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"lead": "врожденное нарушение развития нервных сплетений, которые отвечают на иннервацию толстого кишечника",
"description": "<p> </p>\r\n<p style=\"line-height: 1.38; margin-top: 0pt; margin-bottom: 0pt;\" dir=\"ltr\"><span style=\"font-size: 6.999999999999999pt; font-family: Verdana; color: #000000; background-color: transparent; font-weight: 400; font-style: normal; font-variant: normal; text-decoration: none; vertical-align: baseline; white-space: pre-wrap;\">Болезнь Гиршпрунга — это врожденная патология толстой кишки с недоразвитием или отсутствием нервных сплетений в подслизистом и мышечном слоях всего толстого кишечника или его сегмента.</span></p>",
"etiology": "<p style=\"line-height: 1.38; margin-top: 0pt; margin-bottom: 0pt;\" dir=\"ltr\"><span style=\"font-size: 6.999999999999999pt; font-family: Verdana; color: #000000; background-color: transparent; font-weight: 400; font-style: normal; font-variant: normal; text-decoration: none; vertical-align: baseline; white-space: pre-wrap;\">Болезнь Гиршпрунга имеет полиэтиологическое происхождение, роль предполагающих и производящих факторов, способствующих развитию аганглиоза толстокишечной стенки, продолжает уточняться. Вероятнее всего, врожденный порок становится результатом критического повреждения генов, регулирующих формирование толстокишечных нервных структур. По мнению специалистов в области практической проктологии, возникновению аномалии Гиршпрунга способствуют:</span></p>\r\n<ul>\r\n<li><span style=\"font-size: 6.999999999999999pt; font-family: Verdana; color: #000000; background-color: transparent; font-weight: 400; font-style: normal; font-variant: normal; text-decoration: none; vertical-align: baseline; white-space: pre-wrap;\">Отягощенная наследственность. У 20% пациентов прослеживается семейный характер болезни. По результатам молекулярно-генетических исследований, при наследуемых мутациях генов нарушается миграция нейробластов из вагусного нейрогребешка, из-за чего возникает аганглиоз стенки кишечника. В 12% случаев болезни прослеживаются хромосомные аберрации, в 18% — порок проявляется в структуре наследственных синдромов.</span></li>\r\n<li><span style=\"font-size: 7pt; font-family: Verdana; background-color: transparent; font-style: normal; font-variant-numeric: normal; font-variant-east-asian: normal; vertical-align: baseline; white-space: pre-wrap;\">Дизонтогенез. Ненаследственные формы болезни связаны с влиянием внутриутробной вирусной инфекции, высокой радиации, мутагенных химических веществ, нарушающих дифференцировку нейробластов. Риск развития аномалии повышается при акушерской патологии и хронических заболеваниях беременной, сопровождающихся тканевой гипоксией, — гестозах, кардиопатологии (гипертонической болезни, сердечной недостаточности), сахарном диабете.</span></li>\r\n</ul>\r\n<p><span style=\"font-size: 6.999999999999999pt; font-family: Verdana; color: #000000; background-color: transparent; font-weight: 400; font-style: normal; font-variant: normal; text-decoration: none; vertical-align: baseline; white-space: pre-wrap;\"> </span></p>",
"pathogenesis": "<p style=\"line-height: 1.38; margin-top: 0pt; margin-bottom: 0pt;\" dir=\"ltr\"><span style=\"font-size: 6.999999999999999pt; font-family: Verdana; color: #000000; background-color: transparent; font-weight: 400; font-style: normal; font-variant: normal; text-decoration: none; vertical-align: baseline; white-space: pre-wrap;\">Развитие болезни Гиршпрунга обусловлено нарушением эмбриогенеза предположительно на 7-12 неделях гестационного срока, когда формируются нервные сплетения Мейснера (в подслизистом слое толстой кишки) и Ауэрбаха (в мышечной оболочке кишечника). Из-за преждевременного прекращения миграции нейробластов или их недостаточной дифференцировки вместо типичных подслизистого и мышечно-кишечного сплетений с ганглиями нейроструктуры кишки представлены отдельными нервными волокнами и глиальными элементами.