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"code": "D86",
"name": "Саркоидоз",
"icd_name": "Саркоидоз",
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"lead": "Нарушение здоровья, относящееся к группе отдельные нарушения, вовлекающие иммунный механизм",
"description": "Саркоидоз – это заболевание, при котором могут поражаться различные органы и системы (чаще всего легкие), характеризующееся образованием в пораженных тканях гранулем (ограниченные очаги воспаления, состоящие из скопления нормальных и видоизмененных клеток).",
"etiology": "<p>Этиология саркоидоза до конца не выяснена. В настоящее время существуют лишь теории возможного вклада в развитие заболевания следующих факторов:</p>\r\n<ul>\r\n<li>наследственная предрасположенность (отмечаются семейные случаи заболевания);</li>\r\n<li>инфекции, которая рассматривается не как причина развития саркоидоза, а как пусковой фактор. В качестве таких пусковых агентов обсуждаются микобактерии туберкулеза, Chlamydophila pneumoniae, Borrelia burgdorferi, отдельные виды вирусов: вирус гепатита С, вирус герпеса;</li>\r\n<li>факторов окружающей среды: вдыхание металлической пыли (пыль алюминия, бария, бериллия, кобальта, меди, золота, титана и циркония);</li>\r\n<li>курения, которое не рассматривается как непосредственная причина саркоидоза, однако у курильщиков заболевание протекает тяжелее (в большей степени снижаются функциональные возможности легких).</li>\r\n</ul>\r\n<p> </p>",
"pathogenesis": "<p>В основе патогенеза саркоидоза лежит накопление Т-лимфоцитов вследствие иммунного ответа Th-1 типа. По неизвестной пока причине активированные макрофаги и лимфоциты скапливаются в том или ином органе и продуцируют повышенное количество интерлейкинов, фактора некроза опухолей (TNF-альфа). TNF-альфа считают ключевым цитокином, участвующим в формировании гранулемы при саркоидозе.</p>\r\n<p>Развитие гранулематозной реакции связывают также с нарушениями механизмов апоптоза (запрограммированной гибели клеток) иммунокомпентентных клеток.</p>\r\n<p>Как правило, саркоидоз характеризуется полиорганным течением. Легочный саркоидоз начинается с поражения альвеолярной ткани и сопровождается развитием атипичного воспаления в легких с последующим образованием саркоидных гранулем в легких. В дальнейшем гранулема либо рассасывается, либо прорастает соединительной тканью, превращаясь в бесклеточную гиалиновую (стекловидную) массу.</p>\r\n<p>При прогрессировании саркоидоза легких развиваются выраженные нарушения вентиляционной функции, как правило, по рестриктивному типу (снижается способность легких расправляться). При сдавлении лимфатическими узлами стенок бронхов возможны обструктивные нарушения, а иногда и развитие зон гиповентиляции и спадение альвеол (ателектаз).</p>\r\n<p>Одновременно с легкими поражаются различные группы внутригрудных лимфатических узлов, которые увеличиваются за счет скопления в них множественных гранулем Саркоидные гранулемы нередко обнаруживаются в печени и селезенке, в почках. Поражение почек может приводить к почечной недостаточности.</p>\r\n<p>Кожа вовлекается в процесс гораздо реже легких и ВГЛУ, однако именно поражение кожи часто служит основной причиной обращения больных к врачу. Поражения кожи отмечаются приблизительно в 10 - 15% случаев. Выявляются мелкоузелковая, крупноузелковая, а также атипичные формы саркоидоза кожи.</p>",
