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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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"lead": "врождённая аномалия развития кожи крестцово-копчиковой области, связанная с неполной редукцией рудиментарной каудальной связки. ",
"description": "Для заболевания характерно наличие врождённых первичных свищевых втяжений в межягодичной складке. Нередко из таких втяжений выстоят волосы. ",
"etiology": "<p>Считают, что эпителиальный копчиковый ход является врожденным дефектом развития кожи, обусловленным неполной редукцией мышц и связок хвоста.</p>",
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"diagnostics": "<p>Местные изменения настолько типичны, что спутать копчиковый ход с другими поражениями этой области почти невозможно. При введении в один из свищей метиленового синего краска выходит из всех остальных ходов и вторичных свищей и не проходит в прямую кишку.</p>",
"treatment": "<p>Радикальное лечение пилонидальной кисты проводится только хирургическим путем, в условиях стационара хирургического или колопроктологического отделения. Существуют две тактики хирургического лечения:</p>\r\n<ol>\r\n<li>В острый период вскрывается и дренируется гнойник. После чего через 1–2 недели проводится радикальная операция – иссечение кисты в пределах здоровых тканей.</li>\r\n<li>В острый период сразу проводится радикальная операция – иссечение кисты единым блоком в пределах здоровых тканей (при этом необходимо сохранить целостность кисты, во избежание инфицирования операционной раны).</li>\r\n</ol>\r\n<p>Операция завершается ушиванием краев раны. Хотя, на усмотрение хирурга, рана может оставаться открытой и долго заживать вторичным натяжением; иногда до 6 месяцев (Прим. автора). При иссечении кисты копчика всегда имеется дефицит тканей, что приводит к чрезмерному натяжению краев раны и негативно сказывается на ее заживлении. Практически всегда послеоперационные раны заживают долго, причиняя значительные неудобства пациенту. При этом частота рецидива достигает 10–40%.</p>\r\n<p>Радикальная операция всегда проводится в условиях наркоза. В послеоперационном периоде пациенты в обязательном порядке получают антибактериальные препараты широкого спектра действия, а также противовоспалительные средства.</p>",
"prevention": "<p>Всем, кто перенес радикальное оперативное вмешательство по поводу эпителиального копчикового хода, желательно соблюдать следующие рекомендации: <br />- в течение трех недель после операции не разрешается сидеть, поднимать тяжести; после снятия швов целесообразно ежедневно принимать гигиенический душ с промыванием межъягодичной складки; <br />- в течение 6 месяцев после операции 2 раза в месяц необходимо производить эпиляцию в зоне операции.</p>",
"clinical_picture": "<p>Эпителиальный копчиковый ход расположен по средней линии в межъягодичной складке, слепо оканчивается в подкожной клетчатке крестцово-копчиковой области, открывается на коже одним или несколькими точечными отверстиями (первичный эпителиальный ход), и представляет собой узкую эпителиальную трубку. Продукты жизнедеятельности эпителия, выстилающего ход, периодически выделяются через точечные отверстия наружу. Закупорка или инфицирование выводных протоков, механическая травма приводят к задержке содержимого в просвете хода и способствуют возникновению воспаления. В результате эпителиальный копчиковый ход расширяется, стенка его разрушается и в воспалительный процесс вовлекается окружающая клетчатка. Развившиеся при этом абсцесс или флегмона крестцово-копчиковой области иногда достигают значительной величины и могут вскрыться наружу в виде гнойного свища, который принято называть вторичным отверстием. <br /><br />В 25-50% случаев внутри эпителиального копчикового хода или возникшей на его месте кисты содержатся волосы, которые могут внедряться в боковые стенки и образовывать дополнительные ходы. Гнойник иногда достигает значительной величины и, как правило, прорывается через кожу, образуя наружное отверстие гнойного свища. <br />Различают неосложненный и осложненный гнойным процессом эпителиальный копчиковый ход.</p>",
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},
"code": "L21",
"name": "Себорейный дерматит",
"icd_name": "Себорейный дерматит",
"gender": 0,
"age_min": 0,
"age_max": 100,
"cause": [
"0"
],
"periodicity": 3,
"slug": "l21_seboreynyy_dermatit",
"lead": "заболевание кожи, связанное с увеличением выделения и изменением качества кожного сала, и сопровождающееся воспалением кожи",
"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;\">Себорейный дерматит, или себорея, — это хроническое нарушение деятельности эпителия кожи и сальных желез, при котором развиваются воспаление, болезненные ощущения, зуд и шелушение кожи.</span></p>",