</span></p>\r\n<p style=\"line-height: 1.38; margin-top: 0pt; margin-bottom: 0pt;\" dir=\"ltr\"> </p>\r\n<p style=\"line-height: 1.38; margin-top: 0pt; margin-bottom: 0pt;\" dir=\"ltr\"><span style=\"font-size: 6.999999999999999pt; font-family: Verdana; color: #000000; background-color: transparent; font-weight: 400; font-style: normal; font-variant: normal; text-decoration: none; vertical-align: baseline; white-space: pre-wrap;\">Чем раньше завершается миграция нейробластов, тем более протяженным является аганглиозный участок толстокишечной стенки. В слизистом слое накапливается ацетилхолинэстераза, которая вызывает спазм кишки, что является патогномоничным признаком аномалии Гиршпрунга. Из-за тонического спазмирования и отсутствия перистальтики денервированный сегмент становится функциональным препятствием для продвижения каловых масс. Хроническая задержка кишечного содержимого приводит к постоянным запорам и значительному расширению вышележащего отдела кишечника.</span></p>",
"diagnostics": "<p style=\"line-height: 1.38; margin-top: 0pt; margin-bottom: 0pt;\" dir=\"ltr\"><span style=\"font-size: 6.999999999999999pt; font-family: Verdana; color: #000000; background-color: transparent; font-weight: 400; font-style: normal; font-variant: normal; text-decoration: none; vertical-align: baseline; white-space: pre-wrap;\">Заподозрить болезнь Гиршпрунга можно при наличии характерных физикальных признаков (пальпации тестоватой «опухоли» и появлении «симптома глины» — четко определяемых через переднюю стенку живота следов сдавления толстой кишки пальцами). Диагностический поиск предполагает проведение комплексного лабораторно-инструментального обследования больного, позволяющего верифицировать диагноз. Наиболее информативными в диагностике аганглиоза являются:</span></p>\r\n<ul>\r\n<li><span style=\"font-size: 6.999999999999999pt; font-family: Verdana; color: #000000; background-color: transparent; font-weight: 400; font-style: normal; font-variant: normal; text-decoration: none; vertical-align: baseline; white-space: pre-wrap;\">Ретроградная рентгенография толстого кишечника. Обратное заполнение толстой кишки рентгеновским контрастом дает возможность визуализировать четкий переход между расширенным проксимальным отделом кишечника и суженным дистальным, который не имеет иннервации. При ирригоскопии также определяется отсутствие толстокишечной гаустрации.</span></li>\r\n<li><span style=\"font-size: 6.999999999999999pt; font-family: Verdana; color: #000000; background-color: transparent; font-weight: 400; font-style: normal; font-variant: normal; text-decoration: none; vertical-align: baseline; white-space: pre-wrap;\">Эндоскопическое исследование прямой и сигмовидной кишки. Ректороманоскопия и ректосигмоскопия проводится без специальной подготовки пациента. При патологии Гиршпрунга выявляется спазмированная кишечная стенка, отсутствие каловых масс. Проксимальнее расположен расширенный участок толстого кишечника, заполненный твердым калом.</span></li>\r\n<li><span style=\"font-size: 6.999999999999999pt; font-family: Verdana; color: #000000; background-color: transparent; font-weight: 400; font-style: normal; font-variant: normal; text-decoration: none; vertical-align: baseline; white-space: pre-wrap;\">Цитоморфологический анализ по Свенсону. Гистология биоптатов прямой и толстой кишки — «золотой стандарт» в диагностике болезни. Для получения достоверных результатов забор биологического материала осуществляют по задней стенке кишечника на протяжении 6 см, начиная от зубчатой линии. Дополнительно проводят оценку активности АХЭ.