"diagnostics": "<p>Диагностика саркоидоза включает:</p>\r\n<ul>\r\n<li>Сбор анамнеза (истории развития) и жалоб заболевания (расспрос о беспокоящих симптомах).</li>\r\n<li>Общий осмотр (осмотр кожных покровов, лимфатических узлов, выслушивание легких с помощью фонендоскопа).</li>\r\n<li>Общий анализ крови.</li>\r\n<li>Биохимический анализ крови.</li>\r\n<li>Туберкулиновая проба (реакция Манту) – внутрикожный тест на определение специфической чувствительности организма к микобактериям туберкулеза.</li>\r\n<li>Рентгенография органов грудной клетки, которая позволяет обнаружить увеличение внутригрудных лимфатических узлов и изменения в легких.</li>\r\n<li>Компьютерная томография органов грудной клетки.</li>\r\n<li>Спирометрия (спирография). Позволяет оценить воздушную проходимость дыхательных путей и способность легких к расправлению.</li>\r\n<li>Проба с бронходилятатором – выполнение спирометрии до и после ингаляции препарата, расширяющего бронх. Применяется для оценки обратимости сужения бронха.</li>\r\n<li>Исследование газового состава крови (определение напряжения в крови кислорода, углекислого газа, оценка насыщения крови кислородом).</li>\r\n<li>Бодиплетизмография – метод оценки функции внешнего дыхания, который позволяет определять все объемы и емкости легких, в том числе те, которые не определяются спирографией.</li>\r\n<li>Фибробронхоскопия – исследование, позволяющее осмотреть слизистую оболочку бронхов изнутри и исследовать ее клеточный состав с помощью специального аппарата.</li>\r\n<li>Биопсия легких, внутригрудных лимфатических узлов и кожных проявлений саркоидоза. Исключение составляет узловатая эритема: ее биопсия не является информативной.</li>\r\n<li>Электрокардиография (ЭКГ), холтеровское мониторирование ЭКГ, эхокардиография (ЭХО-КГ, УЗИ сердца) – для оценки функционального состояния сердца.</li>\r\n<li>Магнитно-резонансная томография (МРТ) сердца, позитронно-эмиссионная томография (ПЭТ), сцинтиграфия миокарда – редко использующиеся дорогостоящие методы, позволяющие оценить локализацию и объем поражения сердца.</li>\r\n<li>УЗИ органов брюшной полости – при их вовлечении в процесс.</li>\r\n</ul>",
"treatment": "<p>Этиотропной терапии саркоидоза не существует. Во всех случаях основной тактикой врача является сопоставление необходимости назначения лечения с тяжестью последствий от применения современной кортикостероидной, цитостатической и биологической («таргетной») терапии.</p>\r\n<p>В лечении больных саркоидозом используются средства, направленные на подавление воспалительной реакции и предотвращение фиброзной трансформации гранулем. В настоящее время наиболее эффективным средством являются кортикостероиды. Кортикостероидная терапия проводится длительно: в течение 6-8 мес.</p>\r\n<p>Используют также нестероидные противовоспалительные средства (делагил, плаквенил), антиоксиданты (тиосульфат натрия, альфа-токоферол, аскорутин), иммуномодулирующую терапию.</p>\r\n<p>Немедикаментозные методы лечения: разгрузочно-диетическая терапия, физиотерапия, плазмаферез.</p>",
"prevention": "<p>В качестве возможной меры профилактики можно рекомендовать избегать инфекционных заболеваний и контакта с металлической пылью (пыль алюминия, бария, бериллия, кобальта, меди, золота, титана и циркония).</p>\r\n<p>Для профилактики обострений саркоидоза следует стараться соблюдать здоровый образ жизни. Самое главное при поражении лёгких — не курить, так как это может привести к затруднению дыхания и осложнениям болезни. Также следует избегать лекарств и контактов с химикатами, вредными для печени, с токсичными летучими веществами, пылью, парами, газами, которые могут повреждать лёгкие. При саркоидозе наблюдается увеличение кальция в крови, что может привести к образованию камней в почках и мочевом пузыре. Поэтому следует избегать употребления продуктов, богатых кальцием. По этой же причине не следует загорать.</p>\r\n<p> </p>",
"clinical_picture": "<p>Общие симптомы:</p>\r\n<ul>\r\n<li>повышение температуры тела;</li>\r\n<li>слабость;</li>\r\n<li>повышенная утомляемость;</li>\r\n<li>боли и припухлость в суставах;</li>\r\n<li>боли в мышцах.</li>\r\n</ul>\r\n<p>Поражение легких:</p>\r\n<ul>\r\n<li>боль и дискомфорт в грудной клетке;</li>\r\n<li>одышка (чувство нехватки воздуха, затруднение дыхания);</li>\r\n<li>кашель – обычно сухой.