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"code": "Q53",
"name": "Неопущение яичка",
"icd_name": "Неопущение яичка",
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"lead": "Нарушение здоровья, относящееся к группе врожденные аномалии [пороки развития] половых органов",
"description": "Крипторхизм — неопущение яичка в мошонку, расположение яичек вне мошонки (в брюшной полости или в паховом канале), либо неправильное опускание, при котором яичко, выйдя из пахового канала, меняет направление и располагается под кожей в паху, на бедре, в лобке или промежности.",
"etiology": "<p>Причины развития крипторхизма точно не известны. Среди них называют эндокринные нарушения, генетические отклонения и механические препятствия.</p>\r\n<ol>\r\n<li>Хромосомная патология (синдром Дауна, синдром Нунана и др.);</li>\r\n</ol>\r\n<ol start=\"2\">\r\n<li>Недоношенность. Это проявляется незрелостью соединительной ткани и связочного аппарата. У недоношенных мальчиков процесс формирования еще не завершен и продолжается 2-3 месяца после рождения. В этот период яички опускаются спонтанно; </li>\r\n</ol>\r\n<ol start=\"3\">\r\n<li>Гормональный дисбаланс у плода:\r\n<ul>\r\n<li>o дисфункцией гипофиза у плода, вследствие чего вырабатывается недостаточно лютеинизирующего гормона, ответственного за продвижение яичек;</li>\r\n<li>o нарушения развития яичек у эмбриона;</li>\r\n<li>o избыток эстрогенов у плода;</li>\r\n</ul>\r\n</li>\r\n</ol>\r\n<p>Эти состояния вызывают снижение уровня тестостерона или чувствительности к нему. На сегодня это считается основной причиной развития крипторхизма у плода. Именно тестостерон запускает механизмы, обеспечивающие опущение яичек в полость мошонки. </p>\r\n<ol start=\"4\">\r\n<li>Болезни беременной женщины\r\n<ul>\r\n<li>o грипп;</li>\r\n<li>o краснуха;</li>\r\n<li>o токсоплазмоз;</li>\r\n<li>o сахарный диабет;</li>\r\n<li>o нарушение работы щитовидной железы.</li>\r\n</ul>\r\n</li>\r\n</ol>\r\n<p>Во время болезни у женщины нарушается синтез гормонов гонадотропного типа, которые стимулируют выработку мужских половых гормонов у плода. </p>\r\n<ol start=\"5\">\r\n<li>Механические факторы\r\n<ul>\r\n<li>o фиброзные перегородки в паховом канале;</li>\r\n<li>o сужение пахового канала;</li>\r\n<li>o укорочение семенного канатика и сосудов яичка;</li>\r\n<li>o недоразвитие связок яичек;</li>\r\n<li>o незаращение влагалищного отростка брюшины.</li>\r\n</ul>\r\n</li>\r\n</ol>\r\n<p>Травмы и повреждения могут стать причиной приобретенного крипторхизма во взрослом возрасте. Например, при длительном ношении бандажа после удаления паховой грыжи, при травмировании наружного пахового кольца.</p>",
"pathogenesis": "<p>Патогенез крипторхизма определяется задержкой развития направляющей (гюнтеровой) связки яичка, играющей роль основного проводника опускающегося яичка, а также развитием соединительнотканных фиброзных препятствий по ходу его миграции. Особенно важна роль соединительнотканного барьера у входа в мошонку. Сужение этого так называемого «третьего пахового кольца» создаёт препятствие для продвижения яичка и приводит к его ретракции в паховый канал. Одновременно имеет место недоразвитие яичковой артерии и укорочение влагалищного отростка брюшины.</p>\r\n<p>Нарушение гонадотропной функции гипоталамо-гипофизарной системы приводит к дефициту дифференцирующих гормонов и к дисгенезии гонад. В дальнейшем в процессе постнатального развития диспластически-дистрофические явления в неопустившемся яичке прогрессируют. Дополнительными факторами при этом становятся нарушение температурного режима и извращение ферментативных процессов в ткани яичек, постоянная их травматизация, что приводит к накоплению в крови антител и аутоагрессии. Аутоиммунный конфликт усугубляет поражение паренхимы яичка.