"etiology": "<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-variant-numeric: normal; font-variant-east-asian: normal; vertical-align: baseline; white-space: pre-wrap;\">Естественный химический состав секрета сальных желез кожи содержит линоленовую кислоту. Она препятствует размножению грибков. При нарушении функции сальных желез (гипо- или гиперфункции) на коже начинают развиваться болезнетворные микроорганизмы, в том числе грибки. Они имеются на поверхности кожи и в норме, но в небольшом количестве. При изменении pH кожи грибки начинают размножаться и негативно воздействуют на кожный эпителий.</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-variant-numeric: normal; font-variant-east-asian: normal; vertical-align: baseline; white-space: pre-wrap;\">К причинам развития себорейного дерматита относят следующие факторы:</span></p>\r\n<ul style=\"margin-top: 0; margin-bottom: 0; padding-inline-start: 48px;\">\r\n<li><span style=\"font-size: 7pt; color: #000000; background-color: transparent; font-variant-numeric: normal; font-variant-east-asian: normal; vertical-align: baseline; white-space: pre-wrap;\">размножение дрожжеподобного грибка Малассезия, который изменяет состав микрофлоры кожи;</span></li>\r\n<li><span style=\"font-size: 7pt; color: #000000; background-color: transparent; font-variant-numeric: normal; font-variant-east-asian: normal; vertical-align: baseline; white-space: pre-wrap;\">продолжительное пребывание в состоянии стресса;</span></li>\r\n<li><span style=\"font-size: 7pt; color: #000000; background-color: transparent; font-variant-numeric: normal; font-variant-east-asian: normal; vertical-align: baseline; white-space: pre-wrap;\">гормональные нарушения, которые часто бывают в подростковом периоде, при сбоях в работе щитовидной железы;</span></li>\r\n<li><span style=\"font-size: 7pt; color: #000000; background-color: transparent; font-variant-numeric: normal; font-variant-east-asian: normal; vertical-align: baseline; white-space: pre-wrap;\">неблагоприятные воздействия окружающей среды (например, качество воды, сухой воздух в помещении в отопительный период, резкая смена климатических условий);</span></li>\r\n<li><span style=\"font-size: 7pt; color: #000000; background-color: transparent; font-variant-numeric: normal; font-variant-east-asian: normal; vertical-align: baseline; white-space: pre-wrap;\">прием некоторых лекарственных препаратов, использование косметики, вызывающей раздражение кожи;</span></li>\r\n<li><span style=\"font-size: 7pt; color: #000000; background-color: transparent; font-variant-numeric: normal; font-variant-east-asian: normal; vertical-align: baseline; white-space: pre-wrap;\">нарушения функции желудочно-кишечного тракт</span></li>\r\n<li><span style=\"font-size: 7pt; background-color: transparent; font-variant-numeric: normal; font-variant-east-asian: normal; vertical-align: baseline; white-space: pre-wrap;\">наследственная предрасположенность.</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\"><span style=\"font-size: 7pt; font-family: Verdana; background-color: transparent; font-variant-numeric: normal; font-variant-east-asian: normal; vertical-align: baseline; white-space: pre-wrap;\">Кроме того, развитию себорейного дерматита способствуют такие факторы как несоблюдение правил личной гигиены, ожирение, частое употребление острого, соленого, сладкого.</span></p>\r\n<p><span style=\"font-size: 7pt; font-family: Verdana; background-color: transparent; font-variant-numeric: normal; font-variant-east-asian: normal; vertical-align: baseline; white-space: pre-wrap;\"> </span></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;\">Для постановки диагноза необходимы:</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;\">Осмотр дерматолога, трихолога.</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;\">Дифференциальная диагностика с псориазом, атопическим дерматитом, лишаем и другими заболеваниями со схожей клинической картиной.</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;\">Дерматоскопия – исследование кожи под микроскопом.</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;\">Анализы крови: общий, биохимический, на гормоны, сахар.</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;\">Дополнительно могут понадобиться:</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;\">Копрограмма.</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;\">Анализ кала на дисбактериоз.</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;\">УЗИ щитовидной железы, брюшной полости.</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;\">Трихограмма.</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>",
"treatment": "<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-variant-numeric: normal; font-variant-east-asian: normal; vertical-align: baseline; white-space: pre-wrap;\">Лечение себорейного дерматита должно быть комплексным, направленным как на устранение клинических проявлений, так и на коррекцию заболеваний, которые послужили причиной его возникновения. В первую очередь необходимо нормализовать режим питания, рекомендована гипоаллергенная диета. В рацион необходимо включить продукты богатые витаминами группы В, А, С, селеном и цинком. Однако следует учитывать, что гипервитаминоз и переизбыток микроэлементов в организме может стать причиной других осложнений.</span></p>\r\n<p><span><span> </span></span></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-variant-numeric: normal; font-variant-east-asian: normal; vertical-align: baseline; white-space: pre-wrap;\">Местно прибегают к этиотропной терапии себорейного дерматита, используя противогрибковые препараты, содержащие кетоконазол и миконазол. Однако из-за толстого слоя чешуек активное вещество не в полной мере воздействует на грибы, поэтому при наличии чешуек применяют аппликации с растительными маслами и кератолитическими мазями, после чего начинают противогрибковую терапию. Весь период лечения себорейного дерматита необходимо пользоваться шампунями для лечения волос, в состав которых входит цинк, кератолитические компоненты и компоненты, устраняющие избыток кожного сала, но не пересушивающие кожу.