</li>\r\n</ul>\r\n<p>Поражение кожи:</p>\r\n<ul>\r\n<li>узловатая эритема – наиболее часто встречаемый вариант поражения. Узлы размером от одного до нескольких сантиметров в диаметре, плотные, полушаровидной формы, располагаются в коже или подкожной клетчатке, несколько возвышаются над поверхностью кожи. Сначала узлы бывают ярко-красными, но по прошествии нескольких дней они приобретают синевато-багровый оттенок и в итоге становятся желтыми или зеленоватыми. Узлы могут рассасываться без лечения, иногда оставляя после себя временную пигментацию;</li>\r\n<li>феномен « оживших рубцов» — нарастающее покраснение, уплотнение и болезненность длительно существовавших послеоперационных рубцов и посттравматических шрамов;</li>\r\n<li>саркоидные бляшки – обычно располагаются симметрично на коже туловища, ягодиц, конечностей и лица, представляют собой безболезненные четко очерченные возвышающиеся участки уплотнения кожи багрово-синюшной окраски по периферии с западением более бледной окраски в центре. Сохраняются длительно и требуют лечения;</li>\r\n<li>ознобленная волчанка– хроническое поражения кожи носа, щек, ушей и пальцев. Пораженные участки кожи уплотнены, окрашены в красный, пурпурный или фиолетовый цвет вследствие большого числа сосудов в зоне изменений. Обострения возникают обычно в зимнее время.</li>\r\n</ul>\r\n<p>Изменения сохраняются длительно и мало поддаются лечению.</p>\r\n<p>Поражение периферических лимфатических узлов:</p>\r\n<ul>\r\n<li>чаще в процесс вовлечены задние и передние шейные лимфатические узлы, надключичные, локтевые, подмышечные и паховые;</li>\r\n<li>лимфатические узлы увеличены, плотно-эластической консистенции, не размягчаются.</li>\r\n</ul>\r\n<p>Возможно поражение глаз, печени, селезенки, почек, ЛОР-органов, сердца, нервной системы и желудочно-кишечного тракта.</p>",
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"lead": "состояние, характеризующееся повышенным содержанием метгемоглобина (окисленного гемоглобина) в крови и тканевой гипоксией",
"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;\">Метгемоглобин может быть увеличен за счет двух основных причин. Часть случаев заболевания имеет генетическую причину, это означает, что врожденная метаболическая ошибка способствует увеличению доли метгемоглобина в крови. В большинстве случаев метгемоглобинемия, однако, является приобретенной, а не врожденной. Воздействие некоторых окислителей может привести к превращению гемоглобина в метгемоглобин. Известные токсины, которые могут вызвать это заболевание:</span></p>\r\n<ul style=\"margin-top: 0; margin-bottom: 0; padding-inline-start: 48px;\">\r\n<li style=\"list-style-type: disc; font-size: 6.5pt; font-family: Verdana; color: #252525; background-color: transparent; font-weight: 400; font-style: normal; font-variant: normal; text-decoration: none; vertical-align: baseline; white-space: pre;\" dir=\"ltr\">\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;\">анилиновые красители;</span></p>\r\n</li>\r\n<li style=\"list-style-type: disc; font-size: 6.5pt; font-family: Verdana; color: #252525; background-color: transparent; font-weight: 400; font-style: normal; font-variant: normal; text-decoration: none; vertical-align: baseline; white-space: pre;\" dir=\"ltr\">\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;\">нитраты или нитриты;</span></p>\r\n</li>\r\n<li style=\"list-style-type: disc; font-size: 6.5pt; font-family: Verdana; color: #252525; background-color: transparent; font-weight: 400; font-style: normal; font-variant: normal; text-decoration: none; vertical-align: baseline; white-space: pre;\" dir=\"ltr\">\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;\">употребление большого количества различных лекарств;</span></p>\r\n</li>\r\n<li style=\"list-style-type: disc; font-size: 6.5pt; font-family: Verdana; color: #252525; background-color: transparent; font-weight: 400; font-style: normal; font-variant: normal; text-decoration: none; vertical-align: baseline; white-space: pre;\" dir=\"ltr\">\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;\">лидокаин, пролокаин;</span></p>\r\n</li>\r\n<li style=\"list-style-type: disc; font-size: 6.5pt; font-family: Verdana; color: #252525; background-color: transparent; font-weight: 400; font-style: normal; font-variant: normal; text-decoration: none; vertical-align: baseline; white-space: pre;\" dir=\"ltr\">\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;\">дапсон – препарат, используемый для лечения и профилактики пневмоцистной инфекции.