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"lead": "группа наследственных заболеваний кроветворной системы, которые характеризуются нарушением синтеза гемоглобина",
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"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;\">Талассемия – группа генетически детерминированных болезней крови, развивающихся при нарушении синтеза a- или β-цепей гемоглобина, сопровождающихся гемолизом, гипохромной анемией, микроцитозом. В гематологии талассемия относится к наследственным гемолитическим анемиям - количественным гемоглобинопатиям.</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;\">Талассемия широко распространена среди населения Средиземноморского и Черноморского региона; название заболевания буквально переводится как «анемия морского побережья». Также случаи талассемии нередки в странах Африки, Ближнего Востока, Индии и Индонезии, Средней Азии и Закавказья. С синдромом талассемии каждый год в мире рождается 300 тыс. детей. В зависимости от формы патологии течение талассемии может быть тяжелым, фатальным или легким, бессимптомным. Так же, как серповидно-клеточная анемия, талассемия играет роль защитного фактора против малярии.</span></p>",
"pathogenesis": "<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;\">Талассемия является генетическим заболеванием с аутосомно-рецессивным наследованием. Непосредственной причиной патологии выступают различные мутационные нарушения в гене, кодирующем синтез той или иной цепи гемоглобина. Молекулярную основу дефекта могут составлять синтез аномальной матричной РНК, делеции структурных генов, мутации регуляторных генов либо их неэффективная транскрипция. Следствием подобных нарушений служит снижение или отсутствие синтеза одной из полипептидных гемоглобиновых цепей.</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;\">Так, при b-талассемии бета-цепи синтезируются в недостаточном количестве, что приводит к избытку альфа-цепей, и наоборот. Избыточно продуцируемые полипептидные цепи откладываются в клетках эритроидного ряда, вызывая их повреждение. Это сопровождается деструкцией эритрокариоцитов в костном мозге, гемолизом эритроцитов в периферической крови, гибелью ретикулоцитов в селезенке. Кроме этого, при b-талассемии в эритроцитах накапливается фетальный гемоглобин (НbF), не способный транспортировать кислород в ткани, что вызывает развитие тканевой гипоксии. Вследствие костномозговой гиперплазии развивается деформация костей скелета. Анемия, тканевая гипоксия и неэффективный эритропоэз в той или иной степени нарушают развитие и рост ребенка.</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;\">Для гомозиготной формы талассемии характерно наличие двух дефектных генов, унаследованных от обоих родителей. При гетерозиготном варианте талассемии пациент является носителем мутантного гена, унаследованного от одного из родителей.</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;\">Талассемию следует заподозрить у лиц с семейным анамнезом, характерными клиническими признаками и лабораторными показателями. Больные талассемией нуждаются в консультации гематолога и медицинского генетика.</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;\">Типичными гематологическими изменениями служат снижение уровня гемоглобина и цветового показателя, гипохромия, наличие мишеневидных эритроцитов, повышение уровня железа сыворотки крови и непрямого билирубина. Электрофорез Hb на ацетат-целлюлозной пленке используется для определения различных гемоглобиновых фракций. При изучении пунктата костного мозга обращает внимание гиперплазия красного кроветворного ростка с высоким числом эритробластов и нормобластов. Молекулярно-генетические исследования позволяют выявить мутацию в локусе a- или β-глобина, нарушающую синтез полипептидной цепи.</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;\">На краниограммах при большой b-талассемии выявляется игольчатый периостоз (феномен «волосатого черепа»). Характерна поперечная исчерченность трубчатых и плоских костей, наличие мелких очагов остеопороза. С помощью УЗИ брюшной полости обнаруживается гепатоспленомегалия, камни желчного пузыря.</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 подозрении на талассемию требуется исключить железодефицитную анемию, наследственный микросфероцитоз, серповидно-клеточную анемию, аутоиммунную гемолитическую анемию. В семьях, имеющих больных талассемией, рекомендуется проведение генетического консультирования супругов и инвазивной дородовой диагностики (биопсии хориона, кордоцентеза, амниоцентеза) для выявления гемоглобинопатии на ранних сроках беременности. Подтверждение гомозиготных форм талассемии у плода служит показанием для искусственного прерывания беременности.