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},
"code": "J63.2",
"name": "Бериллиоз",
"icd_name": "Бериллиоз",
"gender": 0,
"age_min": 20,
"age_max": 100,
"cause": [
"0"
],
"periodicity": 1,
"slug": "j63.2_berillioz",
"lead": "воспаление соединительной ткани легких, вызванное вдыханием пыли или паров, которые содержат бериллий",
"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;\">Патология относится к категории профессиональных заболеваний. Возникает у шахтёров, добывающих бериллий, литейщиков и работников предприятий, использующих данный металл или его соединения в производстве. Бериллий широко применяется в самолётостроении, космических исследованиях, атомной энергетике и других отраслях промышленности. Его пары и пыль обычно поступают в респираторный тракт при вдыхании. При кожном контакте с очень высокими концентрациями бериллиевых соединений возможен трансдермальный путь проникновения вредных веществ во внутренние органы и системы. Бериллий, присутствующий в организме женщины, преодолевает фетоплацентарный и лактационный барьеры.</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;\">Элемент высокотоксичен. При вдыхании воздуха с высоким содержанием кислых солей бериллия возникает острый тяжёлый химический пневмонит. Допустимые концентрации соединений этого металла вызывают хронический бериллиоз, которому подвержены генетически предрасположенные лица. Корреляция распространенности данной формы болезни с количеством металла во внешней среде и временем его воздействия отсутствует. При наличии предрасположенности заболевают не только пациенты, непосредственно контактирующие с бериллием и его сплавами, но и люди, работающие или проживающие в соседних помещениях.</span></p>",
"pathogenesis": "<p style=\"line-height: 1.38; margin-top: 0pt; margin-bottom: 0pt;\" dir=\"ltr\"><span style=\"font-size: 7pt; font-family: Verdana; background-color: transparent; font-variant-numeric: normal; font-variant-east-asian: normal; vertical-align: baseline; white-space: pre-wrap;\">Патогенез бериллиоза изучен не до конца. В ходе научных исследований в области пульмонологии и профпатологии установлено, что существуют определённые аллели генов, кодирующих предрасположенность к возникновению хронической бериллиевой болезни. При ингаляции паров бериллиевых соединений частицы металла попадают в воздухоносные пути, накапливаются в лёгких и внутригрудных лимфатических узлах. Очень медленно и постепенно в патологический процесс вовлекаются другие органы. У генетически предрасположенных к данной патологии пациентов бериллий распознаётся, как антиген. Формируется клеточный иммунный ответ. В процессе реакции выделяются цитокины, повреждающие собственные ткани.</span></p>\r\n<p style=\"line-height: 1.38; margin-top: 0pt; margin-bottom: 0pt;\" dir=\"ltr\"> </p>\r\n<p style=\"line-height: 1.38; margin-top: 0pt; margin-bottom: 0pt;\" dir=\"ltr\"><span style=\"font-size: 7pt; font-family: Verdana; background-color: transparent; font-variant-numeric: normal; font-variant-east-asian: normal; vertical-align: baseline; white-space: pre-wrap;\">В лёгких образуются многочисленные эпителиоидно-клеточные гранулёмы без казеоза. Они локализуются в интерстициальной ткани, окружают сосудистые пучки, бронхиолы и мелкие бронхи. При этом утолщаются межальвеолярные перегородки, страдает газообмен. В дальнейшем происходит расширение бронхиол, появление воздушных булл, грубое нарушение архитектоники ацинуса – формирование сотового лёгкого. Газообмен ухудшается ещё больше. Развивается тяжёлая дыхательная недостаточность. Растворимые бериллиевые соединения при попадании в дыхательную систему вызывают выраженную экссудативную воспалительную реакцию и отёк лёгочного интерстиция.</span></p>\r\n<p><span style=\"font-size: 7pt; font-family: Verdana; background-color: transparent; font-variant-numeric: normal; font-variant-east-asian: normal; vertical-align: baseline; white-space: pre-wrap;\"> </span></p>",
"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;\">При подозрении на бериллиоз большое диагностическое значение имеет установление контакта пациента с бериллиевыми парами или пылью в анамнезе. При осмотре обращает на себя внимание акроцианоз либо диффузный цианоз кожных покровов. Иногда обнаруживаются подкожные гранулёмы. Дыхание учащенное, одышка усиливается при незначительной физической нагрузке и разговоре. Наблюдается дистальная гипертрофическая остеоартропатия. Пальпируются увеличенные лимфатические узлы. Характерным признаком бериллиоза является увеличение локтевых лимфоузлов до размера горошины. Для окончательного подтверждения диагноза выполняются:</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;\">Физикальное исследование. Перкуторно выявляется коробочный звук с обеих сторон. При аускультации обычно выслушиваются рассеянные крепитирующие или влажные мелкопузырчатые хрипы, локализованные преимущественно в проекции базальных отделов лёгких. Иногда хрипы сухие, слышен шум трения плевры. Тоны сердца обычно приглушены, определяется акцент II тона на лёгочной артерии.</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;\">Рентгенография, КТ лёгких. Наблюдаются интерстициальные и гранулёматозные изменения. На рентгенологических снимках просматривается усиление и деформация лёгочного рисунка, единичные или многочисленные милиарные тени, буллёзная эмфизема, плевральные сращения. В терминальной стадии болезни лёгкие напоминают пчелиные соты (сотовое лёгкое).