</span></p>\r\n</li>\r\n</ul>\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;\">С химической точки зрения метгемоглобин формируется присутствием в эритроцитах железа в трехвалентной форме вместо обычной формы железа. Это приводит к снижению доступности кислорода к тканям. Симптомы заболевания обычно проявляются пропорционально уровню метгемоглобина. Только внешне болезнь проявляется, пока количество метгемоглобина не достигнет показателя 15%. Если эта величина превышается, появляются неврологические и кардиологические симптомы, являющиеся следствием гипоксии.</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;\">Важным диагностическим признаком метгемоглобинемии служит темно-коричный цвет крови, которая, будучи помещенной в пробирку или на фильтровальную бумагу, не изменяет свой цвет на красный. При положительной пробе проводится спектроскопия, определение концентрации MtHb, активности НАД-зависимой метгемоглобинредуктазы, электрофорез гемоглобина.</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 общем анализе крови может присутствовать компенсаторный эритроцитоз, увеличение Hb, ретикулоцитоз, уменьшение СОЭ. При исследовании биохимических показателей крови определяется незначительная билирубинемия, обусловленная увеличением непрямой фракции пигмента. Для хронической метгемоглобинемии типично появление в эритроцитах телец Гейнца-Эрлиха.</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;\">У больных с энзимопенической или токсической метгемоглобинемией показательна терапевтическая проба с внутривенным введением метиленового синего – после инъекции цианоз быстро исчезает, а кожа и видимые слизистые приобретают розовую окраску.</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;\">При анализе причин метгемоглобинемии важно выяснить, имел ли больной контакт с токсическими веществами, принимал ли метгемоглобинобразующие лекарственные препараты. При подозрении на врожденную метгемоглобинемию изучается родословная, проводится консультация генетика, определяется тип наследования патологии крови. </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;\">Ранняя диагностика заболевания и начало лечения, в особенности при обнаружении приобретенной формы метгемоглобинемии, имеют решающее значение в управлении симптомами заболевания и сохранении жизненных функций пациента. Больные нуждаются в комплексных исследованиях, иногда и инвазивных, для исключения сердечной и легочной патологии. Первая помощь при метгемоглобинемии заключается во введении дополнительного количества кислорода и устранении отравляющего вещества, токсина, окислителя.</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;\">Пациенты с разовой метгемоглобинемией, возникшей в результате отравления отравляющим веществом, могут быть выписаны после 6-часового наблюдения, если симптомы заболевания были устранены. Симптоматические пациенты с длительной метгемоглобинемией должны наблюдаться в стационаре, в особенности при осложняющих факторах, таких как анемия, интенсивный цианоз, сопутствующее заболевание сердца или периферических сосудов.</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;\">Лекарственным средством первой линии против метгемоглобинемии является внутривенное введение метиленового синего. Рекомендовано также переливание крови и гипербарическое лечение кислородом. В качестве вспомогательного средства лечения применяется аскорбиновая кислота (витамин С) в таблетках или любой другой фармакологической форме.</span></p>",
