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"etiology": "<p>ВИЧ-инфекцию вызывает вирус иммунодефицита человека, относящийся к семейству ретровирусов </p>\r\n<p>ВИЧ – нестойкий вирус. Быстро погибает вне организма носителя, чувствителен к воздействию температуры (снижает инфекционные свойства при температуре 56 °С, погибает через 10 минут при нагревании до 70-80 °С). Хорошо сохраняется в крови и ее препаратах, подготовленных для переливания. Антигенная структура вируса весьма изменчива. </p>\r\n<p>Резервуаром и источником ВИЧ-инфекции является человек: страдающий СПИД и носитель. Вирус содержится в высоких концентрациях в крови, сперме, секрете вагинальных желез и менструальных выделениях. Может выделяться из женского молока, слюны, слезного секрета и ликвора, но эти биологические жидкости представляют меньшую эпидемиологическую опасность. </p>",
"pathogenesis": "<p>ВИЧ поражает клетки крови человека, имеющие на своей поверхности CD4-рецепторы: Т-лимфоциты, макрофаги и дендритные клетки. . Инфицированные вирусом Т-лимфоциты гибнут из-за разрушения вирусом, апоптоза или уничтожения цитотоксическими Т-лимфоцитами. После того, как число CD4+ T-лимфоцитов становится ниже 200 в одном микролитре крови, система клеточного иммунитета перестает защищать организм </p>\r\n<p>Первое время организм компенсирует массовую гибель иммунных клеток, вырабатывая новые, со временем компенсация становится недостаточной, количество лимфоцитов и макрофагов в крови значительно снижается, иммунная система разрушается, организм становится беззащитен как по отношению к экзогенной инфекции, так и к бактериям, населяющим органы и ткани в норме (что ведет к развитию оппортунистических инфекций). Кроме того, нарушается механизм защиты от размножения дефектных бластоцитов - злокачественных клеток. </p>\r\n<p>Заселение вирусом иммунных клеток часто провоцирует различные аутоиммунные состояния, в частности характерны неврологические расстройства в результате аутоиммунного поражения нейроцитов, которые могут развиться даже раньше, чем проявится клиника иммунодефицита. </p>",
"diagnostics": "<p>В настоящее время существуют следующие методы диагностики ВИЧ: косвенные тесты позволяют обнаружить специфические антитела к ВИЧ, которые имеются почти у 100 % ВИЧ-инфицированных; прямые тесты определяют собственно ВИЧ, антигены ВИЧ или нуклеиновые кислоты ВИЧ (вирусная нагрузка). </p>\r\n<p>Вирусную нагрузку(число молекул геномной РНК ВИЧ в мл крови) напрямую связывают со скоростью снижения количества CD4+ лимфоцитов, эта характеристика является важным прогностическим показателем на ранних стадиях заболевания </p>\r\n<p>Для определения антител к ВИЧ, необходимо использовать, по крайней мере, два разных теста: предварительный тест (скрининг-тест) и подтверждающий тест. Большинства современных скрининг-тестов основываются на иммуноферментом анализе (ИФА) или сходных методах; они обладают высокой чувствительностью (до 99 %) и специфичностью (до 99,5 %). </p>\r\n<p>Методы экспресс-анализа основаны на методах реакция агглютинации, ИФА на полимерных мембранах (тест-полоски), иммунологическом фильтрационном анализе и иммунохроматографии. Экспресс-тесты дают результат в течение 15—30 минут. </p>\r\n<p>Подсчёт CD4+ лимфоцитов является критическим параметром в мониторинге ВИЧ-инфекции и позволяет оценивать состояние иммунной системы и склонность к развитию СПИД. </p>",
"treatment": "<p>До настоящего времени не разработано лечения ВИЧ-инфекции, которое могло бы устранить вирус иммунодефицита человека из организма. Современная высокоактивная антиретровирусная терапия замедляет прогрессирование ВИЧ-инфекции и её переход в стадию СПИД, позволяя ВИЧ-инфицированному человеку жить полноценной жизнью. </p>\r\n<p>На сегодняшний день преимущественным этиотропным лечением является назначение препаратов, снижающих репродуктивные способности вируса. Антиретровирусную терапию следует начинать у пациентов с ВИЧ-инфекцией независимо от клинической стадии заболевания, при любом количестве клеток СД4, в том числе всем ВИЧ-инфицированным беременным женщинам. </p>\r\n<p>Лечение ВИЧ-инфицированных включает общеукрепляющие и поддерживающие организм средства (витамины и биологически активные вещества) и методики физиотерапевтической профилактики вторичных заболеваний. </p>",