</p>\r\n<p>В последующие годы в яичке прогрессируют необратимые явления атрофии. Дифференциация сперматогониев и пресперматидов отсутствует, количество семенных канальцев резко снижено, наблюдается значительное разрастание соединительной ткани и развитие гиалиноза. Патологические изменения отмечаются уже в возрасте двух лет.</p>\r\n<p>Степень репродуктивной (сперматогенной) функции зависит от стадии развития патологических изменений. При двустороннем крипторхизме только у 10% мужчин наблюдается наличие сперматозоидов. Снижается и андрогенная функция.</p>\r\n<p> </p>",
"diagnostics": "<p>Пальпация или прощупывание мошонки. Если выявляется отсутствие одного яичка, то пальпируется паховый канал. При паховом расположении яичко прощупывается под мышцами, как эластичное, в меру подвижное образование. При ложном крипторхизме его можно легко вернуть в мошонку.</p>\r\n<p>Ультразвуковое исследование позволяет:</p>\r\n<ul>\r\n<li>Выявить яичко. Оно может быть над мошонкой в наружном кольце пахового канала, в паховом канале или в брюшной полости.</li>\r\n<li>Оценить его размер: уменьшенное (атрофированное) или нормальное</li>\r\n<li>Определить структуру. Она может быть нормальной однородной или неоднородной, с признаками патологических изменений.</li>\r\n</ul>\r\n<p>Доплерография – разновидность УЗИ, которая позволяет оценить работу сосудов и особенности движения крови. Этот метод исследования выявляет распределение крови в ткани яичек. Как правило, сосуды расположены равномерно и кровоснабжение нормальное.</p>\r\n<p>Дополнительные методы исследования могут понадобиться, если яичко расположено в брюшной полости и плохо просматривается при УЗИ:</p>\r\n<ul>\r\n<li>компьютерная томография(КТ)</li>\r\n<li>ядерный магнитный резонанс (ЯМТ или МРТ)</li>\r\n<li>селективная флебография</li>\r\n<li>лапароскопия</li>\r\n</ul>\r\n<p>При двустороннем крипторхизме, который сочетается с другими аномалиями половых органов, необходимы лабораторные исследования:</p>\r\n<ol>\r\n<li>Исследование кариотипа (набора хромосом) для выявления генетических аномалий</li>\r\n<li>Тест с хорионическим человеческим гонадотропином (ХГЧ) превышение нормы может свидетельствовать о появлении опухоли в яичке.</li>\r\n<li>Определение мужских половых гормонов:\r\n<ul>\r\n<li>o тестостерон</li>\r\n<li>o фолликулостимулирующий гормон (ФСГ)</li>\r\n<li>o дигидротестостерон (ДГТ)</li>\r\n</ul>\r\n</li>\r\n</ol>\r\n<p>Снижение уровня гормонов может говорить о нарушении функций яичек.</p>",
"treatment": "<p>К лечению крипторхизма приступают по достижении ребенком 9-месячного возраста, до этого срока рассчитывая на возможность самопроизвольного опущения яичка в мошонку. Медикаментозная терапия проводится с помощью внутримышечного введения человеческого ХГ или интраназального введения гонадорелина. В результате консервативной терапии удается достичь опущения яичка у 30-60% пациентов. Гормональная медикаментозная терапия крипторхизма противопоказана в пубертатном возрасте, когда наблюдается повышение уровня собственного тестостерона и гонадотропных гормонов.</p>\r\n<p>При безуспешности консервативной терапии крипторхизма к возрасту 15-24 месяцев показана хирургическая тактика – орхипексия (хирургическое низведение яичка в мошонку). В процессе операции требуется проведение биопсии яичка для исключения злокачественных процессов и дискенезии гонад. Хирургическое вмешательство абсолютно необходимо в случае перекрута, ущемления яичка, паховой грыжи, эктопии яичка.</p>\r\n<p>При выявлении гипотрофии или некроза железы выполняется резекция яичка или его удаление – орхиэктомия. В последнем случае, а также при анорхизме в более старшем возрасте требуется пересадка донорского яичка или имплантация искусственного яичка для косметической коррекции дефекта.</p>",