"prevention": "<p><span id=\"docs-internal-guid-3b15565d-7fff-b966-7154-4d9082696da3\"><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;\">Пациентам с подобной патологией следует избегать контакта с метгемоглобинобразующими веществами, переохлаждений и других провоцирующих факторов. Профилактика врожденной метгемоглобинемии заключается в проведении медико-генетической консультации для выявления гетерозиготных носителей среди будущих родителей.</span></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;\">Учитывая тип метгемоглобинемии, следует знать, что симптомы заболевания могут отличаться. В некоторых случаях присутствует только внешнее проявление (легкие наследственные подтипы заболевания), могут иметь место и тяжелые неврологические симптомы. Нормальное количество метгемоглобина в человеческой крови составляет около 1% (диапазон от 0-3%).</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;\">Симптомы, связанные с различным уровнем метгемоглобина, превышающим норму:</span></p>\r\n<ul style=\"margin-top: 0; margin-bottom: 0; padding-inline-start: 48px;\">\r\n<li style=\"list-style-type: disc; font-size: 6.5pt; font-family: Verdana; color: #252525; background-color: transparent; font-weight: 400; font-style: normal; font-variant: normal; text-decoration: none; vertical-align: baseline; white-space: pre;\" dir=\"ltr\">\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;\">3-15% - незначительные изменения цвета кожи (например, бледная, серая, синяя);</span></p>\r\n</li>\r\n<li style=\"list-style-type: disc; font-size: 6.5pt; font-family: Verdana; color: #252525; background-color: transparent; font-weight: 400; font-style: normal; font-variant: normal; text-decoration: none; vertical-align: baseline; white-space: pre;\" dir=\"ltr\">\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;\">15-20% - цианоз, хотя не исключено бессимптомное течение;</span></p>\r\n</li>\r\n<li style=\"list-style-type: disc; font-size: 6.5pt; font-family: Verdana; color: #252525; background-color: transparent; font-weight: 400; font-style: normal; font-variant: normal; text-decoration: none; vertical-align: baseline; white-space: pre;\" dir=\"ltr\">\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;\">25-50% - головная боль, одышка, головокружение, обмороки, общая слабость, спутанность сознания, учащенное сердцебиение, боль в груди;</span></p>\r\n</li>\r\n<li style=\"list-style-type: disc; font-size: 6.5pt; font-family: Verdana; color: #252525; background-color: transparent; font-weight: 400; font-style: normal; font-variant: normal; text-decoration: none; vertical-align: baseline; white-space: pre;\" dir=\"ltr\">\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;\">50-70% - аномальные сердечные ритмы; изменения психического состояния, бред, судороги, кома, глубокий ацидоз;</span></p>\r\n</li>\r\n<li style=\"list-style-type: disc; font-size: 6.5pt; font-family: Verdana; color: #252525; background-color: transparent; font-weight: 400; font-style: normal; font-variant: normal; text-decoration: none; vertical-align: baseline; white-space: pre;\" dir=\"ltr\">\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;\">> 70% - летальный исход.</span></p>\r\n</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;\">Физические признаки: изменение цвета кожи и крови, судороги, изменение цвета конъюнктивы, скелетные аномалии, явная умственная отсталость.</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;\">Системы организма с высокими потребностями в кислороде (например, центральная нервная система и сердечно-сосудистая система) первыми проявляют признаки заболевания. Кровь, содержащая большее, чем необходимо количество метгемоглобина, приобретает красно-коричневый цвет.</span></p>",
"image": null,
"image_alt": null,
"standard_type": 0,
"danger": 50,
"published": 1,
"parent": null,
"block_rubric": 43,
"standards": []
},
{
"id": 6203,
"symptoms": [
{
"id": 3238,
"synonyms": [
{
"name": "Селезенка болит"
},
{
"name": "Острая боль в селезенке"
},
{
