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"description": "Ядерная желтуха – патологическое состояние, которое может возникнуть у новорожденных в результате билирубиновой интоксикации. Билирубин появляется в базальных ганглиях и ядрах ствола мозга, что приводит к нарушениям мозговых функций. ",
"etiology": "<p>Причинами ядерной желтухи могут стать следующие факторы: </p>\r\n<ul>\r\n<li>травмы головного мозга новорожденного; </li>\r\n<li>рождение раньше срока; </li>\r\n<li>недостаток ферментов, связывающих и выводящих билирубин; </li>\r\n<li>критическое нарушение обменных процессов у новорожденного; </li>\r\n<li>подкожные кровоизлияния у младенца; </li>\r\n<li>несовместимость резус-факторов матери и ребенка; </li>\r\n<li>серьезные патологии эндокринной системы матери; </li>\r\n<li>последствия приема сильнодействующих препаратов в период беременности; </li>\r\n<li>внутриутробные заболевания инфекционного характера; </li>\r\n<li>заболевания крови (матери и ребенка). </li>\r\n</ul>",
"pathogenesis": "<p>Заболевание развивается в связи с неспособностью организма новорожденного справиться с высокой концентрацией билирубина в крови. В норме реакции распада преобладают в первые дни жизни ребенка. Именно с этим связана физиологическая желтуха новорожденных, но в норме новорожденный справляется, и симптомы исчезают в течение нескольких дней или недель. Ядерная желтуха новорожденных характеризуется чрезмерной гипербилирубинемией, вследствие чего желчный пигмент начинает проходить через гематоэнцефалический барьер, окрашивая базальные ядра, ядра мозжечка и черепно-мозговых нервов в желтушный цвет. </p>\r\n<p>Для развития ядерной желтухи новорожденных необходимо множество условий. В частности, гематоэнцефалический барьер пропускает только свободный билирубин, то есть тот, который не связан с альбуминами и не подвергнутый конъюгации в печени. Значит, одним из факторов, способствующих развитию данного состояния, является гипопротеинемия (снижение белков-переносчиков). По этой причине симптомы чаще наблюдаются у детей с отеками, обширной кефалогематомой и т. д. Гипоксическое, инфекционное или травматическое повреждение мозга, имевшее место внутриутробно или в родах, значительно повышает проницаемость гематоэнцефалического барьера. То же касается недоношенности, когда мозговые структуры к моменту рождения остаются незрелыми. </p>\r\n<p>Гемолитическая болезнь новорожденных всегда является фактором повышенного риска ядерной желтухи новорожденных, поскольку в этом случае концентрация несвязанного билирубина быстрее всего достигает предельно допустимых значений и начинает превышать их, способствуя проникновению билирубина в вещество мозга. В таких случаях состояние развивается стремительно, часто с летальным исходом или глубокой инвалидизацией. </p>",
"diagnostics": "<p>Подозрение на ядерную желтуху возникает у врача еще на этапе визуального обследования новорожденного. Для подтверждения диагноза малышу назначаются лабораторные исследования и комплексное обследование внутренних систем организма. </p>\r\n<p>В некоторых случаях возникает необходимость в дополнительной консультации с профильными специалистами. </p>\r\n<p>Диагностика ядерной желтухи включает в себя следующие процедуры: </p>\r\n<ul>\r\n<li>определение уровня билирубина - при ядерной желтухе более 256 мкмоль/л у доношенных и более 171 мкмоль/л у недоношенных детей. </li>\r\n<li>пробы Кумбса - определение наличия антител к эритроцитам; </li>\r\n<li>биохимический анализ крови и мочи; </li>\r\n<li>общий анализ крови и мочи; </li>\r\n<li>УЗИ печени и селезенки; </li>\r\n<li>УЗИ головного мозга. </li>\r\n</ul>",
"treatment": "<p>Одним из этапов лечения ядерной желтухи новорожденных является фототерапия. Доказано, что излучение видимого спектра, особенно голубого цвета, способствует переходу токсичного непрямого билирубина в его изомер, который может выводиться печенью и почками без связывания с альбуминами крови. </p>\r\n<p>Если на фоне фототерапии уровень билирубина не снижается, показано переливание плазмы крови. Перелитые вместе с плазмой белки способствуют связыванию избыточного билирубина с последующим выведением его из организма. </p>\r\n<p>При неэффективности перечисленных методов лечения или стремительном прогрессировании симптоматики проводится обменно-заменное переливание крови. </p>\r\n<p>Применяются медикаменты, которые способствуют более быстрому выведению билирубина из тела младенца (препараты с желчегонным действием), энтеросорбенты. </p>\r\n<p>По показаниям проводится противосудорожная терапия.</p>",
"prevention": "<p>Профилактика состояния возможна в первые дни жизни. Профилактическая фототерапия проводится всем новорожденным с уровнем билирубина в крови выше 200 мкмоль/л, а также всем недоношенным новорожденным, детям, перенесшим внутриутробные инфекции, гипоксию плода, родовые травмы и др.</p>",
"clinical_picture": "<p>Гипербилирубинемия в первые дни и недели жизни заметна по желтой окраске кожных покровов и склер. В данном случае этот признак выражен в значительной степени. Кожа малыша имеет ярко-желтый оттенок уже в первый день жизни, в отличие от физиологической желтухи, которая развивается спустя 5-7 дней после рождения. Состояние чаще возникает остро в течение первой недели жизни. Выделяют две стадии: асфиксическую и спастическую. Первая стадия ядерной желтухи новорожденных характеризуется появлением приступов апноэ (ребенок не дышит) разной продолжительности. Мышечный тонус снижен, отмечаются отдельные подергивания. Ребенок вялый, плохо сосет. </p>\r\n<p>Спастическая стадия ядерной желтухи новорожденных представлена выраженным гипертонусом мышц разгибателей вплоть до опистотонуса. Голова ребенка непроизвольно резко запрокидывается назад, глаза закатываются вверх (симптом «заходящего солнца»). Дыхание нарушено, отмечаются частые приступы апноэ. Могут наблюдаться тонические судороги и пронзительный крик, связанный с напряжением мышц и отчасти с болевым синдромом, сопровождающим гипертонус. В тяжелых случаях ядерной желтухи новорожденных мышечное напряжение распространяется на конечности с их резким выпрямлением и пронацией сжатых кулачков. </p>\r\n<p>В течение нескольких недель состояние ребенка улучшается. Симптомы ослабевают, судороги и гипертонус отмечаются реже. Однако к концу первого года жизни в 80% случаев развивается мышечная ригидность, судороги вновь учащаются. Прогрессируют симптомы поражения черепно-мозговых нервов, что клинически проявляется снижением зрения и слуха. Часто развивается косоглазие и тугоухость. Массивное поражение нейронов неизбежно отражается на умственном развитии. У детей, перенесших ядерную желтуху новорожденных, впоследствии нередко диагностируется синдром дефицита внимания с гиперактивностью (СДВГ), а также умственная отсталость. </p>",
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