</span></li>\r\n<li><span style=\"font-size: 6.999999999999999pt; font-family: Verdana; color: #000000; background-color: transparent; font-weight: 400; font-style: normal; font-variant: normal; text-decoration: none; vertical-align: baseline; white-space: pre-wrap;\">Манометрическое исследование. Для аганглиоза Гиршпрунга характерно отсутствие рефлекторного раскрытия сфинктера прямой кишки в ответ на повышение давления. Отмечается дискоординация сокращения сфинктеров и прямой кишки. Аноректальная манометрия является важным диагностический критерием и имеет чувствительность около 85%.</span></li>\r\n</ul>\r\n<p> </p>\r\n<p style=\"line-height: 1.38; margin-top: 0pt; margin-bottom: 0pt;\" dir=\"ltr\"><span style=\"font-size: 6.999999999999999pt; font-family: Verdana; color: #000000; background-color: transparent; font-weight: 400; font-style: normal; font-variant: normal; text-decoration: none; vertical-align: baseline; white-space: pre-wrap;\">Изменения в клиническом анализе крови (лейкоцитоз, повышение СОЭ, токсическая зернистость нейтрофилов) возникают в случае осложненного варианта болезни. В биохимическом анализе крови наблюдается гипоальбуминемия, диспротеинемия. При наличии у пациента парадоксальных поносов проводят бактериологическое исследование кала для выделения патогенных возбудителей.</span></p>\r\n<p><span style=\"font-size: 6.999999999999999pt; font-family: Verdana; color: #000000; background-color: transparent; font-weight: 400; font-style: normal; font-variant: normal; text-decoration: none; vertical-align: baseline; white-space: pre-wrap;\"> </span></p>",
"treatment": "<p style=\"line-height: 1.38; margin-top: 0pt; margin-bottom: 0pt;\" dir=\"ltr\"><span style=\"font-size: 6.999999999999999pt; font-family: Verdana; color: #000000; background-color: transparent; font-weight: 400; font-style: normal; font-variant: normal; text-decoration: none; vertical-align: baseline; white-space: pre-wrap;\">Пациентам с подтвержденным диагнозом рекомендована операция, направленная на восстановление кишечной проходимости за счет удаление денервированного участка. Консервативные методы (коррекция метаболических расстройств, устранение запоров с помощью очистительных, гипертонических и сифонных клизм) применяют на этапе диагностики и предоперационной подготовки. При затягивании консервативной терапии мегаколон прогрессирует, состояние пациента ухудшается, возрастает риск послеоперационных осложнений.</span></p>\r\n<p style=\"line-height: 1.38; margin-top: 0pt; margin-bottom: 0pt;\" dir=\"ltr\"> </p>\r\n<p style=\"line-height: 1.38; margin-top: 0pt; margin-bottom: 0pt;\" dir=\"ltr\"><span style=\"font-size: 6.999999999999999pt; font-family: Verdana; color: #000000; background-color: transparent; font-weight: 400; font-style: normal; font-variant: normal; text-decoration: none; vertical-align: baseline; white-space: pre-wrap;\">При выборе объема и техники хирургического вмешательства учитывают протяженность аганглиозного сегмента, степень престенотического расширения, возраст больного. В ходе полостной операции производится резекция денервированного участка и патологически измененной расширенной части кишки, создается колоректальный анастомоз. С учетом выбранной техники хирургического лечения патологии Гиршпрунга возможны два подхода к проведению плановых вмешательств:</span></p>\r\n<p style=\"line-height: 1.38; margin-top: 0pt; margin-bottom: 0pt;\" dir=\"ltr\"> </p>\r\n<p style=\"line-height: 1.38; margin-top: 0pt; margin-bottom: 