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"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;\">Основная причина дизэмбриогенеза структур наружного уха – негативное внешнее влияние на развитие плода во время беременности. Наследственная микротия может являться составляющей одного из генетических синдромов – Нагера, Тричера-Коллинза, Конигсмарка, Гольденхара. К наиболее распространенным тератогенным факторам, обуславливающим развитие патологии, относятся:</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;\">TORCH-инфекции. Это общее название инфекционных заболеваний, при которых имеется риск внутриутробного заражения плода и формирования пороков развития. Включают токсоплазмоз, герпес-вирусы типов 1, 2 и 3, краснуху, сифилис, цитомегаловирус, парвовирус.</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;\">Физические факторы. Обычно это ионизирующее излучение при рентгенографии или компьютерной томографии, которые проводятся по жизненным показаниям или при недиагностированной беременности. Также в эту группу входят лучевая терапия при онкопатологиях, лечение радиоактивным йодом, продолжительная гипертермия.</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;\">Вредные привычки. Губительное влияние на внутриутробное развитие ребенка оказывает употребление беременной алкогольных напитков, наркотических веществ (чаще всего – кокаина), табачных изделий.</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;\">Фармакологические препараты. Некоторые медикаменты способны провоцировать врожденные аномалии у ребенка. Это антибиотики (тетрациклины, пеницилламин), гипотензивные (эналаприл, каптоприл) средства, препараты на основе йода или лития, антикоагулянты (варфарин), гормональные средства (андрогены).</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;\">Эндокринные патологии. К ним относятся сахарный диабет в стадии декомпенсации, фенилкетонурия, недостаточность фолиевой кислоты, эндемический зоб. Отдельно выделяют гормонально активные опухоли, в том числе – андрогенпродуцирующие.</span></li>\r\n</ul>\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\"> </p>\r\n<p style=\"line-height: 1.38; margin-top: 0pt; margin-bottom: 0pt;\" dir=\"ltr\"> </p>\r\n<p> </p>\r\n<p style=\"line-height: 1.38; margin-top: 0pt; margin-bottom: 0pt;\" dir=\"ltr\"> </p>",
"pathogenesis": "",
"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;\">Постановка диагноза возможна сразу после родов на основании визуального осмотра. В последующем ребенок направляется к детскому отоларингологу для выявления сопутствующих поражений среднего и внутреннего уха, оценки функциональных возможностей звукопроводящей и звуковоспринимающей систем. Структура диагностической программы зависит от возраста пациента на момент обращения к специалисту. Помимо внешнего осмотра деформированной ушной раковины и сбора анамнеза она может включать в себя:</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;\">Изучение слухового восприятия. Основывается на разговоре или использовании звучащих игрушек. Этот тест позволяет провести первичную оценку общего звукового восприятия и звукопроводимости каждого уха в отдельности. У младенцев вместо речи используются громкие внезапные звуки.</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;\">Тональную пороговую аудиометрию. Дает возможность оценить воздушную проводимость и костное восприятие со стороны поражения. При микротии легкой, средней и тяжелой степени наблюдается кондуктивная тугоухость до 60-70 дБ. При анотии может присутствовать поражение звуковоспринимающего аппарата. Аудиометрия применяется у пациентов в возрасте от 3-4 лет, поскольку исследование требует адекватного восприятия звуковых сигналов и понимания сути теста.</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;\">Акустическую импедансометрию. Используется для оценки функционального состояния барабанной перепонки, адекватности реакции цепи слуховых косточек на звук, определения патологий внутреннего уха. На основе полученных результатов устанавливается целесообразность слухопротезирования. При необходимости это методика дополняется ABR-тестом, изучающим реакцию ЦНС на звук.