"prevention": "<p>Особых методов профилактики не существует. Следует серьёзно и основательно подготовиться к будущей беременности, вылечить хронические инфекции, стараться избегать стрессов и физических нагрузок. В первые месяцы беременности, когда внутренние органы плода лишь начинают закладываться, следует избегать контактов с вредными веществами (ремонт, работа на вредном предприятии), а также отказаться от приёма ненаркотических обезболивающих.</p>",
"clinical_picture": "<p>Клинически наблюдается асимметрия, уплощение, недоразвитие мошонки. Отсутствие одного яичка можно заметить или выявить при прощупывании.</p>\r\n<p>Ноющие и тянущие боли в паху и в нижней части живота. Они связаны с усилением кровообращения в яичке и сдавливанием его окружающими тканями. Эти явления проявляются в подростковом возрасте и усиливаются при физической нагрузке, кашле, натуживании, половом возбуждении.</p>\r\n<p>Крипторхизм вызывает повышение температуры в яичке и нарушение образования спермы. Это проявляется в ухудшении качества спермы и снижении ее оплодотворяющей способности (бесплодие).</p>\r\n<p>При развитии перекрута семенного канатика (перекрут яичка) появляются резкие боли в месте расположения яичка, усиливающиеся со временем. Семенной канатик перекручивается, при этом пережимаются нервы и кровеносные сосуды, обеспечивающие работу яичка. Это может привести к некрозу (гибели яичка).</p>\r\n<p> </p>",
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"standard_type": 3,
"danger": 1,
"published": 2,
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},
"code": "Q54",
"name": "Гипоспадия",
"icd_name": "Гипоспадия",
"gender": 0,
"age_min": 0,
"age_max": 89,
"cause": [
3
],
"periodicity": 1,
"slug": "q54_gipospadiya",
"lead": "Нарушение здоровья, относящееся к группе врожденные аномалии [пороки развития] половых органов",
"description": "Гипоспадия — это врожденное нарушение анатомического строения мочеиспускательного канала, при котором его отверстие открывается не в типичном месте (то есть на головке), а на задней поверхности полового члена, в промежности или на мошонке. ",
"etiology": "<p> К возможным причинам развития гипоспадии относятся: </p>\r\n<ul>\r\n<li>нарушение внутриутробного развития плода на 7-15 неделях, которое возникает из-за различных факторов: </li>\r\n<li>воздействие неблагоприятных факторов окружающей среды: курение, алкоголь, некоторые лекарственные препараты, неполноценное питание (недостаток белков, витаминов); </li>\r\n<li>резкие гормональные колебания в организме матери во время беременности, которые часто возникают из-за стрессов, нервных перегрузок, отсутствия режима труда и отдыха; </li>\r\n<li>многоплодная беременность; </li>\r\n<li>частые беременности матери; </li>\r\n<li>внутриутробные инфекции плода; </li>\r\n<li>наследственные причины (генетические нарушения). </li>\r\n</ul>",