</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;\">Функциональная диагностика. Исследование функции внешнего дыхания позволяет выявить рестриктивные нарушения. На ЭКГ отмечаются признаки гипертрофии правых отделов сердца, тахикардия, аритмии. При проведении пульсоксиметрии определяется снижение насыщения крови кислородом.</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;\">Лабораторные анализы. Являются вспомогательными методами. При остром процессе и во время обострения хронической формы в анализе периферической крови наблюдается лейкоцитоз, ускорение СОЭ. При биохимическом исследовании крови выявляется гипоальбуминемия и гипергаммаглобулинемия. В моче, плевральной жидкости, промывных водах бронхов можно обнаружить бериллий.</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;\">Кожные и серологические пробы. Появление эритемы в результате накожной аппликации раствора хлорида бериллия с большой долей вероятности свидетельствует о наличии бериллиевой болезни. Для подтверждения сенсибилизации к бериллию производится ряд серологических тестов (РПГА, РТМЛ, РПЛ и другие) с сывороткой крови.</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;\">Необходимо немедленно исключить любой контакт с патогенным агентом. В случае острого отравления назначаются системные кортикостероиды, антигистаминные и противокашлевые лекарственные средства, щелочные ингаляции. При присоединении вторичной бактериальной инфекции рекомендованы антибиотики. Острый бериллиевый пневмонит является показанием для оксигенотерапии. При необходимости выполняется искусственная вентиляция лёгких, используются кардиопротекторы.</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;\">Препаратом выбора для осуществления базисной терапии хронического процесса являются кортикостероидные гормоны. При неэффективности лечения кортикостероидами иногда назначается метотрексат. Базисное лечение проводится в два этапа. Сначала применяются гормоны системного действия. Для предотвращения развития возможных побочных эффектов вместе с ними используются гастро- и ангиопротекторы, препараты кальция и калия. На втором этапе пациент получает ингаляционные кортикостероиды через небулайзер или в виде дозированного аэрозоля. Больному дыхательная гимнастика и лечебная физкультура. В последней стадии болезни возможна трансплантация сердца и лёгких.</span></p>",
"prevention": "<p> </p>\r\n<p style=\"line-height: 1.38; margin-top: 0pt; margin-bottom: 0pt;\" dir=\"ltr\"><span style=\"font-size: 6.999999999999999pt; font-family: Verdana; color: #000000; background-color: transparent; font-weight: 400; font-style: normal; font-variant: normal; text-decoration: none; vertical-align: baseline; white-space: pre-wrap;\">В целях первичной профилактики данного профессионального заболевания необходимо использовать средства индивидуальной защиты при работе с соединениями бериллия. Все лица, работающие во вредных условиях, подлежат регулярным профилактическим осмотрам с применением рентгенологического исследования органов грудной полости и определением наличия гиперчувствительности к бериллию.</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;\">Клиническая картина острой интоксикации бериллием обычно складывается из симптомов раздражения верхних дыхательных путей и конъюнктивы. Болезнь проявляется слезотечением, светобоязнью и покраснением слизистых оболочек глаз. Пациента беспокоит заложенность носа или, наоборот, обильные светлые слизистые выделения. Изменяется голос – появляется охриплость или осиплость, присоединяется поверхностный лающий кашель. Присутствуют боли и першение в горле, жжение за грудиной. Такая форма заболевания обычно купируется самостоятельно в течение нескольких дней после прекращения контакта с вредным агентом, реже осложняется тяжёлой бериллиевой пневмопатией.</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;\">Острый бериллиоз с поражением альвеолярной ткани протекает тяжело. Характерно повышение температуры тела до фебрильных и гипертермических значений, сопровождающееся ознобами. Пациент жалуется на резкую головную боль, одышку, мучительный сухой или малопродуктивный кашель. В мокроте может присутствовать примесь крови. Развивается миокардиодистрофия, проявляющаяся различными нарушениями сердечного ритма, тахикардией и снижением артериального давления. Заболевание обычно затяжное, длится 2-3 месяца. Временное улучшение состояния периодически сменяется ухудшением.</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;\">Хронический бериллиоз развивается спустя 1-30 лет после контакта с пылью, дымом или парами соединений бериллия. Начало болезни постепенное. Медленно нарастает общая слабость, утомляемость. Пациент резко худеет, потеря массы тела может достигать 20 кг в течение 3-6 месяцев. Неуклонно прогрессирует одышка, ей сопутствуют постоянный сухой кашель, боли в груди разной интенсивности и локализации. Дистальные фаланги пальцев конечностей деформируются по типу барабанных палочек. Больного беспокоит тахикардия. Из-за увеличения печени появляются боли в правом подреберье. Состояние пациента ухудшается в период обострения. Усиление кашля, одышки и слабости нередко сопровождается повышением температуры.</span></p>",
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"standard_type": 0,
"danger": 52,
"published": 1,
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}
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}