</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;\">Компьютерную и магнитно-резонансную томографию. КТ височной кости в аксиальной, фронтальной и коронарной проекциях позволяет послойно визуализировать просвет наружного уха, барабанную полость и слуховые косточки или определить их отсутствие. У детей с рудиментарным проходом наружного уха по результатам исследования принимается решение о характере и объеме будущей операции, исключается наличие холестеатомы. Для диагностики потенциальных аномалий мягких тканей и изучения хода лицевого нерва показана МРТ височной кости.</span></li>\r\n</ul>\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\"> </p>\r\n<p> </p>\r\n<p style=\"line-height: 1.38; margin-top: 0pt; margin-bottom: 0pt;\" dir=\"ltr\"> </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;\">Цели лечения – устранение косметического дефекта, улучшение слуховой функции, профилактика развития осложнений. Основной метод их достижения – хирургический. Выбор оперативного вмешательства зависит от выраженности микротии, степени развития слухового канала и сопутствующей дисплазии региональных костных структур. Из социальных и психологических соображений лечение рекомендуется проводить в дошкольном периоде – в 5-6 лет. В детской отоларингологии с этой целью используются:</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;\">Аурикулопластика. В зависимости от клинической ситуации проводится пластика ушной раковины собственными тканями или (при III-IV ст.) с помощью имплантатов. Во втором случае используется аутотрансплантат, взятый из хряща VI, VII, VIII ребра с противоположной поражению стороны. Самая распространенная методика – многоэтапная реконструкция по Танзеру-Бренту.</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;\">Меатотимпанопластика. Заключается в формировании косметически и функционально приемлемого слухового прохода при наличии такой возможности. В настоящее время обычно осуществляется меатотимпанопластика по методике С. Н. Лапченко.</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;\">Слухопротезирование. При двухсторонней тяжелой кондуктивной тугоухости применяются слуховые аппараты с костным вибратором. При сохранении слухового прохода показаны классические слуховые проборы или кохлеарные имплантаты.</span></li>\r\n</ul>\r\n<p style=\"line-height: 1.38; margin-top: 0pt; margin-bottom: 0pt;\" dir=\"ltr\"> </p>\r\n<p> </p>\r\n<p style=\"line-height: 1.38; margin-top: 0pt; margin-bottom: 0pt;\" dir=\"ltr\"> </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;\">Специфических профилактических мер в отношении микротии не разработано. Неспецифическая профилактика подразумевает рациональное планирование беременности, ограничение или минимизацию влияния тератогенных факторов на плод: прием медикаментозных средств во время вынашивания ребенка строго по предписанию специалистов, отказ от вредных привычек, раннюю диагностику и лечение инфекционных патологий, эндокринных нарушений.</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;\">На основании морфологических характеристик выделяют 4 клинических формы заболевания: истинная микротия, анотия, малая и сложенная ушная раковина. Наиболее распространенная – истинная. Она проявляется наличием вертикального кожно-хрящевого валика с мочкой на конце. Последняя по сравнению с физиологическим расположением смещена кверху и кпереди. Слуховой проход часто заращен или отсутствует. Кости лицевого черепа, как правило, не деформированы. Анотия является самой редкой формой микротии. Внешне она характеризуется полным отсутствием ушной раковины или хрящевым бугорком, не имеющим мочки. Может сохраняться входное отверстие слухового канала. Часто сопровождается аномалиями развития лицевого скелета.</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;\">Сложенная раковина наружного уха представляет собой вариант микротии, при котором наблюдается недостаточное развитие верхней половины органа. Клинически это проявляется сращиванием ножки завитка и козелка. Из-за этого визуально ухо выглядит «свернутым», а его вертикальный размер существенно уменьшается. Противозавиток часто недоразвит. В тяжелых случаях верхний край завитка находится на одном уровне с козелком. Вся ушная раковина смещается кпереди и книзу. Сочетание этих изменений с дисплазией лицевого скелета является синдромом жаберной дуги. Просвет слухового прохода зачастую сращен, слух на стороне поражения резко снижен или полностью отсутствует. Малая ушная раковина – разновидность заболевания, при которой имеется относительно сформированный завиток и мочка, недостаточно развитые противозавиток и углубления наружного уха, слуховой канал стенозирован или отсутствует.</span></p>",