"prevention": "<p>В настоящее время Всемирная организация здравоохранения проводит общие профилактические мероприятия по снижению заболеваемости ВИЧ-инфекцией по четырем основным направлениям: </p>\r\n<p>просвещение в вопросах безопасности половых отношений, распространение презервативов, лечение заболеваний, передающихся половым путем, пропагандирование культуры половых взаимоотношений; </p>\r\n<p>контроль над изготовлением препаратов из донорской крови; </p>\r\n<p>ведение беременности ВИЧ-инфицированных женщин, обеспечение их медицинской помощи и предоставление им средств химиопрофилактики (в последнем триместре беременности и в родах женщины получают антиретровирусные препараты, которые также на первые три месяца жизни назначаются новорожденным детям); </p>\r\n<p>организация психологической и социальной помощи и поддержки ВИЧ-инфицированных граждан, консультирование. </p>\r\n<p>В настоящее время в мировой практике особое внимание уделяют таким эпидемиологически важным в отношении заболеваемости ВИЧ-инфекцией факторам, как наркомания, беспорядочная половая жизнь. В качестве профилактической меры во многих странах производится бесплатная раздача одноразовых шприцов, метадоновая заместительная терапия. В качестве меры, способствующей снижению половой неграмотности, в учебные программы вводятся обучающие половой гигиене курсы. </p>\r\n<p> </p>\r\n<p> </p>",
"clinical_picture": "<p>Инкубационный период, время от момента заражения ВИЧ до появления симптоматики, называют серологическим окном. Сывороточные реакции на вирус иммунодефицита отрицательны: специфические антитела ещё не определяются. Средняя продолжительность инкубации составляет 12 недель; сроки могут сокращаться до 14 дней при сопутствующих ЗППП, туберкулёзе, общей астении, либо увеличиваться до 10-20 лет. В течение всего периода пациент опасен как источник ВИЧ-инфекции. </p>\r\n<p>Стадия первичных проявлений характеризуется проявлением реакции организма на активную репликацию вируса в виде клиники острой инфекции и иммунной реакции (выработка специфических антител). Вторая стадия может протекать бессимптомно, единственным признаком развивающейся ВИЧ-инфекции будет положительная серологическая диагностика на антитела к вирусу. </p>\r\n<p>Клинические проявления второй стадии протекают по типу острой ВИЧ-инфекции. Начало острое, отмечается у 50-90% пациентов спустя три месяца после момента заражения, зачастую предшествуя формированию ВИЧ-антител. Острая инфекция без вторичных патологий имеет довольно разнообразное течение: могут отмечаться лихорадка, разнообразные полиморфные высыпания на кожных покровах и видимых слизистых оболочках, увеличение лимфатических узлов, боль в горле, увеличение селезенки, диарея. </p>\r\n<p>Острая ВИЧ-инфекция обычно длится от нескольких дней до нескольких месяцев, в среднем 2-3 недели, после чего в подавляющем большинстве случаев переходит в латентную стадию. </p>\r\n<p>Латентная стадия характеризуется постепенным нарастанием иммунодефицита. Гибель иммунных клеток на этой стадии компенсируется их повышенным производством. В это время диагностировать ВИЧ можно с помощью серологических реакций (в крови присутствуют антитела к ВИЧ). Клиническим признаком может быть увеличение нескольких лимфатических узлов из разных, не связанных между собой групп, исключая паховые лимфоузлы. При этом других патологических изменений со стороны увеличенных лимфоузлов (болезненность, изменения окружающих тканей) не отмечается. Латентная стадия может продолжаться от 2-3 лет, до 20 и более. В среднем она длиться 6-7 лет.</p>",
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"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;\">Лабиринтит – это воспаление перепончатых образований и нервных структур внутреннего уха, вызванное различными вирусами, бактериями и их токсинами, а также травмой. </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;\">Нередко причина заболевания остается невыясненной. Считается, что его могут вызвать патогенные вирусы, например, гриппа, или возбудители ОРВИ. Симптомы лабиринтита могут быть связаны с инфекцией микобактериями, стрептококками, менингококками.</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;\">Другие возможные причины, вызывающие асептическое (безмикробное) воспаление внутреннего уха:</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;\">ушиб, травма головы, сотрясение мозга;</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;\">аллергическая реакция, например, при поллинозе;</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;\">злоупотребление алкоголем;</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;\">доброкачественное новообразование среднего уха (холестеатома);</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;\">прием некоторых лекарств, таких как аспирин или фуросемид, в больших дозах.