</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;\">При умеренно выраженных симптомах лечение не назначают. Время от времени проводят только переливание крови. В основном это нужно после операций, родов или для предотвращения возможных осложнений. Люди с бета-талассемией требуют более частых переливаний крови. Для нормализации избыточного уровня железа им также назначают специфические препараты, которые связывают и выводят железо.</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;\">При выраженной и тяжелой формах болезни существует два способа лечения:</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: 8pt; font-family: Verdana; color: #292b2c; 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;\">частые переливания крови (раз в несколько недель), которые сочетают с приемом препаратов, выводящих лишнее железо из организма;</span></p>\r\n</li>\r\n<li style=\"list-style-type: disc; font-size: 8pt; font-family: Verdana; color: #292b2c; 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;\">пересадка костного мозга – единственный метод, который помогает полностью излечить человека от талассемии. К сожалению, далеко не всегда трансплантация бывает успешной.</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;\">Лекарственные препараты при талассемии назначают только для коррекции симптомов и осложнений. Медикаментозной терапии самого заболевания не существует.</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;\">Талассемия – заболевание наследственное. Поэтому, если один или оба родителя больны, необходимо проконсультироваться с врачом-генетиком на этапе планирования беременности.</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;\">Симптомы талассемии зависят от вида генной мутации.</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;\">Большая талассемия (болезнь Кули) развивается при гомозиготной передаче от родителей. Ее проявления заметны у детей сразу после рождения.</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;\">У новорожденного замечают:</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: 8pt; font-family: Verdana; color: #333333; 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;\">удлиненный вверх череп («башенный»);</span></p>\r\n</li>\r\n<li style=\"list-style-type: disc; font-size: 8pt; font-family: Verdana; color: #333333; 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;\">более развитую верхнюю челюсть;</span></p>\r\n</li>\r\n<li style=\"list-style-type: disc; font-size: 8pt; font-family: Verdana; color: #333333; 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;\">монголоидный тип лицевого скелета.</span></p>\r\n</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\"><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;\">К 12-месячному возрасту проявляется:</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: 8pt; font-family: Verdana; color: #333333; 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;\">расширенная носовая перегородка, «приплюснутый» нос;</span></p>\r\n</li>\r\n<li style=\"list-style-type: disc; font-size: 8pt; font-family: Verdana; color: #333333; 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;\">костные наросты на стопах;</span></p>\r\n</li>\r\n<li style=\"list-style-type: disc; font-size: 8pt; font-family: Verdana; color: #333333; 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;\">нарушенный прикус;</span></p>\r\n</li>\r\n<li style=\"list-style-type: disc; font-size: 8pt; font-family: Verdana; color: #333333; 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;\">желтушность кожи (в связи с поражением селезенки).</span></p>\r\n</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\"><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;\">Кислородное голодание тканей приводит к органным нарушениям. При осмотре отмечаются:</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: 8pt; font-family: Verdana; color: #333333; 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;\">увеличение печени, край плотный на ощупь (развивается ранний цирроз);</span></p>\r\n</li>\r\n<li style=\"list-style-type: disc; font-size: 8pt; font-family: Verdana; color: #333333; 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;\">возникновение сердечной недостаточности (лишнее железо откладывается в миокарде и поражает сократимость сердечной мышцы);</span></p>\r\n</li>\r\n<li style=\"list-style-type: disc; font-size: 8pt; font-family: Verdana; color: #333333; 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;\">отставание в физическом и умственном развитии от сверстников.</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;\">У малыша развивается сахарный диабет из-за фиброзирования поджелудочной железы и печеночная недостаточность. В тяжелых случаях он живет не более одного года.