"pathogenesis": "<p>Эмбриогенез уретры представляет собой сложный процесс, включающий закладку и формирование уретральной площадки и уретральной борозды, превращение ее в первичную уретральную трубку, редукцию заднего сегмента первичной уретральной трубки и развитие кавернозного тела уретры. </p>\r\n<p>Разделение указанных этапов формирования уретры во времени делает возможным возникновение аномалий, обусловленных нарушением развития на одном из этапов или всего сложного процесса формирования и эволюции уретральной трубки. Гипоспадия с резким недоразвитием свободной стволовой части полового члена возникает в тех случаях, когда прекращается не только замыкание уретры, но и ее дальнейшая эволюция (рассасывание первичной уретральной площадки, формирование, и рост кавернозного тела уретры). </p>\r\n<p>Это приводит к тому, что в уретре и дистальнее ее остаются эмбриональные тяжи, неспособные к росту, которые замещаются грубой рубцовой тканью. Гипоспадия полового члена с хорошо развитой стволовой частью и незначительными рубцами на волярной поверхности возникает вследствие остановки замыкания уретрального желоба, но продолжающаяся эволюция уретры, рассасывание дорсального сегмента уретральной трубки и не замкнутой уретральной площадки обеспечивают рост уретры и свободного участка полового члена по длине без существенного его искривления. </p>\r\n<p>Патогенез «гипоспадии без гипоспадии» объясняется тем, что при этой аномалии развития после формирования уретры приостанавливается ее дальнейшая эволюция: не проходят редукция заднего сегмента первичной уретральной трубки, развитие поверхностного тела уретры, а вместе с тем и рост ее по длине, что вызывает искривление полового члена. </p>\r\n<p> </p>",
"diagnostics": "<p>Диагностика гипоспадии включает: </p>\r\n<p>Осмотр наружных половых органов: </p>\r\n<ul>\r\n<li>местоположение наружного отверстия уретры (на теле полового члена, на мошонке и т.п.); </li>\r\n<li>выделение мочи во время мочеиспускания из нетипичного места (не из отверстия мочеиспускательного канала на головке полового члена); </li>\r\n<li>отсутствие единой струи мочи или ее ослабление. </li>\r\n</ul>\r\n<p>Исследование хромосомного набора человека (для определения генетического пола ребенка): исследуют хромосомы клеток (структуры клеток, содержащие ДНК – хранилище генов), определяют половой хроматин (специальное вещество, отличающееся по составу у мальчиков и девочек). </p>\r\n<p>УЗИ (ультразвуковое исследование) почек: для исключения врожденных заболеваний почек и мочевых путей. </p>\r\n<p>Цистоуретрография: суть метода в изучении строения уретры и мочевого пузыря. Для этого через катетер (тонкая трубочка), введенный в мочеиспускательный канал, в мочевой пузырь вводится рентгенконтрастное вещество (оно видно на рентгеновских пленках), и выполняется несколько снимков. </p>\r\n<p>Внутривенная урография: суть метода в изучении строения почек и мочевыводящих путей. Для этого внутривенно вводится рентгенконтрастное вещество (оно видно на рентгеновских пленках) и выполняется несколько рентген-снимков почек и мочевого пузыря. </p>",
"treatment": "<p>Лечение хирургическое. Предпочтительно в возрасте 6-18 месяцев, а при головчатой гипоспадии до 3 лет. </p>\r\n<p>Более легкие формы гипоспадии (головчатая, венечная, дистальная стволовая) лечатся с использованием одноэтапных операций. Сложные варианты (стволовая с выраженным искривлением, мошоночная, промежностная) лучше лечить в два, а иногда и в три этапа. </p>\r\n<p>Процесс хирургического лечения гипоспадии состоит из нескольких этапов: </p>\r\n<ol>\r\n<li>Выпрямление полового члена и придание ему естественного вида;</li>\r\n<li>Формирование уретры из эластичного пластического материала лишенного волосяных луковиц;</li>\r\n<li>Формирование головки полового члена;</li>\r\n<li>Выведение уретры на верхушку головки с формированием наружного отверстия уретры нормального расположения и формы;</li>\r\n<li>Устранение транспозиции и расщепления мошонки.</li>\r\n</ol>",