</span></li>\r\n</ul>\r\n<p> </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;\">Вследствие внутреннего расположения лабиринта его инфицирование с развитием лабиринтита возможно только при распространении микроорганизмов или их токсинов из других инфекционных очагов. Наиболее часто причиной лабиринтита бывает средний отит. В костных структурах улитки и преддверия имеются окна, которые закрыты соединительнотканными мембранами. Таким образом внутреннее ухо отделено от барабанной полости среднего уха. При воспалении среднего уха происходит набухание и инфильтрация мембран, в результате чего они становятся проницаемы для микробных токсинов, проникающих из барабанной полости и обуславливающих серозное воспаление внутреннего уха. Развивающийся серозный лабиринтит приводит к повышению давления внутри лабиринта, что может сопровождаться прорывом соединительнотканных мембран окон в барабанную полость. Через возникшие отверстия во внутреннее ухо попадают патогенные микроорганизмы, вызывающие развитие гнойного лабиринтита.</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;\">Инфекционные агенты и их токсины через внутренний слуховой проход могут проникнуть во внутреннее ухо из полости черепа. Причиной лабиринтита в таких случаях является менингит, который в свою очередь может быть обусловлен менингококковой и пневмококковой инфекцией, гриппом, корью, туберкулезом, скарлатиной, сыпным тифом. </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 основе диагностики лежит тщательно собранный анамнез. На приеме врач проводит отоскопию (осмотр наружного уха и барабанной перепонки), оценку вестибулярной функции, аудиометрию (исследование слуха). Пациентам с подозрением на лабиринтит рекомендуется компьютерная томография височных костей или магнитно-резонансная томография головного мозга для выявления очага инфекции.</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 исследовании слуха выявляется тугоухость смешанного типа, чаще с преобладанием сенсоневрального компонента.</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;\">В общеклиническом и биохимическом анализах крови выявляются характерные признаки воспаления (лейко- и лимфоцитоз, ускорение СОЭ, повышение концентрации С-реактивного белка).</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;\">Медикаментозная терапия лабиринтита требует комплексного подхода. Она включает назначение антибактериальных и противовоспалительных препаратов, медикаментов, улучшающих кровообращение внутреннего уха, нейропротекторов, гипосенсибилизирующих средств. Купирование приступов головокружения при лабиринтите производится вестибулолитиками.</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;\">Тимпаногенный гнойный лабиринтит подлежит хирургическому лечению, которое заключается в проведении санирующей операции на среднем ухе, лабиринтотомии или пластики фистулы лабиринта. Если лабиринтит сопровождается мастоидитом или петрозитом, соответственно производится мастоидотомия или вскрытие пирамиды височной кости. При наличие внутричерепных осложнений лабиринтита показана лабиринтэктомия (удаление лабиринта) с дренированием полости черепа.</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;\">Стойкие нарушения слуха в результате перенесенного лабиринтита нуждаются в коррекции с помощью слухопротезирования или путем проведения слуховосстанавливающей операции (кохлеарной имплантации). С целью выбора оптимальной для пациента методики слуховой реабилитации необходима консультация сурдолога и слухопротезиста.</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;\">Профилактика лабиринтита сводится к своевременному и правильному выявлению воспаления среднего уха (средний отит).</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;\">Также необходимо дезинфицировать нос, оральную и носовую часть глотки. Санация —лечебно-профилактические меры по оздоровлению организма. Во время санации лор-органов (носовой полости, носовых пазух, глотки, гортани, ушей) разрушаются микроорганизмы, которые живут там и могут привести к различным заболеваниям при ослабленном иммунитете.</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;\">Наиболее частые симптомы лабиринтита:</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;\">головокружение, тошнота и рвота, не приносящие облегчения;</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;\">потеря равновесия, неожиданные падения;</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;\">умеренная головная боль, звон или шелест в ухе, потеря слуха;</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;\">нистагм – движения глазных яблок в сторону поражения при серозной форме или в здоровую сторону – при гнойном варианте течения.