</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 старшем возрасте проявляются такие осложнения:</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: 8pt; font-family: Verdana; color: #333333; 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;\">трофические язвы на коже, вызванные локальными нарушениями кровообращения;</span></p>\r\n</li>\r\n<li style=\"list-style-type: disc; font-size: 8pt; font-family: Verdana; color: #333333; 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;\">частые переломы костей;</span></p>\r\n</li>\r\n<li style=\"list-style-type: disc; font-size: 8pt; font-family: Verdana; color: #333333; 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;\">повторные воспаления в легких;</span></p>\r\n</li>\r\n<li style=\"list-style-type: disc; font-size: 8pt; font-family: Verdana; color: #333333; 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;\">калькулезный холецистит;</span></p>\r\n</li>\r\n<li style=\"list-style-type: disc; font-size: 8pt; font-family: Verdana; color: #333333; 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;\">кардиосклероз;</span></p>\r\n</li>\r\n<li style=\"list-style-type: disc; font-size: 8pt; font-family: Verdana; color: #333333; 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;\">сепсис при контакте с инфекцией;</span></p>\r\n</li>\r\n<li style=\"list-style-type: disc; font-size: 8pt; font-family: Verdana; color: #333333; 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;\">нарушенное полового развитие.</span></p>\r\n</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\"><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;\">Малая или гетерозиготная форма передается одним из родителей. Второй «здоровый» ген сглаживает повреждения. Симптомы талассемии могут длительно не проявляться или вызывать признаки, общие с другими заболеваниями:</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: 8pt; font-family: Verdana; color: #333333; 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;\">слабость, повышенную утомляемость;</span></p>\r\n</li>\r\n<li style=\"list-style-type: disc; font-size: 8pt; font-family: Verdana; color: #333333; 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;\">головные боли;</span></p>\r\n</li>\r\n<li style=\"list-style-type: disc; font-size: 8pt; font-family: Verdana; color: #333333; 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;\">головокружение.</span></p>\r\n</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\"><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 осмотре обращается внимание на следующее:</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: 8pt; font-family: Verdana; color: #333333; 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;\">бледность кожи с желтушным оттенком;</span></p>\r\n</li>\r\n<li style=\"list-style-type: disc; font-size: 8pt; font-family: Verdana; color: #333333; 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;\">возможно увеличение печени и селезенки.</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;\">У ребенка с малой формой резко увеличена восприимчивость к инфекциям, снижен иммунитет. Очень тяжело и часто протекают вирусные и кишечные инфекционные заболевания.</span></p>",
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"lead": "Хемоз — отек конъюнктивы, при котором поверхность глаза в области склер заметно увеличивается, может менять цвет и болеть. Такая патология возникает на фоне множества заболеваний глаза, установить точную причину может только врач, поэтому и самолечение в этом случае недопустимо. Особенно опасны ситуации, при которых хемоз возникает часто и склонен к длительному течению. \r\nПричины отека конъюнктивы могут быть острыми и локальными. Среди наиболее частых причин наблюдается: острое воспаление придаточного глазного аппарата и оболочек глаза, укусы насекомых, появление ячменя на глазу, орбитальный целлюлит (воспаление тканей глаза позади орбитальной перегородки), гонорейный конъюнктивит (опасная для зрения патология, которая развивается при гонореи), нарушение микроциркуляции или застой крови, которые могут быть вызваны воздействием или давлением на глаз опухолей орбитальных областей (зона вокруг глазного яблока). Часто при отеке конъюнктивы диагностируется анемия (малокровие), нефрит (воспаление, которое приводит к изменению парных органов), крапивница (кожное заболевание, преимущественно аллергического происхождения).\r\n\r\nПричинами заболевания может быть патологии глаз, черепно-мозговые травмы, бесконтрольное применение медицинских препаратов.",