0pt;\" dir=\"ltr\"><span style=\"font-size: 6.999999999999999pt; font-family: Verdana; color: #000000; background-color: transparent; font-weight: 400; font-style: normal; font-variant: normal; text-decoration: none; vertical-align: baseline; white-space: pre-wrap;\">Одноэтапная операция. Показана при компенсированной форме болезни и небольшой длине аганглиозного сегмента. Удаляется пораженная кишка и сразу же формируется анастомоз. Преимуществом одноэтапного подхода является меньшая травматичность, однако при неправильной оценке клинической ситуации увеличивается вероятность возникновения осложнений в послеоперационном периоде.</span></p>\r\n<p style=\"line-height: 1.38; margin-top: 0pt; margin-bottom: 0pt;\" dir=\"ltr\"> </p>\r\n<p style=\"line-height: 1.38; margin-top: 0pt; margin-bottom: 0pt;\" dir=\"ltr\"><span style=\"font-size: 6.999999999999999pt; font-family: Verdana; color: #000000; background-color: transparent; font-weight: 400; font-style: normal; font-variant: normal; text-decoration: none; vertical-align: baseline; white-space: pre-wrap;\">Двухэтапная операция. Рекомендована пациентам с субкомпенсированным и декомпенсированным вариантами болезни, значительными изменениями толстой кишки выше денервированного участка, протяженным аганглиозным сегментом. На первом этапе после резекции кишечника формируется колостома, которая через некоторое время ушивается с созданием толстокишечного анастомоза в ходе реконструктивного вмешательства.</span></p>\r\n<p> </p>\r\n<p style=\"line-height: 1.38; margin-top: 0pt; margin-bottom: 0pt;\" dir=\"ltr\"><span style=\"font-size: 6.999999999999999pt; font-family: Verdana; color: #000000; background-color: transparent; font-weight: 400; font-style: normal; font-variant: normal; text-decoration: none; vertical-align: baseline; white-space: pre-wrap;\">Экстренно или срочно операция производится при возникновении острой кишечной непроходимости, перфорации кишечника, пролежне стенки кишки каловым камнем. Вмешательство выполняется в объеме резекции сигмовидной кишки, левосторонней гемиколэктомии, колопроктэктомии с наложением колостомы или илеостомы. В дальнейшем пассаж кишечного содержимого восстанавливается хирургическими методами. Диспансерный клинический осмотр прооперированных больных проводится еженедельно в течение месяца после операции, ежеквартально в течение года и ежегодно на протяжении 3-х лет.</span></p>\r\n<p><span style=\"font-size: 6.999999999999999pt; font-family: Verdana; color: #000000; background-color: transparent; font-weight: 400; font-style: normal; font-variant: normal; text-decoration: none; vertical-align: baseline; white-space: pre-wrap;\"> </span></p>",
"prevention": "<p> </p>\r\n<p style=\"line-height: 1.38; margin-top: 0pt; margin-bottom: 0pt;\" dir=\"ltr\"><span style=\"font-size: 6.999999999999999pt; font-family: Verdana; color: #000000; background-color: transparent; font-weight: 400; font-style: normal; font-variant: normal; text-decoration: none; vertical-align: baseline; white-space: pre-wrap;\">Из-за врожденного характера патологии меры специфической профилактики не разработаны. При появлении первых признаков болезни необходимо немедленно обратиться к врачу, чтобы избежать развития тяжелых осложнений.</span></p>",