</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;\">Эти признаки усиливаются при движении головой, поворотах и взгляде вверх. Они могут сохраняться в течение нескольких дней или даже недель в зависимости от степени тяжести болезни. Даже после начатого лечения лабиринтита его симптомы могут рецидивировать, поэтому больному следует быть осторожным при вождении автомобиля и работе на высоте в течение как минимум недели после выздоровления.</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;\">Случаи, требующие срочной консультации специалиста и лечения лабиринтита:</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;\">рвота, затрудняющая прием пищи, воды или лекарств;</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;\">лихорадка, боль в ухе, прогрессирующая потеря слуха;</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;\">сильная головная боль или головокружение, сохраняющееся в течение нескольких часов или более;</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;\">двоение в глазах, нарушение речи, слабость лицевых или скелетных мышц;</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;\">недавно перенесенная травма уха или головы.</span></li>\r\n</ul>\r\n<p> </p>",
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},
"code": "Q82.1",
"name": "Ксеродерма пигментная",
"icd_name": "Ксеродерма пигментная",
"gender": 0,
"age_min": 1,
"age_max": 100,
"cause": [
"0"
],
"periodicity": 3,
"slug": "q82.1_kseroderma_pigmentnaya",
"lead": "хроническое аутосомно-рецессивное заболевание кожных покровов генетического характера",
"description": "",
"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;\">Пигментная ксеродерма — генетическое заболевание, которое передается рецессивным геном от родителей к детям. Патология имеет семейный характер, довольно часто наблюдается при близкородственных браках. Как правило, данную генетическую патологию отмечают у изолятов — обособленных человеческих популяций.</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;\">В этиологии дерматологической патологии лежит наследственно обусловленный дефицит ферментов УФ-эндонуклеазы, полимеразы-1, которые принимают активное участие в восстановлении ДНК после ее повреждения УФ-лучами. Недуг передается аутосомно-доминантным или аутосомно-рецессивным путем.</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;\">В организме больного человека полностью отсутствуют ферменты, которые нейтрализуют вредное воздействие ультрафиолета на эпидермис. При этом под воздействием УФ-лучей в структурах дермы накапливаются мутирующие клеточные структуры, что приводит к развитию рака кожи.</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;\">Чрезмерная инсоляция, длительное пребывание на солнце провоцируют развитие и обострение пигментной ксеродермы</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;\">Помимо УФ-излучения, причиной ксеродермы является ионизирующее излучение, солнечная радиация.</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;\">Факторы, причины пигментной ксеродермы:</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;\">врожденные генетические аномалии аутосомно-рецессивного характера;</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;\">повреждение ферментов УФ-эндонуклеазы;</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;\">разрушение ДНК- РНК-полимеразы;</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;\">повреждение экзонуклеазы;</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;\">увеличение концентрации в кровеносном русле пигментов порфиринов;</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;\">интенсивное воздействии радиации, УФ-облучения с длиной волн от 260 до 330 нм.</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;\">Как правило, в 70% развитие дерматологической патологии приходится на первые годы жизни, поэтому ксероз кожи у детей диагностируют в возрасте двух-трех лет. Реже ее отмечают у пациентов после 25-35 лет. Случаи более позднего развития и проявления болезни в традиционной медицине встречаются крайне редко.</span></p>\r\n<p> </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;\">Специфический метод диагностики пигментной ксеродермы проводится при помощи монохроматора и заключается в выявлении повышенной чувствительности кожного покрова к воздействию ультрафиолета.</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;\">Для уточнения диагноза дерматолог назначает биопсию пораженного участка кожи. Последующее гистологическое исследование в ранней стадии заболевания определяет гиперкератоз, отек и воспалительную инфильтрацию дермы, истончение росткового слоя, пигментацию базального слоя. В атрофической и гиперкератической стадии наблюдается атрофия эпидермиса, дегенеративные изменения коллагеновых и эластических волокон. В стадии злокачественных опухолей — атипические клетки и гистологическая картина рака кожи.</span></p>",
