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},
"code": "D55",
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"icd_name": "Анемия вследствие ферментных нарушений",
"gender": 0,
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3,
0
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"slug": "d55_anemiya_vsledstvie_fermentnyh_narusheniy",
"lead": "относится к группе гемолитических анемий, развитие которых связано с дефицитом различных ферментов",
"description": "",
"etiology": "<p>Относительно часто встречается анемия вследствие дефицита пируваткиназы. Заболевание наследственное, передается гомозиготно.</p>\r\n<p>Пируваткиназа (а также другие ферменты гликолизного обмена) принимает участие в синтезе АТФ, дефицит этого фермента ведет к снижению содержания АТФ в эритроцитах, а это, в свою очередь, – к нарушению концентрации ионов натрия и калия в эритроцитах (Na+↑; K+↓). В результате такого сдвига возникает функциональная и морфологическая неполноценность красных кровяных клеток.</p>",
"pathogenesis": "",
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"treatment": "<p>Проводится заместительная терапия (ферментативная), используются симптоматические средства. Нередко назначение рибофлавина дает стойкий положительный эффект.</p>",
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},
"code": "L10",
"name": "Пузырчатка [пемфигус]",
"icd_name": "Пузырчатка [пемфигус]",
"gender": 0,
"age_min": 18,
"age_max": 100,
"cause": [
3,
0
],
"periodicity": 4,
"slug": "l10_puzyrchatka_pemfigus",
"lead": "хроническое заболевание аутоиммунной природы, которое характеризуется появлением пузырей на клинически здоровой коже и слизистых оболочках.",
"description": "Не заразное, крайне тяжелое заболевание кожи.",
"etiology": "<p>Наиболее вероятными причиной пузырчатки являются нарушения аутоиммунных процессов, в результате чего клетки организма становятся для иммунной системы антителами. Нарушение антигенной структуры клеток эпидермиса происходит под воздействием внешних факторов, в частности воздействие ретровирусов и агрессивных условий окружающей среды.</p>",
"pathogenesis": "<p>Повреждающее действие на клетки эпидермиса и выработка специфических антигенов приводит к нарушению связи между клетками в результате чего и образуются пузыри.</p>",
"diagnostics": "<p>Диагностика пузырчатки осуществляется врачом-дерматологом и основана на совокупности результатов осмотра, цитологического, гистологического и иммунологического обследований. При всех формах заболевания отмечают отложение иммуноглобулинов G в поверхностных слоях кожи.</p>",
"treatment": "<p align=\"justify\">Все пациенты больные пузырчаткой должны находиться на диспансерном наблюдении у дерматолога, рекомендован щадящий режим работы, отсутствие физических нагрузок и избегание инсоляции. Частая смена нательного и постельного белья предотвращает присоединение вторичной инфекции.</p>\r\n<p> </p>\r\n<p align=\"justify\">Показано назначение глюкокортикостероидов сразу в высоких дозах, так как иначе терапевтического эффекта достигнуто не будет, после купирования острых проявлений пузырчатки дозировки гормональных препаратов постепенно снижают до минимально эффективных. В лечении пузырчатки применяют методы экстракорпоральной гемокоррекции: гемосорбцию, криоаферез и мембранный плазмаферез. </p>\r\n<p> В качестве местного лечения пузырчатки используют анилиновые красители и неагрессивные антисептические растворы.</p>\r\n<p>Прогноз пузырчатки всегда неблагоприятный, так как в случае отсутствия адекватного лечения смерть пациентов наступает довольно быстро от присоединившихся осложнений. Длительная гормональная терапия в высоких дозах повышает риск возникновения побочных эффектов, но при отказе от глюкокортикостероидов пузырчатка начинает рецидивировать</p>",
"prevention": "",
"clinical_picture": "<p align=\"justify\">Пузырчатка имеет длительное волнообразное течение, и отсутствие адекватного лечения приводит к нарушению общего состояния пациента.</p>\r\n<p> </p>\r\n<p align=\"justify\">При вульгарной форме пузырчатки пузыри локализуются по всему телу, имеют разный размер и наполнены серозным содержимым, при этом покрышка на пузырях вялая и тонкая.</p>\r\n<p> </p>\r\n<p align=\"justify\">Дебютирует вульгарная пузырчатка как правило на слизистой рта и носа, а потому пациенты длительно и безуспешно получают терапию у стоматологов и отоларингологов. На этой стадии пузырчатки пациенты предъявляют жалобы на боль во время приема пищи и при разговоре, гиперсаливацию и специфический неприятный <a href=\"/symptom/zapah_izo_rta/\" title=\"Перейти на страницу симптома Запах изо рта\">запах изо рта</a>. Продолжительность этого периода от трех месяцев до года, после чего пузырчатка принимает распространенный характер и в воспалительный процесс вовлекаются кожные покровы.</p>\r\n<p>Иногда пациенты не замечают наличие пузырей из-за их небольшого размера и тонкой покрышки, пузыри вскрываются быстро, а потому основные жалобы больных пузырчаткой на данном периоде – это болезненные <a href=\"/symptom/izmeneniya_struktury_kozhi/\" title=\"Перейти на страницу симптома Изменения структуры кожи\">эрозии</a>. Проводится длительная и безуспешная терапия стоматитов, прежде чем диагностируют пузырчатку. Пузыри, которые локализуются на коже, имеют тенденцию к самопроизвольному вскрытию с обнажением эрозированной поверхности и с остатками покрышки, которая ссыхается в корки.</p>\r\n<p> <a href=\"/symptom/izmeneniya_struktury_kozhi/\" title=\"Перейти на страницу симптома Изменения структуры кожи\">Эрозии</a> при пузырчатке ярко-розовые, с гладкой глянцевой поверхностью, отличаются от эрозий при других заболеваниях склонностью к периферическому росту и к генерализации с формированием обширных очагов поражения. Если пузырчатка принимает такое течение, то общее состояние пациента ухудшается, развивается интоксикация, присоединяется вторичная инфекция и без должного лечения такие пациенты умирают.</p>\r\n<p> При вульгарной пузырчатке синдром Никольского положительный в очаге поражения и порой на здоровой коже - при незначительном механическом воздействии происходит отслойка верхнего слоя эпителия.</p>",
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