"clinical_picture": "<p style=\"line-height: 1.38; margin-top: 0pt; margin-bottom: 0pt;\" dir=\"ltr\"><span style=\"font-size: 6.999999999999999pt; font-family: Verdana; color: #000000; background-color: transparent; font-weight: 400; font-style: normal; font-variant: normal; text-decoration: none; vertical-align: baseline; white-space: pre-wrap;\">Симптомы недуга зависят от количества поражённых нервных окончаний: чем их меньше, тем ниже интенсивность проявлений. Иногда патология не проявляется никакими симптомами и её выявляют уже во взрослом возрасте.</span></p>\r\n<p style=\"line-height: 1.38; margin-top: 0pt; margin-bottom: 0pt;\" dir=\"ltr\"><span style=\"font-size: 6.999999999999999pt; font-family: Verdana; color: #000000; background-color: transparent; font-weight: 400; font-style: normal; font-variant: normal; text-decoration: none; vertical-align: baseline; white-space: pre-wrap;\">При поражении большей части нервных окончаний появляются следующие признаки болезни:</span></p>\r\n<p> </p>\r\n<ul style=\"margin-top: 0; margin-bottom: 0; padding-inline-start: 48px;\">\r\n<li style=\"list-style-type: disc; font-size: 6.5pt; font-family: Verdana; color: #2e2d31; background-color: transparent; font-weight: 400; font-style: normal; font-variant: normal; text-decoration: none; vertical-align: baseline; white-space: pre;\" dir=\"ltr\">\r\n<p style=\"line-height: 1.38; margin-top: 0pt; margin-bottom: 0pt;\" dir=\"ltr\"><span style=\"font-size: 6.999999999999999pt; font-family: Verdana; color: #000000; background-color: transparent; font-weight: 400; font-style: normal; font-variant: normal; text-decoration: none; vertical-align: baseline; white-space: pre-wrap;\">отсутствие позывов к дефекации, запоры;</span></p>\r\n</li>\r\n<li style=\"list-style-type: disc; font-size: 6.5pt; font-family: Verdana; color: #2e2d31; background-color: transparent; font-weight: 400; font-style: normal; font-variant: normal; text-decoration: none; vertical-align: baseline; white-space: pre;\" dir=\"ltr\">\r\n<p style=\"line-height: 1.38; margin-top: 0pt; margin-bottom: 0pt;\" dir=\"ltr\"><span style=\"font-size: 6.999999999999999pt; font-family: Verdana; color: #000000; background-color: transparent; font-weight: 400; font-style: normal; font-variant: normal; text-decoration: none; vertical-align: baseline; white-space: pre-wrap;\">чрезмерное образование газов, вздутие живота;</span></p>\r\n</li>\r\n<li style=\"list-style-type: disc; font-size: 6.5pt; font-family: Verdana; color: #2e2d31; background-color: transparent; font-weight: 400; font-style: normal; font-variant: normal; text-decoration: none; vertical-align: baseline; white-space: pre;\" dir=\"ltr\">\r\n<p style=\"line-height: 1.38; margin-top: 0pt; margin-bottom: 0pt;\" dir=\"ltr\"><span style=\"font-size: 6.999999999999999pt; font-family: Verdana; color: #000000; background-color: transparent; font-weight: 400; font-style: normal; font-variant: normal; text-decoration: none; vertical-align: baseline; white-space: pre-wrap;\">формирование в кишечнике каловых камней;</span></p>\r\n</li>\r\n<li style=\"list-style-type: disc; font-size: 6.5pt; font-family: Verdana; color: #2e2d31; background-color: transparent; font-weight: 400; font-style: normal; font-variant: normal; text-decoration: none; vertical-align: baseline; white-space: pre;\" dir=\"ltr\">\r\n<p style=\"line-height: 1.38; margin-top: 0pt; margin-bottom: 0pt;\" dir=\"ltr\"><span style=\"font-size: 6.999999999999999pt; font-family: Verdana; color: #000000; background-color: transparent; font-weight: 400; font-style: normal; font-variant: normal; text-decoration: none; vertical-align: baseline; white-space: pre-wrap;\">скопление каловых масс в кишечнике и интоксикация организма вредными веществами, которые они выделяют.</span></p>\r\n</li>\r\n</ul>",
"image": null,
"image_alt": null,
"standard_type": 0,
"danger": 30,
"published": 1,
"parent": 7121,
"block_rubric": 181,
"standards": []
},
{
"id": 14193,
"symptoms": [],
"alternative_names": [],
"complications": [],
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}
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