"treatment": "<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;\">Пациентам следует избегать воздействия УФ-лучей: носить шляпы с большими полями и вуали, применять солнцезащитные крема и мази, использовать пудры с танином. Медикаментозное лечение пигментной ксеродермы в основном симптоматическое и, к сожалению, малоэффективно. Применяют ароматические ретиноиды, токоферол, хингамин. При развитии злокачественных процессов дополнительно назначают проспидин, пиридоксин, тиамин, цианокобаламин. Папилломатозные и бородавчатые разрастания удаляют хирургически, путем криодеструкции, электрокоагуляции или удаления лазером.</span></p>",
"prevention": "<p>Профилактических мер не разработано.</p>",
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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;\">Точные причины возникновения ксантелазмы неизвестны. Ксантоматоз кожи развивается на фоне нарушенного обмена жиров в организме и представляет собой локальные отложения жиров в сосочковом слое дермы. Хотя ксантелазмы по своему строению практически не отличаются от ксантом, при их изолированном появлении по данным анализов часто не удается обнаружить значительных нарушений жирового обмена. Ксантелазмы и ксантомы часто наблюдаются у пациентов с ожирением, сахарным диабетом, микседемой, липоидным нефрозом, панкреатитом, циррозом печени, повышенным содержанием холестерина в крови.</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;\">Ксантоматоз может иметь наследственную природу. В таких случаях развивается генетически обусловленное нарушение жирового обмена. Заболевание проявляется в течение первого года жизни.</span></p>",
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"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;\">Пациентам с ксантелазмой рекомендована консультация дерматолога и эндокринолога. Характерный внешний вид и локализация ксантелазмы позволяют поставить диагноз сразу же после осмотра пациента. При осмотре ксантелазмы или ксантомы используют надавливание предметным стеклом (диаскопия). Таким образом добиваются обескровливания образований, что позволяет четко увидеть их желтый цвет.</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;\">Обязательно проводят исследование липидного обмена. Для этого назначают определение холестерина в сыворотке крови и липопротеидов. В некоторых случаях необходим дифференциальный диагноз с сирингомой, эластической псевдоксантомой и опухолевыми заболеваниями кожи.</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;\">Ксантелазма не имеет специфического лечения. При возникновении ксантелазмы или ксантоматоза на фоне заболевания, которое может быть причиной нарушения жирового обмена, необходимо лечение этого заболевания. По показаниям может быть назначен инсулин, тиреоидин.</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;\">Пациентам с выявленным нарушением липидного состава крови или повышением холестерина следует придерживаться диеты с пониженным содержанием животных жиров. Для этого животные жиры заменяют растительными, например, подсолнечным и оливковым маслом. Таким пациентам с ксантелазмой назначают липотропные препараты и средства, снижающие содержание холестерина в крови. К ним относятся: цетамифен, пирикарбат, эфиры ненасыщенных жирных кислот, липоевая кислота, кислота тиоктовая, диоспонин, клофибрат.</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;\">Из препаратов растительного происхождения липотропным действием обладают: березовые почки, корень одуванчика, плоды шиповника, кукурузные рыльца, сок подорожника, цветки бессмертника. Следует помнить, что эти препараты обладают желчегонным эффектом и их применение противопоказано при нарушениях отведения желчи по желчевыводящим путям. В лечении ксантелазмы применяют никотиновую и аскорбиновую кислоты, цианокобаламин, пиридоксин, кальция пангамат, холина хлорид.