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},
"code": "Q20.0",
"name": "Общий артериальный ствол",
"icd_name": "Общий артериальный ствол",
"gender": 0,
"age_min": 0,
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"cause": [
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],
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"slug": "q20.0_obschiy_arterialnyy_stvol",
"lead": "врожденный порок развития сердечно-сосудистой системы, при котором вместо двух сосудов от сердца отходит один общий",
"description": "",
"etiology": "<p><span style=\"background-color: transparent; font-family: Verdana; font-size: 7pt; white-space: pre-wrap;\">Врожденные пороки сердца могут быть спровоцированы воздействием негативных факторов на ребенка во внутриутробном периоде (особенно в первые 3 месяца беременности).</span></p>\r\n<p style=\"line-height: 1.38; margin-top: 0pt; margin-bottom: 0pt;\" dir=\"ltr\"><span style=\"font-size: 7pt; background-color: transparent; font-style: normal; font-variant-numeric: normal; font-variant-east-asian: normal; vertical-align: baseline; white-space: pre-wrap;\">Заболевания матери во время беременности (могут действовать на плод неблагоприятно, в ряде случаев вызывая недостаточное или неправильное развитие):</span></p>\r\n<ul style=\"margin-top: 0; margin-bottom: 0; padding-inline-start: 48px;\">\r\n<li><span style=\"background-color: transparent; font-family: Verdana; font-size: 7pt; white-space: pre-wrap;\">хронический алкоголизм;</span></li>\r\n<li><span style=\"background-color: transparent; font-family: Verdana; font-size: 7pt; white-space: pre-wrap;\">краснуха (инфекционное заболевание, вызываемое вирусом краснухи, проявляющееся красной сыпью на теле);</span></li>\r\n<li><span style=\"background-color: transparent; font-family: Verdana; font-size: 7pt; white-space: pre-wrap;\">грипп (вирусное простудное заболевание);</span></li>\r\n<li><span style=\"background-color: transparent; font-family: Verdana; font-size: 7pt; white-space: pre-wrap;\">сахарный диабет (заболевание, связанное с недостаточностью или неполным усвоением инсулина – гормона поджелудочной железы);</span></li>\r\n<li><span style=\"background-color: transparent; font-family: Verdana; font-size: 7pt; white-space: pre-wrap;\">аутоиммунные заболевания (заболевания, при которых собственные клетки воспринимаются защитной системой организма как чужеродные).</span></li>\r\n</ul>\r\n<p><span style=\"background-color: transparent; font-size: 7pt; white-space: pre-wrap;\">Физические факторы (в основном, это действие радиации), вызывающие различные мутации и уродства. К ним относятся и лучевые методы исследования (рентгенологическое исследование), которые должны выполняться по очень серьезным показаниям, а лучше быть заменены другими способами исследования.</span></p>\r\n<p><span style=\"font-size: 7pt; background-color: transparent; font-style: normal; font-variant-numeric: normal; font-variant-east-asian: normal; vertical-align: baseline; white-space: pre-wrap;\">Химические факторы:</span></p>\r\n<ul style=\"margin-top: 0; margin-bottom: 0; padding-inline-start: 48px;\">\r\n<li><span style=\"background-color: transparent; font-family: Verdana; font-size: 7pt; white-space: pre-wrap;\">алкоголь;</span></li>\r\n<li><span style=\"background-color: transparent; font-family: Verdana; font-size: 7pt; white-space: pre-wrap;\">никотин;</span></li>\r\n<li><span style=\"background-color: transparent; font-family: Verdana; font-size: 7pt; white-space: pre-wrap;\">наркотические вещества;</span></li>\r\n<li><span style=\"background-color: transparent; font-family: Verdana; font-size: 7pt; white-space: pre-wrap;\">некоторые (в особенности сильнодействующие) лекарственные препараты.</span></li>\r\n</ul>",
"pathogenesis": "",