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"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;\">Гемоглобинопатии относятся к аутосомно-рецессивным генетическим заболеваниям. Качественные гемоглобинопатии развиваются вследствие мутаций генов, ответственных за синтез определенных аминокислот в бета-цепи глобина. В результате происходит замена одной аминокислоты на другую (глутаминовой кислоты на валин, лизин и пр.). Это приводит к образованию аномального гемоглобина, гораздо менее растворимого, чем нормальный гемоглобин А, придающего красным кровяным тельцам иную форму (мишеневидную, серповидную), что нарушает их функции и уменьшает продолжительность жизни.</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;\">Количественные гемоглобинопатии обусловлены мутацией генов, которые кодируют целую цепь глобина (чаще альфа и бета). При этом сдвигается баланс между глобиновыми цепями – при недостаточном синтезе альфа-цепей возникает избыток бета-цепей, и наоборот. Уменьшаются размеры эритроцитов, в них снижается содержание гемоглобина, а мембрана становится более подверженной различным повреждениям.</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;\">Существуют факторы, провоцирующие тяжелые приступы (кризы). К ним относятся обезвоживание, переохлаждения, инфекции, сопровождающиеся высокой лихорадкой. У женщин обострения нередко развиваются на фоне беременности. Но главный патологический стимул качественных гемоглобинопатий – уменьшение концентрации в крови кислорода (гипоксия). Это может произойти, например, при подъеме на большую высоту (восхождение на гору, полет на самолете), где снижено парциальное давление кислорода воздуха, или при тяжелых болезнях дыхательной системы (пневмонии).</span></p>",
"pathogenesis": "<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;\">Гемоглобинопатии имеют сходные патогенетические механизмы. Модифицированная структура гемоглобина предрасполагает к интенсивному гемолизу. Текущая длительная анемия способствует компенсаторной гиперплазии костного мозга. Появляется деформация костей черепа, искривление позвоночника. Развиваются экстрамедуллярные очаги кроветворения, что приводит к увеличению размеров печени и селезенки (гепатоспленомегалия). Из-за спленомегалии возникает гиперспленизм: повышенное разрушение эритроцитов синусоидами селезенки.</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;\">И</span><span style=\"font-size: 7pt; font-family: Verdana; background-color: transparent; font-style: normal; font-variant-numeric: normal; font-variant-east-asian: normal; vertical-align: baseline; white-space: pre-wrap;\">з-за регулярного гемолиза эритроцитов печень выделяет большое количество билирубина в желчь, создавая условия для образования камней желчного пузыря. У пациентов с гемоглобинопатиями часто возникает перегрузка железом как в результате постоянных переливаний крови, так и из-за повышенной абсорбции железа желудочно-кишечным трактом. Большое количество железа в тканях улучшает процессы перекисного окисления липидов, которые повреждают различные органы.</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;\">При высококачественных гемоглобинопатиях под влиянием пониженного содержания кислорода в крови аномальные нерастворимые молекулы гемоглобина растягивают мембрану эритроцита, что приводит к изменению его формы. Деформированные эритроциты переносят худший кислород и также могут прилипать к эндотелию сосудов, блокируя таким образом мелкие сосуды, вызывая тромбоз, окклюзию и сердечные приступы.</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 общем осмотре уделяется внимание цвету кожи (бледность, желтуха), нарушению конституции (задержка нервно-психического развития, физическое развитие ребенка, аномалии в строении скелета). Дополнительные тесты включают в себя: </span></p>\r\n<ul>\r\n<li><span style=\"font-size: 7pt; background-color: transparent; font-style: normal; font-variant-numeric: normal; font-variant-east-asian: normal; vertical-align: baseline; white-space: pre-wrap;\">Для общего анализа крови пациентов с высококачественными гемоглобинопатиями характерна типичная умеренная анемия, а для пациентов с количественными гемоглобинопатиями характерна тяжелая микроцитарная анемия. Мазок крови содержит измененные деформированные эритроциты (серповидные, сферические). При биохимическом анализе крови уровни свободного железа, ферритина и непрямого билирубина повышаются.</span></li>\r\n<li><span style=\"font-size: 7pt; font-family: Verdana; background-color: transparent; font-style: normal; font-variant-numeric: normal; font-variant-east-asian: normal; vertical-align: baseline; white-space: pre-wrap;\">Электрофорез гемоглобина. Основной метод диагностики гемоглобиновой патологии, широко используемый в клинической гематологии, определяет соотношение фракции гемоглобина - мембранный электрофорез с ацетатом целлюлозы или агара лимонной кислоты. </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;\">Генетические исследования. ПЦР-тестирование ДНК проводится только в рамках пренатальной диагностики с целью генетического консультирования семей с повышенным риском развития патологий гемоглобина. Образцы ДНК получают амниоцентезом на 8-10 и 14-16 неделях беременности. Наличие мутаций различных генов определяется.</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;\">Инструментальные исследования. На рентгенограмме костей конечностей имеются признаки роста костного мозга - истончение кортикального слоя, зоны остеопороза, расширение канала костного мозга. На рентгенограмме костей черепа выявляется игольчатый периостоз («феномен волосатого черепа»). УЗИ брюшной полости выявляет гепатоспленомегалию, камни в желчном пузыре.</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>",
