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},
"code": "B59",
"name": "Пневмоцистоз",
"icd_name": "Пневмоцистоз",
"gender": 0,
"age_min": 0,
"age_max": 100,
"cause": [
4
],
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"slug": "b59_pnevmocistoz",
"lead": "Протозойное заболевание, обусловленное Pneumocystis carinii, которая является одной из самых частых причин развития пневмоний у лиц с ослабленным иммунитетом.",
"description": "Инфекционное заразное заболевание.",
"etiology": "<p>Возбудитель - Pneumocystis carinii относится к типу простейших, подтип Sporozoa, класс Haplospora. Местом естественного обитания в нормальных условиях являются легкие. Жизненный цикл построен таким образом, что в альвеолярной ткани можно обнаружить 2 основные формы этого микроорганизма: мелкие одноядерные трофозоиты (1-5 мкм) и размножающиеся бинарным делением цисты (10 мкм), имеющие толстую стенку и содержащие от 2 до 8 клеток (1-2 мкм), называющихся спорозоитами. Когда зрелая циста разрывается, спорозоиты либо продолжают цикл развития в альвеолах, превращаясь в трофозоит, либо выходят во внешнюю среду (с капельками слизи при кашле) и, в случае обретения нового хозяина, также включаются в свой цикл развития.Пневмоцисты широко распространены у людей и животных. Передаются воздушно-капельным путем. Известно, что от 1 до 10% здоровых людей являются носителями пневмоцист. </p>",
"pathogenesis": "<p>Заболевания развиваются только у лиц с первичными или приобретенными нарушениями иммунитета. Ведущую роль играют нарушения механизмов клеточного иммунитета. У больных пневмоцистозом отмечается местная и системная продукция антител, не обладающих, однако, протективным действием. Не встречая противодействия со стороны клеточных элементов защиты (в первую очередь альвеолярных макрофагов), пневмоцисты постепенно заполняют всю полость альвеол. При гистологическом исследовании обнаруживают типичный пенистый вакуолизированный альвеолярный <a title=\"Перейти на страницу симптома Выпот\" href=\"../../../symptom/vypot/\">экссудат</a>, содержащий микроорганизмы, белки сыворотки крови и остатки органических веществ. Отмечается выраженная дистрофия альвеолоцитов I типа, репаративная гипертрофия альвеолоцитов II типа. Указанные изменения постепенно приводят к нарушению газообменной функции альвеолярного эпителия и, соответственно, тяжелой (при отсутствии лечения фатальной) дыхательной недостаточности.</p>\r\n<full></full>\r\n<p>Отягощающим моментом является образование участков ателектаза, что усугубляет нарушения вентиляции и газообмена. У ослабленных детей можно обнаружить интенсивный инфильтрат из плазматичеких клеток, что и легло в основу прежнего названия болезни - интерстициальная плазмоклеточная пневмония. Вне ткани легких пневмоцисты практически не обнаруживаются.</p>",
"diagnostics": "<p align=\"justify\">Клинические, физикальные и рентгенологические данные при пневмоцистозе не являются патогномоничными, что затрудняет своевременную диагностику заболевания. Между тем, пневмоцистную пневмонию всегда следует исключать у иммунокомпрометированных пациентов. С целью верификации пневмоцистоза проводится комплекс лабораторных и инструментальных исследований.</p>\r\n<full></full>\r\n<p align=\"justify\">На рентгенограммах и КТ легких в типичных случаях определяется облаковидное понижение прозрачности легочных полей, получившее название «хлопьев снега» или «ватного легкого». Иногда рентгенологические изменения отсутствуют или имеют атипичную картину. С помощью ФВД обнаруживаются признаки дыхательной недостаточности по рестриктивному типу; исследование газового состава крови свидетельствует о гипоксемии.</p>\r\n<p align=\"justify\">Для подтверждения диагноза пневмоцистоза выполняется бронхоскопия с забором бронхиального секрета, трансбронхиальная биопсия легкого, сцинтиграфия легких с галлием-67. Для лабораторной детекции P.carinii микроскопически исследуются окрашенные мазки мокроты, бронхиальный и трахеальный аспират; выполняется гистологическое исследование биоптатов, исследование мокроты методом ПЦР. Проводится иммунологическая диагностика: определение титра противопневмоцистных IgG и IgM в сыворотке крови с помощью РИФ и ИФА.</p>",