"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;\">Диагноз предполагают на основании клинических данных, с учетом рентгенографии органов грудной клетки и ЭКГ, точный диагноз устанавливают на основании двухмерной эхокардиографии с цветной допплеркардиографией. Катетеризация сердца часто необходима для уточнения сопутствующих аномалий перед операцией.</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;\">Рентгенография грудной клетки демонстрирует кардиомегалию различной степени выраженности с усилением легочного рисунка, право расположенной дугой аорты (примерно у 30 %) и относительно высокое расположение легочных артерий. На ЭКГ часто обнаруживают гипертрофию обоих желудочков. Существенное усиление легочного кровотока может вызывать появление признаков и гипертрофии левого предсердия.</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;\">Консервативное (безоперационное) лечение используется для лечения осложнений заболевания и для предоперационной подготовки.</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;\">Лечение самого дефекта общего артериального ствола только хирургическое. Желательно проводить его в первые месяцы жизни.</span></p>\r\n<p style=\"line-height: 1.38; margin-top: 0pt; margin-bottom: 0pt;\" dir=\"ltr\"><span style=\"background-color: transparent; font-family: Verdana; font-size: 7pt; white-space: pre-wrap;\">Паллиативная (временно облегчающая, но не излечивающая) операция Мюллера-Альберта у пациентов с тяжелой формой заболевания – на легочную артерию накладывается специальная манжетка, суживающая просвет сосуда и уменьшающая сброс смешанной крови в кровеносное русло.</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=\"background-color: transparent; font-family: Verdana; font-size: 7pt; white-space: pre-wrap;\">Радикальная (полностью устраняющая причину) коррекция порока (в основном применяется в настоящее время) — используется хирургическая операция, которая состоит из трех этапов:</span></p>\r\n<ul style=\"margin-top: 0; margin-bottom: 0; padding-inline-start: 48px;\">\r\n<li><span style=\"background-color: transparent; font-family: Verdana; font-size: 7pt; white-space: pre-wrap;\">прекращение сообщения между аортой и легочной артерией;</span></li>\r\n<li><span style=\"background-color: transparent; font-family: Verdana; font-size: 7pt; white-space: pre-wrap;\">закрытие дефекта межпредсердной перегородки при помощи заплаты;</span></li>\r\n<li><span style=\"background-color: transparent; font-family: Verdana; font-size: 7pt; white-space: pre-wrap;\">создание искусственного ствола легочной артерии с помощью специального протеза.</span></li>\r\n</ul>\r\n<p><span style=\"background-color: transparent; font-size: 7pt; white-space: pre-wrap;\">Успешное хирургическое восстановление нормального кровотока проводится в первые месяцы жизни. Это сложная операция, проводящаяся на открытом сердце с использованием аппарата искусственного кровообращения.</span></p>\r\n<p><span style=\"font-size: 7pt; background-color: transparent; font-style: normal; font-variant-numeric: normal; font-variant-east-asian: normal; vertical-align: baseline; white-space: pre-wrap;\">В дальнейшем, при удачном исходе, пациент находится под регулярным наблюдением специалистов и принимает специфические препараты, регулирующие кровоток.</span></p>\r\n<p><span style=\"font-size: 7pt; background-color: transparent; font-style: normal; font-variant-numeric: normal; font-variant-east-asian: normal; vertical-align: baseline; white-space: pre-wrap;\">С возрастом необходимо выполнять дальнейшие тяжелые операции по реконструкции сосудов, обусловленные недостатком современных синтетических материалов.</span></p>",
"prevention": "<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;\">Минимизация негативного воздействия на плод во время беременности матери:</span></p>\r\n<ul style=\"margin-top: 0; margin-bottom: 0; padding-inline-start: 48px;\">\r\n<li><span style=\"background-color: transparent; font-family: Verdana; font-size: 7pt; white-space: pre-wrap;\">благоприятное социальное и экологическое окружение;</span></li>\r\n<li><span style=\"background-color: transparent; font-family: Verdana; font-size: 7pt; white-space: pre-wrap;\">предотвращение вредного химического воздействия на плод (алкоголь, наркотики, лекарственные препараты, другие химикаты);</span></li>\r\n<li><span style=\"background-color: transparent; font-family: Verdana; font-size: 7pt; white-space: pre-wrap;\">ранняя диагностика пороков развития у детей во внутриутробном периоде благодаря современным генетическим методам исследования.</span></li>\r\n</ul>",
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