"name": "Сильные боли в области селезенки"
},
{
"name": "Боль в селезенке при кашле"
},
{
"name": "Боль в селезенке при беге"
}
],
"body_points": [
{
"x": 821,
"y": 3452,
"r": 330
}
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"code": "D70",
"name": "Агранулоцитоз",
"icd_name": "Агранулоцитоз",
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"cause": [
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"periodicity": 1,
"slug": "d70_agranulocitoz",
"lead": "Нарушение здоровья, относящееся к группе другие болезни крови и кроветворных органов",
"description": " \r\nАгранулоцитоз – клинико-лабораторный синдром, основное проявление которого состоит в резком снижении или полном отсутствии нейтрофильных гранулоцитов в периферической крови, что сопровождается повышением восприимчивости организма к грибковым и бактериальным инфекциям. \r\n",
"etiology": "<p>К причинам развития агранулоцитоза относят: </p>\r\n<ul>\r\n<li>Ионизирующая радиация и лучевая терапия, химические вещества (бензол), инсектициды. </li>\r\n<li>Лекарственные средства могут вызвать агранулоцитоз в результате прямого угнетения кроветворения (цитостатики, вальпроевая кислота, карбамазепин, бета-лактамные антибиотики), либо действуя как гаптены (препараты золота, антитиреоидные препараты и пр.). </li>\r\n<li>Аутоиммунные заболевания (например, красная волчанка, аутоиммунный тиреоидит). </li>\r\n<li>Вирусные инфекции (вызванные вирусом Эпштейна – Барра, цитомегаловирус, желтая лихорадка, вирусные гепатиты) обычно сопровождаются умеренной нейтропенией, однако в некоторых случаях может развиться агранулоцитоз. </li>\r\n<li>Тяжелые генерализованные инфекции (как бактериальные, так и вирусные). </li>\r\n<li>Исхудание. </li>\r\n<li>Генетические нарушения. </li>\r\n</ul>",
"pathogenesis": "<p>В зависимости от причины возникновения и механизма развития патологии, различают следующие виды агранулоцитоза: </p>\r\n<ol>\r\n<li>Миелотоксический.</li>\r\n<li>Иммунный: аутоиммунный;гаптеновый. </li>\r\n<li>Генуинный.</li>\r\n</ol>\r\n<p>Миелотоксический агранулоцитоз возникает в результате подавляющего воздействия неблагоприятных факторов на красный костный мозг, и представляет собой глубокую депрессию кроветворного ростка, ответственного за продукцию гранулоцитов. </p>\r\n<p>В основе аутоиммунного агранулоцитоза лежит патологическая реакция иммунной системы, сопровождающаяся образованием антител к собственным нейтрофилам. Такая разновидность агранулоцитоза встречается при аутоиммунном тиреоидите, ревматоидном артрите, системной красной волчанке и других коллагенозах. Агранулоцитоз, развивающийся при некоторых инфекционных заболеваниях (гриппе, инфекционном мононуклеозе, малярии, желтой лихорадке, брюшном тифе, вирусном гепатите, полиомиелитеи др.) также имеет иммунный характер. </p>\r\n<p>Гаптеновый агранулоцитоз возникает, когда в иммунных реакциях участвуют гаптены – вещества, сами по себе безвредные, однако при определенных условиях способные провоцировать иммунные реакции, разрушающие гранулоциты. В роли гаптенов чаще всего выступают лекарственные вещества, так что гаптеновый агранулоцитоз рассматривают, как грозное осложнение различных лечебных мероприятий. О генуинном агранулоцитозе говорят в тех случаях, когда причина резкого снижения количества гранулоцитов в крови остается неизвестной. </p>",
"diagnostics": "<p> </p>\r\n<p>Наиболее важным для подтверждения агранулоцитоза является исследование общего анализа крови и пункция костного мозга. Картина периферической крови характеризуется лейкопенией (1-2х109/л), гранулоцитопенией (менее 0,75х109/л) или агранулоцитозом, умеренной анемией, при тяжелых степенях – тромбоцитопенией. При исследовании миелограммы выявляется уменьшение количества миелокариоцитов, снижение числа и нарушение созревания клеток нейтрофильного ростка, наличие большого количества плазматических клеток и мегакариоцитов. Для подтверждения аутоиммунного характера агранулоцитоза производится определение антинейтрофильных антител. </p>\r\n<p>Всем пациентам с агранулоцитозом показано проведение рентгенографии легких, повторные исследования крови на стерильность, исследование биохимического анализа крови, консультация стоматолога и отоларинголога. Дифференцировать агранулоцитоз необходимо от острого лейкоза, гипопластической анемии. Также необходимо исключение ВИЧ-статуса</p>",