</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;\">Хирургическое лечение ксантелазм показано по косметическим соображениям. Оно проводится путем иссечения ксантелазмы, ее удаления лазером, электрокоагуляции, криовоздействия или деструкции радиоволновым способом. Удаление в большинстве случаев производится под местным обезболиванием в амбулаторных условиях.</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;\">Мелкие ксантелазмы обычно удаляют при помощи диатермокоагуляции. Более крупные бляшки отсепаровывают ножницами и пинцетом. Края раны сводят и смазывают полуторахлористым железом, которое образует прочный струп и позволяет ране зажить первичным натяжением в течение 1-1,5 недель. После отсепаровывания ксантелазм с широким основанием края раны прижигают путем диотермокоагуляции. При сочетании ксантелазм с нависанием кожной складки на веке производят их хирургическое иссечение совместно с избытком кожи верхнего века.</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;\">Не существует точных рекомендаций в плане профилактики при ксантелазме. </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;\">Специалисты могут дать лишь общие рекомендации, которые помогут пациентам выйти из группы риска и избежать появления таких образований с возрастом: </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;\">следить за своим весом и ввести в рацион больше полезных продуктов; </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;\">в рацион добавить продукты, содержащие клетчатку, а также ежедневно съедать до трехсот граммов овощей и фруктов; животные жиры заменить растительными, но употреблять их в разумных количествах; </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;\">выпивать в день не менее 1,5 литров воды; </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;\">регулярно заниматься физическими упражнениями; </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;\">постараться отказаться от курения и алкоголя; </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;\">контролировать обмен веществ, при необходимости – посещать диетолога и эндокринолога.</span></li>\r\n</ul>\r\n<p> </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;\">Ксантелазма представляет собой немного выступающую бляшку желтого цвета, расположенную на верхнем веке. Она безболезненна при ощупывании и имеет мягкую консистенцию. Как правило, ксантелазмы появляются на обоих веках. Они могут быть единичными и множественными. В последнем случае ксантелазмы могут сливаться, образуя бугристые элементы. Иногда ксантелазмы сливаются в сплошную желтую полоску с неравномерным контуром, проходящую через все верхнее веко.</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;\">Для ксантелазмы характерно внезапное, без предшествующих изменений кожи века, появление. Развитие ее происходит постепенно и достаточно медленно, не доставляя пациенту никаких субъективных ощущений. Ксантелазма может достигать размеров от маленькой горошины до крупной фасолины. Она никогда не подвергается трансформации в злокачественное новообразование и не представляет угрозы для организма человека. Однако крупные и множественные ксантелазмы, не смотря на свою безобидность с медицинской точки зрения, представляют заметную косметическую проблему.</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;\">Если ксантелазмы являются проявлением ксантоматоза, то они часто сопровождаются поражением нижнего века, на котором образуются ксантомы. При этом ксантомы локализуются и на других участках кожного покрова: лицо, шея, коленные и локтевые суставы, разгибательная поверхность конечностей, ягодицы и др. Возможно их появление на слизистой мягкого и твердого неба, губ. Ксантомы могут быть мелкоузелковыми (эруптивными), плоскими в виде бляшек или бугорчатыми в виде крупных узлов с неровной поверхностью. Диаметр этих образований варьирует от 2 мм до 5 см. В отдельных случаях ксантомы сливаются между собой и происходит образование крупной бляшки с дольчатым строением.</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;\">Возникшие ксантомы и ксантелазмы сохраняются всю жизнь. Постепенно они растут в размерах, их количество увеличивается. Появление ксантелазм и ксантом у маленьких детей может быть признаком наследственного гиперхолестеринемического ксантоматоза, который затем проявляется нарушениями со стороны сердечно-сосудистой системы и печени, может сопровождаться образованием костных кист.</span></p>",
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