"prevention": "<p>Профилактика гипоспадии включает:</p>\r\n<ul>\r\n<li>Исключение или снижение влияния вредных факторов на организм беременной женщины (курение, алкоголь, необоснованный прием лекарственных препаратов, психоэмоциональных нагрузок (стрессов) и т.д.). </li>\r\n<li>Регулярное посещение женщиной акушера-гинеколога во время беременности, своевременная постановка ее на учет в женской консультации (до 12 недели беременности). </li>\r\n<li>Своевременная подготовка к планируемой беременности. </li>\r\n<li>Своевременное обращение к урологу при выявлении у мальчика гипоспадии – до 6 -тимесячного возраста. </li>\r\n</ul>",
"clinical_picture": "<p>Головчатая форма гипоспадии встречается в 75% случаев и является самой легкой и наиболее частой формой порока. Наружное отверстие уретры расположено низко, обычно сужено (меатостеноз), что затрудняет мочеиспускание. Может отмечаться искривление полового члена, усиливающееся с началом половой жизни </p>\r\n<p>Венечная форма гипоспадии (отверстие уретры расположено по краю головки полового члена) сопровождается нарушением мочеиспускания и выраженным искривлением полового члена. Моча выделяется тонкой струйкой, с усилиями; ребенок постоянно мочится себе на ноги, что заставляет его во время мочеиспускания приподнимать пенис вверх. </p>\r\n<p>Стволовая форма гипоспадии может иметь несколько вариантов, поскольку отверстие уретры может быть расположен на разных уровнях задней поверхности полового члена. Мочеиспускание по мужскому типу (стоя) сильно затруднено: дети вынуждены мочиться сидя или подтягивая половой член вверх, к животу. Значительно выражена деформация полового члена, отмечается болезненность эрекций. Половая жизнь при данной форме гипоспадии возможна, однако, если наружное отверстие уретры расположено ближе к основанию пениса, то при эякуляции сперма не попадает во влагалище. </p>\r\n<p>Мошоночная форма гипоспадии является наиболее тяжелым проявлением патологии. Наружное отверстие уретры открывается на мошонке, расщепляя ее на 2 части. Половой член резко недоразвит и искривлен, напоминает гипертрофированный клитор; мошонка похожа по виду на большие половые губы. При рождении мальчики с данной формой гипоспадии могут быть приняты за девочек с адреногенитальным синдромом (врожденной гиперплазии надпочечников). Мочеиспускание при мошоночной форме гипоспадии может осуществляться только сидя; вследствие недоразвития и деформации пениса половая жизнь становится невозможной. Раздражение кожи мошонки мочой вызывает покраснение и воспаление. </p>\r\n<p>Промежностная форма гипоспадии характеризуется расположением отверстия уретры позади мошонки. У больных определяется малый половой член, расщепление мошонки, что нередко затрудняет определение половой принадлежности ребенка. Промежностная и мошоночная формы гипоспадии чаще, чем другие, сочетаются с крипторхизмом (неопущением яичка), паховой грыжей, водянкой оболочек яичка. </p>\r\n<p>Гипоспадия без гипоспадии: мочеиспускательный канал недоразвит и короче, чем половой член. При этом наружное отверстие уретры открывается в типичном месте – на кончике головки полового члена, но за счет разницы длины уретры и полового члена (он длиннее, чем уретра) происходит искривление пениса. При эрекции пенис выгибается в форме лука, что сопровождается болезненностью, затрудняет или делает невозможным половой акт. </p>\r\n<p>Женская гипоспадия характеризуется влагалищной эктопией наружного отверстия уретры и сопровождается рецидивирующими инфекциями мочевыводящих путей (уретритами и циститами), вульвитами и вульвовагинитами, нередко - признаками гермафродитизма и псевдогермафродитизма. </p>",
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"danger": 1,
"published": 2,
"parent": null,
"block_rubric": 182,
"standards": []
}
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}