"treatment": "<p> В первую очередь необходимо устранение причинного повреждающего фактора, срочная госпитализация больного агранулоцитозом, желательно в гематологическое отделение, и помещение его в стерильные условия. </p>\r\n<p>Необходим тщательный уход за кожей и слизистыми оболочками. Подкожные и внутримышечные инъекции следует заменить внутривенными, чтобы предотвратить развитие абсцессов. </p>\r\n<p>Назначаются антибиотики широкого спектра действия, по показаниям — противогрибковые и противовирусные препараты, иммуноглобулин. Могут применяться также препараты гранулоцитарного колониестимулирующего фактора (КСФ). При некротической энтеропатии больному следует проводить парентеральное питание. </p>\r\n<p>При аутоиммунном агранулоцитозе производится угнетение аутоиммунной агрессии, больному показаны глюкокортикоиды в высоких дозах до нормализации числа гранулоцитов в крови с последующей постепенной отменой гормонов. При гаптеновом агранулоцитозе введение глюкокортикоидов не эффективно. </p>\r\n<p>При лечении цитостатического агранулоцитоза средствами выбора являются препараты рекомбинантных гемопоэтических факторов роста. </p>",
"prevention": "<p>Чтобы не допустить развитие агранулоцитоза, следует избегать контакта с фактором, способным вызвать заболевание. Во время приема цитостатиков необходим тщательный контроль показателей гемограммы (картины крови). </p>\r\n<p>Большой проблемой является профилактика повторных гаптеновых агранулоцитозов. При гаптеновых агранулоцитозах - 80% смертельных исходов, и прогноз ухудшается при повторном применении вызвавшего нейтропению лекарства. Установить, какой препарат является гаптеном, нелегко, поэтому нужно навсегда исключать из употребления все препараты, которые применялись перед появлением агранулоцитоза. </p>",
"clinical_picture": "<p>Клиническая картина во многом напоминает симптомы цитостатической болезни и проявляется в виде поражения организма различными инфекциями в результате снижения эффективности иммунного ответа. Характерны (особенно при лекарственном агранулоцитозе) острое начало и быстрое нарастание клинических симптомов. Иногда возникновению основных признаков агранулоцитоза предшествует короткий скрытый период, характеризующийся слабостью, недомоганием, головными болями. Возникает лихорадка, афтозный стоматит, ангина, озноб, боль в суставах. Развиваются язвенно-некротические поражения слизистой оболочки полости рта в виде изъязвлений, покрытых сероватым налётом, некротических бляшек и язв с грязно-серым налётом на миндалинах (агранулоцитарная ангина). </p>\r\n<p>Процесс распространяется на нёбные дужки, язычок, твёрдое нёбо, дёсны и порой сопровождается незначительным увеличением регионарных лимфатических узлов, чаще всего шейных и подчелюстных. Данные изменения могут быть также на слизистой оболочке желудочно-кишечного тракта, лёгких, печени, мочевого пузыря, половых органов. </p>\r\n<p>При гаптеновом агранулоцитозе развитие болезни бурное. После приёма препаратов, вызвавших агранулоцитоз, возникает нарастающая слабость, боль в горле, повышение температуры тела до 39—40 °С, появляется и нарастает гнилостный запах изо рта (глоточно-ротовая форма). Нередко развиваются стоматит, ларингоспазм и асфиксия по причине скопления некротических плёнок и отёка гортани. К ошибке в диагностике может привести тот факт, что начальные проявления кишечно-некротической энтеропатии и поражение слизистых оболочек ротовой полости опережают появление лабораторных показателей агранулоцитоза. </p>",
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}
]
}
