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"lead": "Нарушение здоровья, относящееся к группе врожденные аномалии [пороки развития] и деформации костно-мышечной системы",
"description": "Синдактилия – врожденный порок развития конечностей, заключающийся в неполном либо полном сращении двух или более пальцев кисти/стопы. При синдактилии может наблюдаться сращение как правильно развитых, так и недоразвитых и деформированных пальцев; следствием синдактилии является косметический и функциональный дефект конечности.",
"etiology": "<p>Чаще всего патология у ребенка обусловлена наследственным фактором по аутосомно-доминантному типу, и синдактилия носит семейный характер. Еще одной причиной синдактилии может быть нарушение формирования и дифференциации конечностей у плода в период внутриутробного развития, на что влияют различные неблагоприятные факторы, воздействующие на организм беременной женщины. К таковым относятся: </p>\r\n<ul>\r\n<li>Токсические воздействия: неблагоприятная экология, употребление алкоголя, прием некоторых медикаментов, профессиональные вредности и др.; </li>\r\n<li>Инфекционные заболевания (например, туберкулез, грипп, сифилис); </li>\r\n<li>Рентгеновское облучение. </li>\r\n</ul>\r\n<p>Также известны случаи приобретенной синдактилии, которая возникает после химических или термических ожогов. Сращение пальцев может быть сопутствующим генным и хромосомным синдромам пороком. </p>",
"pathogenesis": "<p>Наличие синдактилии у ребенка может быть обусловлено наследственным фактором. В этом случае имеет место аутосомно-доминантный тип наследования, а синдактилия носит семейный характер. Свыше 20% сложных форм синдактилии имеют наследственную форму. </p>\r\n<p>При отсутствии семейной истории синдактилии следует думать о нарушении формирования и дифференциации конечностей плода в процессе эмбриогенеза под влиянием различных неблагоприятных факторов. Закладка кисти происходит на 4-5 неделе внутриутробного развития; на этом сроке для плода характерна физиологическая синдактилия. При нормальном развитии на 7-8 неделе формируются пальцы кисти за счет быстрого роста пальцевых лучей и замедления роста межпальцевых промежутков. При нарушении редукции межпальцевых перегородок пальцы не разъединяются, т. е. развивается синдактилия. </p>",
"diagnostics": "<p>Синдактилия выявляется у новорожденного сразу после рождения врачом-неонатологом. Дальнейшее обследование и наблюдение ребенка проводится детским хирургом или детским ортопедом. Для исключения генных и хромосомных аномалий ребенок с синдактилией должен быть проконсультирован генетиком. </p>\r\n<p>Несмотря на то, что диагноз синдактилии может быть установлен уже в ходе визуального осмотра, для уточнения формы порока и выработки лечебной тактики необходимо проведение инструментальных исследований. Рентгенография кистей и стоп в двух проекциях позволяет оценить состояние суставов, плотность костной ткани, наличие и протяженность сращения костей. С целью выявления особенностей архитектоники сосудистой сети и кровообращения в сращенных пальцах выполняется реовазография, УЗИ, ангиография, электротермометрия.</p>",
"treatment": "<p>Существует пять методик, которыми может быть выполнена процедура устранения дефекта: </p>\r\n<ul>\r\n<li>рассечение перепонки без применения пластики; </li>\r\n<li>разделение пальцев, подвергшихся сращению путём кожной пластики региональными тканями; </li>\r\n<li>разъединение пальцев с применением свободной кожной пластики; </li>\r\n<li>рассечение пальцев при помощи комбинированной пластики; </li>\r\n<li>многоэтапная процедура, подразумевающая осуществление кожной, сухожильной, мышечной и костной пластики. </li>\r\n</ul>\r\n<p>Подходящая методика терапии устанавливается в индивидуальном порядке для каждого пациента, при этом клиницистами учитываются форма и характер патологии. </p>\r\n<p>Оптимальной возрастной категорией, для выполнения хирургической процедуры является пять лет, однако иногда врачебное вмешательство может быть проведено раньше указанного срока. </p>\r\n<p>Послеоперационный период включает в себя наложение съёмной гипсовой лонгеты, обеспечивающей иммобилизацию разогнутых или разведённых пальцев. Продолжительность применения такой меры составляет примерно четыре месяца. </p>\r\n<p>Спустя четырнадцать дней, после осуществления той или иной операции начинают восстановительную терапию при помощи физиотерапевтических процедур. </p>",
"prevention": "<p>Профилактика полидактилии предполагает медико-генетическое консультирование пар, в семьях которых имеются наследуемые случаи полидактилии; тщательное планирование беременности и исключение любых возможных неблагоприятных влияний в первом триместре развития плода. Профилактика послеоперационных рецидивов и осложнений заключается в выборе оптимального метода хирургической коррекции, проведении полноценной реабилитации. После операции дети должны находиться на диспансерном наблюдении у детского ортопеда до завершения периода интенсивного роста кисти и стопы (14-15 лет). </p>",
"clinical_picture": "<p>Наиболее часто при подобной патологии у детей происходит сращивание среднего и безымянного пальца кисти и второго и третьего пальца ноги. Крайне редко на верхних конечностях неразделенными будут безымянный палец и мизинец, большой и указательный палец. В единичных ситуациях диагностируется сращивание всех пальцев. При двустороннем протекании патологии наблюдается симметричное сращивание. </p>\r\n<p>Соединённые пальцы бывают вполне нормальными или же недоразвитыми, что будет выражаться в: </p>\r\n<ul>\r\n<li>уменьшении толщины; </li>\r\n<li>деформациях; </li>\r\n<li>укорочении; </li>\r\n<li>недостаточном количестве фаланг. </li>\r\n</ul>\r\n<p>Если присутствует базальная перепонка или при кожной разновидности синдактилии функционирование кисти остаётся в норме. Однако несмотря на то что ребёнок может выполнять широкий спектр движений, у него могут наблюдаться проблемы со стороны нормального гармоничного развития. Это означает, что может пострадать развитие: </p>\r\n<ul>\r\n<li>психомоторное; </li>\r\n<li>интеллектуальное; </li>\r\n<li>речевое. </li>\r\n</ul>\r\n<p>Неполноценность работоспособности кисти в значительной мере затрудняет учёбу, а также сильно сужает круг будущей профессиональной деятельности. </p>",
