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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;\">Миоглобинурия — синдром, выражающийся в наличии в моче пигмента миоглобина, являющегося хромопротеидом; наблюдается при патологическом распаде мышечного белка. При этом моча приобретает красно-бурую окраску. </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;\">Причины миоглобинурии</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;\">Наследственно-семейные патологии:</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;\">с выясненным механизмом аномалии (синдром Мак-Ардла, синдром Таруи, недостаточность карнитинпальмитилтрансферазы);</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;\">с неизвестной первичной аномалией (злокачественная форма гипертермии, расстройство синтеза молочной кислоты, неправильное окисление жирных кислот);</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;\">врожденный вариант миопатии на фоне дополнительного фактора – наркоза (дистрофия мышечная врожденная Ши и Меджи, миопатия Дюшенна, хондродистрофическая миотония).</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;\">Приобретенные патологии:</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;\">изменения, связанные с механическим нарушением (травма, инфаркт, ишемия);</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;\">гипернагрузка на мускулатуру (перегруз нетренированной мускулатуры, подъем тяжелых предметов, эпистатус, психоз, электрошок);</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;\">лихорадочные состояния (интоксикации, инфекции и пр.);</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;\">инфекции без лихорадки (грипп, столбняк, заболевания, которые сопровождаются ацидозом и нарушением электролитного баланса);</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;\">воспалительные процессы, отравления, интоксикации.</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;\">Провоцирующими факторами при миоглобинурии могут выступать:</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;\">физические гиперусилия;</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;\">чрезмерное напряжение 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;\">ограниченное поступление углеводов в организм;</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;\">воспалительные заболевания мышечной ткани;</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;\">механические повреждения мышечной ткани (травмы, краш-синдром, синдром позиционного сдавления);</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;\">перетренировки;</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;\">токовое воздействие;</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;\">интоксикации (лекарственные, алкогольные и пр.).</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;\">Миоглобинурия может развиваться у людей, страдающих алкогольной зависимостью, а также у тех, кто выкуривает более пачки сигарет ежедневно. В подобной ситуации патология возникает в результате токсического влияния этилового спирта, угарного газа и никотиновых смол.</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;\">Заболевание нередко поражает любителей кокаина, героина, амфетамина, а также наблюдается после укусов змей, скорпионов, ядовитых пауков, после отравления грибами.</span></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>",
"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 оценки состояния больного, уточнения вида патологии, сопровождающейся миоглобинурией, назначаются следующие диагностические процедуры:</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;\">Биохимическое исследование крови. Типична увеличенная концентрация миоглобина и КФК. Уровень соединений постепенно повышается на протяжении первых 12 часов. Максимальная активность миоглобина наблюдается в течение первых суток, креатинфосфокиназы – на 3-5 день. В анализах повышено количество мочевой кислоты и креатинина, наблюдаются гипокальциемия, гиперкалиемия, гиперфосфатемия, гиперурикемия.</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;\">Оценка диуреза. Имеет большую клиническую значимость из-за риска ОПН. О наличии осложнения свидетельствует уменьшение количества отделяемой мочи на 8-10%. В дальнейшем диурез сокращается на четверть, в тяжелых случаях развивается анурия. При восстановлении почечных функций объем суточного диуреза увеличивается, затем постепенно нормализуется.</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;\">Измерение субфасциального давления. Дает возможность подтвердить миофасциальный компартмент-синдром, исключить иные патологии. Выполняется специальным прибором. Полученные результаты сравнивают с диастолическим давлением. Разница менее 40 мм рт. ст. говорит в пользу сдавления 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;\">Электрофизиологические методы. Электромиография и электронейрография применяются для диагностики миопатии, дифференцировки патологии от миелита, нарушений спинномозгового кровообращения, новообразований спинного мозга. По результатам ЭМГ определяется сокращение длительности, уменьшение амплитуды мышечных потенциалов. Множество коротких пиков указывает на прогрессирующий характер процесса.</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;\">Другие методики. При сдавлении тканей в отдельных случаях выполняются УЗИ или МРТ, визуализирующие отек, разрушение скелетных мышц. Для исключения переломов и вывихов требуется рентгенологическое исследование. Тяжесть поражения миокарда при инфаркте, миопатиях, гаффской болезни оценивают с помощью ЭКГ, УЗИ сердца.</span></li>\r\n</ul>\r\n<p style=\"line-height: 1.38; margin-top: 0pt; margin-bottom: 0pt;\" dir=\"ltr\"> </p>\r\n<p style=\"line-height: 1.38; margin-top: 0pt; margin-bottom: 0pt;\" dir=\"ltr\"> </p>\r\n<p style=\"line-height: 1.38; margin-top: 0pt; margin-bottom: 0pt;\" dir=\"ltr\"> </p>\r\n<p> </p>\r\n<p style=\"line-height: 1.38; margin-top: 0pt; margin-bottom: 0pt;\" dir=\"ltr\"> </p>",