"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;\">Подбор консервативной терапии производится с учетом типа гемоглобинопатии, течения заболевания, наличия определенных осложнений. Клинические симптомы и лабораторные данные (в основном, анализ крови) оцениваются. Различают следующие области лечения:</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;\">Остановка кризисов. В случае вазоокклюзионных кризов используются анальгетики (нестероидные противовоспалительные препараты) и синдром сильной боли - наркотические анальгетики. Кроме того, для подавления деформации деформированных эритроцитов назначают ингаляции кислорода, оральную или внутривенную регидратацию.</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;\">Предотвращение кризисов. Для постоянного приема пациентов с качественными гемоглобинопатиями назначают гидроксимочевину. Это лекарство стимулирует образование внутриутробного гемоглобина, который подавляет экспрессию гена, ответственного за синтез аномальных нерастворимых гемоглобинов, что снижает склонность к деформации эритроцитов и снижает частоту кризов.</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;\">Терапия осложнений. Инфекционные осложнения лечат антибактериальными препаратами, которые эффективны против пневмококков, гемофильной палочки и менингококков до тех пор, пока не станут доступны результаты бактериологических исследований. Используются антибиотики из группы пенициллина (амоксициллин). Антикоагулянты (низкомолекулярный, нефракционированный гепарин) используются при развитии тромбоза. Поскольку у пациентов с количественной патологией гемоглобина всегда тяжелая анемия, основой лечения являются регулярные переливания крови. У людей с качественной гемоглобинопатией переливание крови проводится только во время секвестрации, гемолитического и апластического кризов.</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;\">Борьба с перегрузкой железом и дефицитом фолиевой кислоты. Хелатирующие агенты (дефероксамин) используются для удаления избытка железа из организма. Этот препарат обычно назначают с аскорбиновой кислотой, поскольку он усиливает хелатирующий эффект дефероксамина. Из-за постоянного гемолиза у пациентов увеличивается потребление фолата. Таким образом показано длительное применение больших доз фолиевой кислоты.</span></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;\">Эффективным методом лечения нескольких пациентов с тяжелым гемолизом является спленэктомия - хирургическое удаление селезенки. Другим хирургическим лечением для достижения полной ремиссии является аллотрансплантат гемопоэтических стволовых клеток. Однако этот метод используется редко, только в очень тяжелых случаях, так как он связан с высокой частотой смертей. При желчнокаменной болезни проводится холецистэктомия.</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;\">Первичная профилактика проводится среди семей с высоким риском развития гемоглобинопатий. Он состоит из пренатальной диагностики с прерыванием беременности по медицинским показаниям. Пациенты, страдающие от качественных нарушений гемоглобина, обязательно должны получить вакцину против гемофильной палочки, пневмококка, менингококка.</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 гемоглобинопатии разнообразна. Гетерозиготные пациенты протекают бессимптомно или слабо. Гомозиготные формы начинают появляться с раннего детства (6 месяцев - 1 год). Общие симптомы включают в себя симптомы гемолитической анемии (бледность, пожелтение кожи и слизистых оболочек, увеличение селезенки), патология скелета - черепная башня (четырехугольная), уплощенная переносица, изогнутый позвоночник.</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;\">Из-за повышенной секреции билирубина в желчи симптомы желчнокаменной болезни могут вызывать беспокойство уже в детстве - обострение или боль в правом подреберье, приступы желчной колики, обесцвечивание кала. Длительные незаживающие язвы часто появляются на коже ног. Для заболеваний с дефектом в структуре гемоглобина характерен криз. Наиболее серьезные судороги, часто со смертельным исходом, возникают при серповидно-клеточной анемии.</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;\">Ассоциированные симптомы: Билирубинурия. Боль в правом подреберье. Гемоглобинурия. Потливость. Протеинурия. Ретикулоцитоз. Тяжесть в подреберье. Цилиндрурия. Эритропения.</span></p>",
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3,
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}
]
}