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"code": "F84.5",
"name": "Синдром Аспергера",
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"lead": "нарушение психического развития, характеризующееся серьёзными трудностями в социальном взаимодействии, а также ограниченным, стереотипным, повторяющимся репертуаром интересов и занятий",
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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;\">У синдрома Аспергера причины появления точно не определены.</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;\">Среди вероятных факторов — биологические и генетические нарушения.</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;\">Научно доказано, что дети, у которых диагностировано расстройство аутистического спектра, отличаются от других детей структурой головного мозга. Примерно пятая часть нарушений происходит на фоне генетической мутации.</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;\">К факторам риска относят:</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;\">недоношенность младенца;</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;\">возраст родителей, превышающий 40 лет;</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;\">влияние биологически вредных веществ на организм женщины во время беременности;</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;\">наследственность.</span></li>\r\n</ul>\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;\">Существует теория о воздействии негативных факторов экологии на ребенка в первые полгода жизни, что провоцирует появление признаков патологии.</span></p>",
"pathogenesis": "<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;\">Механизм развития синдрома Аспергера до конца не изучен. Ученые выдвигают несколько предположений. Проведенные исследования показали, что механизм включает изменение развития головного мозга эмбриона в скором времени после зачатия. Теория недостаточной связности предполагает, что в основе патологии лежит нарушение нейронных связей в головном мозге. Теория социального мышления объясняет аутистическое поведение нарушениями понимания собственного и чужого психического состояния. Среди других механизмов развития синдрома Аспергера выделяют нарушение выработки гормона серотонина и функциональные нарушения мозжечка (отдела головного мозга, отвечающего за координацию движений, регуляцию равновесия и мышечного тонуса).</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;\">В диагностике синдрома Аспергера широко используются методы анкетирования, интервьюирования родителей и педагогов, наблюдения за ребенком, нейропсихологические тесты. Критерии диагностики синдрома Аспергера разработаны ВОЗ и позволяют оценить способность ребенка к различным видам социальных контактов. Для исключения органических заболеваний головного мозга может потребоваться проведение неврологической диагностики (ЭЭГ, МРТ головного мозга).</span></p>\r\n<p><span id=\"docs-internal-guid-f50463de-7fff-e016-efd0-1917d29adb69\"> </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;\">Специального лечения синдрома Аспергера не существует. В качестве фармакологической поддержки в индивидуальном порядке могут назначаться психотропные препараты (нейролептики, психостимуляторы, антидепрессанты). Немедикаментозная терапия включает:</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;\">тренинги социальных навыков,</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;\">ЛФК,</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;\">занятия с логопедом,</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;\">когнитивно-поведенческую психотерапию.</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>",
"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;\">В силу неясности причин, которые способствуют развитию синдрома Аспергера, специфической профилактики не существует. Единственной профилактикой аутистических расстройств является раннее начало лечения. При адекватной терапии, заботе и внимании со стороны близких людей удается добиться хороших результатов.</span></p>\r\n<p><span id=\"docs-internal-guid-db4d411d-7fff-4ead-6d85-481c800dd84e\"> </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;\">Среди симптомов синдрома Аспергера ключевым проявлением является трудность во взаимодействии с другими людьми.</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: 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;\">Синдром Аспергера — симптомы у детей:</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;\">нарушение координации движений;</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;\">ощущение дискомфорта при изменении стандартов поведения;</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;\">потребность выполнять одни и те же действия ежедневно;</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;\">возникает речь \"механического\" типа, характеризующаяся монотонностью и отсутствием тональности;</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;\">заторможенное развитие моторики.</span></li>\r\n</ul>\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;\">Синдром Аспергера — симптомы у взрослых:</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;\">темы беседы, вызывающие недоумение у окружающих;</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;\">неспособность пациентов понять проблемы собеседника;</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;\">нарушение социального взаимодействия, проявляемого в трудности поддержания разговора;</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;\">замкнутость пациентов, отсутствие или небольшое количество интересующих тем;</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;\">невозможность воспринимать юмор или скрытый смысл предложений.</span></li>\r\n</ul>\r\n<p> </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>",
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},
{
"id": 11275,
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{
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