периода эмбрионального развития (8-я неделя внутриутробного развития). Все это приводит к развитию разной степени выраженности губомошоночного сращения, формированию урогенитального синуса и увеличению клитора.</p>",
"diagnostics": "<p>Основана на осмотре гениталий, вторичных половых признаках, кариотипировании, исследовании уровня эстрогенов, андрогенов, лапаротомии, макроскопическом и гистологическом исследовании гонад.</p>",
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