"treatment": "<p>Лечение пневмоцистоза проводится в стационаре. Лица с иммунодефицитом и недоношенные дети должны быть помещены в отдельные стерильные палаты с ламинарным воздушным потоком. В большинстве случаев для проведения специфической фармакотерапии пневмоцистоза используется ко-тримоксазол (триметоприм, септрин, бактрим), пентамидин, триметрексат, триметоприм+дапсон, эфлорнитин, атоваквон в течение 2-3 недель. Для устранения побочных эффектов терапии назначают фолиевую кислоту, глюкокортикоиды. Проводится инфузионная терапия (введение гамма-глобулина, гемодеза, глюкозы, плазмы крови, альбумина и др.), кислородотерапия. У больных ВИЧ-инфекцией этиотропная терапия пневмоцистной пневмонии сочетается с высокоактивной антиретровирусной терапией.</p>",
"prevention": "",
"clinical_picture": "<p>Пневмоцистоз у детей развивается обычно на 4-6-м месяце жизни (недоношенные, больные рахитом, гипотрофией, поражениями ЦНС) и в более старших возрастных группах (при гемобластозе, злокачественных новообразованиях, СПИДе). Заболевание начинается постепенно - у ребенка снижается аппетит, прекращается нарастание массы тела, появляются бледность и <a title=\"Перейти на страницу симптома Цианоз\" href=\"../../../symptom/cianoz/\">цианоз</a> носогубного треугольника, легкое покашливание. Нормальная в начале заболевания температура сменяется субфебрильной с подъемами до фебрильной. В легких появляются непостоянные мелко- и среднепузырчатые <a title=\"Перейти на страницу симптома Хрипы\" href=\"../../../symptom/hripy/\">хрипы</a>. Появляются <a title=\"Перейти на страницу симптома Одышка\" href=\"../../../symptom/odyshka/\">одышка</a> (до 50-70 в 1 мин), <a title=\"Перейти на страницу симптома Цианоз\" href=\"../../../symptom/cianoz/\">цианоз</a>, <a title=\"Перейти на страницу симптома Кашель\" href=\"../../../symptom/kashel/\">кашель</a> коклюшеобразного характера. Нередко <a title=\"Перейти на страницу симптома Кашель\" href=\"../../../symptom/kashel/\">кашель</a> сопровождается выделением пенистой мокроты, в которой могут обнаруживаться пневмоцисты. Рентгенологически регистрируются очаговые тени разной величины и плотности, дающие картину \"облаковидного\" легкого. В крови обнаруживается лейкоцитоз, умеренная эозинофилия и увеличение СОЭ.</p>\r\n<full></full>\r\n<p>Иногда пневмоцистоз у детей протекает под маской острого ларингита, обструктивного бронхита или бронхиолита. В ряде случаев наступает летальный исход при клинической картине отека легких.</p>\r\n<p>У взрослых пневмоцистоз развивается у лиц, получающих иммуносупрессивную терапию (обычно - кортикостероиды), и у больных СПИДом. При медикаментозной иммуносупрессии заболевание часто манифестируется на фоне снижения дозы кортикостероидов. Продромальный период длится обычно 1-2 нед, а у больных СПИДом он достигает 10 нед. Постепенно появляется субфебрилитет, умеренная <a title=\"Перейти на страницу симптома Одышка\" href=\"../../../symptom/odyshka/\">одышка</a> при физической нагрузке, сухой <a title=\"Перейти на страницу симптома Кашель\" href=\"../../../symptom/kashel/\">кашель</a>, боли в грудной клетке. Через 1-2 нед могут появиться <a title=\"Перейти на страницу симптома Высокая температура 38-42° \" href=\"../../../symptom/vysokaya_temperatura_38-42/\">лихорадка</a>, <a title=\"Перейти на страницу симптома Одышка\" href=\"../../../symptom/odyshka/\">одышка</a> в покое, усиливается сухой <a title=\"Перейти на страницу симптома Кашель\" href=\"../../../symptom/kashel/\">кашель</a> (продуктивный <a title=\"Перейти на страницу симптома Кашель\" href=\"../../../symptom/kashel/\">кашель</a> отмечается редко). При осмотре обнаруживается тахипноэ, <a title=\"Перейти на страницу симптома Тахикардия\" href=\"../../../symptom/tahikardiya/\">тахикардия</a>, <a title=\"Перейти на страницу симптома Цианоз\" href=\"../../../symptom/cianoz/\">цианоз</a>. В легких часто выслушиваются сухие, реже - влажные <a title=\"Перейти на страницу симптома Хрипы\" href=\"../../../symptom/hripy/\">хрипы</a>. Количество лейкоцитов обычно зависит от фонового заболевания. При газовом анализе крови обнаруживают прогрессирующую гипоксемию, повышение альвеолярно-артериального кислородного градиента и респираторный алкалоз.</p>\r\n<p>Пневмоцистная пневмония при СПИДе обычно характеризуется вялым хроническим течением. Первоначально аускультативная симптоматика не выявляется, рентгенологическая картина тоже может оставаться без патологических изменений. По мере прогрессирования заболевания появляются двухсторонние прикорневые инфильтраты, трансформирующиеся затем либо в фокусные, либо интерстициальные изменения. Изредка обнаруживаются солитарные узелки, которые могут кавернизироваться с образованием обширной центральной полости. Причиной абсцедирования, вероятно, является присоединение бактериальных и микозных инфекций.</p>",
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