</span></p>\r\n<p><span><span> </span></span></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-variant-numeric: normal; font-variant-east-asian: normal; vertical-align: baseline; white-space: pre-wrap;\">На начальных стадиях себорейного дерматита специалисты стараются избегать назначения наружных средств, содержащих кетоконазол, поскольку длительное применение данных препаратов вызывает импотенцию у мальчиков, снижение либидо и эректильную дисфункцию - у взрослых мужчин.</span></p>\r\n<p><span><span> </span></span></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-variant-numeric: normal; font-variant-east-asian: normal; vertical-align: baseline; white-space: pre-wrap;\">Из физиопроцедур показан криомассаж и воздействие высокочастотных токов по Дарсонвалю. При криомассаже область кожи головы охлаждается, что в первую очередь снимает симптоматику кожного зуда и успокаивает воспаленную кожу, эффективность криомассажа заключается еще в улучшении микроциркуляции и в укреплении сосудов кожи головы. Это повышает местный иммунитет и улучшает состояние кожи у пациентов с себорейным дерматитом. При дарсонвализации происходит подсушивание кожи, за счет чего чешуйки отделяются быстрее, а свежий эпидермис становится более устойчивым к механическим повреждением. В результате дарсонвализации жизненный цикл клеток нормализуется и устраняется их преждевременная отслойка. Помимо этого высокочастотные токи оказывают успокаивающие действие на нервную систему, что способствует нормализации уровня адреналина и тестостерона в крови, так как при их высокой концентрации деятельность сальных желез активизируется.</span></p>\r\n<p><span><span> </span></span></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-variant-numeric: normal; font-variant-east-asian: normal; vertical-align: baseline; white-space: pre-wrap;\">При неэффективности антигрибковых препаратов назначаются гормоносодержащие мази, а при осложнении пиодермиями – антибактериальные средства. Следует учитывать, что использование этих препаратов без показаний недопустимо, так как антибиотики, воздействуя на всю микрофлору, могут вызвать усиленную колонизацию питироспоровых грибов, а гормональные препараты при длительном применении ведут к атрофии эпидермиса.</span></p>\r\n<p><span style=\"font-size: 7pt; font-family: Verdana; background-color: transparent; font-variant-numeric: normal; font-variant-east-asian: normal; 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;\">Себорейный дерматит возникает в результате иммунных и эндокринных нарушений, поэтому профилактика будет направлена на лечение основных заболеваний и исключение провоцирующих факторов. Для профилактики также следует соблюдать личную гигиену и использовать специальные средства по уходу за кожей лица, телом и волосами, избегать стрессов и контролировать состояние органов пищеварения.</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;\">Себорейный дерматит может развиваться как в области головы, так и на туловище. Себорея головы может проявляться воспалением кожи волосистой части головы, бровей, ресниц, границы роста волос, носогубных складок, наружных слуховых проходов и области за ушами. У мужчин часто локализуется в области роста бороды и усов.</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;\">Т</span><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;\">акже себорейная сыпь может поражать грудь и естественные складки тела: подмышки, пах, живот, молочные железы. Себорея лица и тела проявляется красными пятнами, сопровождающиеся зудом и шелушением, ощущением жжения и даже появлением папул, сливающихся в бляшки кольцевидной или гирляндоподобной формы. Бляшки имеют четкие границы, папулы покрыты жирными желтоватыми чешуйками. При прогрессировании болезни отмечается появление ярко-желтых корок на коже, разрастание воспаления на большую площадь поверхности кожи и присоединение бактериальной инфекции.</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;\">Себорейный дерматит волосистой части головы характеризуется отслоением белесых чешуек, которые можно принять за крупную перхоть. Однако в отличие от перхоти себорею сопровождает кожный зуд, а очаги поражения отчетливо различимы. Многие пациенты связывают сухость скальпа с появлением «перхоти», поэтому реже моют голову. Увы, такая тактика вместо желаемого результата приводит лишь к накоплению себорейных чешуек, образованию плотных корочек и патологическому воспалению кожи головы и сальных желез. Отказ от лечения приводит к истончению волос и облысению.</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;\">Себорея головы у младенцев обычно проявляется в возрасте 3 месяцев. Она представляет собой толстые желтые корки, легко удаляющиеся растительным маслом, и не беспокоит малышей. </span></p>",
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"code": "Q26",
"name": "Врожденные аномалии [пороки развития] крупных вен",
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"standard_type": 3,
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"block_rubric": 178,
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}
]
}