"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;\">Консервативная терапия</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;\">Устранение миоглобинурии осуществляется путем лечения основной патологии. При высоком уровне миоглобина в анализе мочи необходима срочная госпитализация в реанимационное отделение, контроль диуреза, диета с низким содержанием белка и калия. Схема медикаментозной терапии включает следующие направления:</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;\">Профилактика ОПН. Подразумевает назначение диуретиков, внутривенное капельное введение раствора гидрокарбоната натрия.</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;\">Восстановление объема жидкости. Инфузионную терапию с использованием солевых растворов начинают при поступлении, оптимально – в первые 6 часов с момента повреждения мышц. Лечение производится под контролем КОС крови и диуреза.</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;\">Устранение интоксикации и обменных нарушений. Предусматривает введение инсулина с глюкозой, агонистов бета-адренорецепторов, гидрокарбоната натрия, глюконата кальция. Для удаления токсинов из ЖКТ применяют энтеросорбенты.</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;\">Экстракорпоральная гемокоррекция. Чаще выполняется гемосорбция, иногда – плазмаферез. Больным с почечной недостаточностью показано проведение гемодиализа.</span></li>\r\n</ul>\r\n<p style=\"line-height: 1.38; margin-top: 0pt; margin-bottom: 0pt;\" dir=\"ltr\"> </p>\r\n<p style=\"line-height: 1.38; margin-top: 0pt; margin-bottom: 0pt;\" dir=\"ltr\"> </p>\r\n<p style=\"line-height: 1.38; margin-top: 0pt; margin-bottom: 0pt;\" dir=\"ltr\"> </p>\r\n<p 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 открытых травмах, газовой гангрене, формировании некротических очагов необходима антибиотикотерапия. Пациентам с гаффской болезнью назначают анальгетики, противосудорожные и антигистаминные средства, медикаменты для улучшения микроциркуляции, поливитаминные комплексы. Иногда требуются гипербарическая оксигенация, переливание свежезамороженной плазмы, тромбоцитарной и эритроцитарной массы.</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;\">Патогенетическое лечение миопатий не разработано. Симптоматическая терапия направлена на активизацию метаболических процессов в мышечной ткани. Рекомендованы аминокислоты, неостигмин, АТФ, анаболические стероиды, антихолинэстеразные препараты, кальций, калий, тиаминпирофосфат. Медикаментозные методы дополняют ЛФК, массажем, физиотерапией.</span></p>\r\n<p><strong id=\"docs-internal-guid-82a2a015-7fff-8e08-88c8-84fe27577349\" style=\"font-weight: normal;\"> </strong></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</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;\">В раннем периоде оперативные вмешательства требуются пациентам с миоглобинурией на фоне обширного рабдомиолиза, тяжелого сдавления мышц, образования участков некроза. В отдаленные сроки некоторые больные нуждаются в устранении вторичных дефектов костно-мышечной системы. С учетом стадии заболевания и имеющейся симптоматики проводятся:</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;\">Декомпрессионная фасциотомия. Показана при высоком давлении в фасциальном футляре вследствие компартмент-синдрома. Позволяет восстановить питание и сохранить жизнеспособность мышечной ткани, снизить риск возникновения ОПН.</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;\">Некрэктомия. Необходима при электротравме, термических ожогах, СДР, газовой гангрене. При обширных поражениях, подтверждении нежизнеспособности конечности, угрозе ОПН из-за поступления токсинов в организм осуществляется ампутация или экзартикуляция.</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;\">Реконструктивно-пластические вмешательства. Проводятся для улучшения функциональности конечностей. Включают пластику рубцов, миопластику, замещение дефектов мягких тканей с использованием филатовского стебля.</span></li>\r\n</ul>\r\n<p style=\"line-height: 1.38; margin-top: 0pt; margin-bottom: 0pt;\" dir=\"ltr\"> </p>\r\n<p> </p>\r\n<p style=\"line-height: 1.38; margin-top: 0pt; margin-bottom: 0pt;\" dir=\"ltr\"> </p>\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>",
"prevention": "",
"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;\">Симптоматика при миоглобинурии далеко не во всех случаях одинакова: это зависит от концентрации миоглобина и наличия сопутствующих патологических расстройств. Большинство больных жалуются на внезапную сильную слабость, болезненность в мышцах, тяжесть в поясничной области, приступы тошноты. Объем выделяемой мочевой жидкости резко и сильно снижается – не исключается также развитие анурии. Обращает на себя внимание изменение цвета мочи. Так, первые признаки миоглобинурии заключаются в том, что мочевая жидкость становится от насыщенно красной до насыщенно бурой, вплоть до почернения.</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;\">Бензидиновая проба оказывается положительной. Удельный вес мочи постепенно снижается, обнаруживается протеинурия. Мочевой осадок содержит цилиндры, гематин, эритроциты.</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;\">Если в дальнейшем развивается острая недостаточность почечной деятельности, то нарастают признаки общей интоксикации, наблюдаются азотемия, гиперкалиемия, явления ацидоза.</span></p>",
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}
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}