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"etiology": "<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;\">Основные причины синдрома Иценко-Кушинга — повышенная выработка гормонов корой надпочечников. Чаще всего причиной такой аномалии становится опухоль, которая продуцирует избыточное количество АКТГ. Опухоль может образоваться в различных органах, чаще в бронхолегочной системе, реже — в мочеполовой. Повышенный синтез АКТГ влияет на функции надпочечниковых желез, которые начинают в усиленных количествах выделять глюкокортикоиды. Кроме этого, опухоль может образоваться в коре надпочечников, и в этом случае также будет наблюдаться повышенный синтез глюкокортикоидов. К повышению содержания в организме глюкокортикоидных гормонов могут привести ожирение, беременность, злоупотребление алкоголем в течение длительного времени, ряд психических заболеваний. Состояние, возникающее в результате воздействия перечисленных факторов, называют псевдо синдромом Кушинга.</span></p>\r\n<p><span><span> </span></span></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;\">Еще одной причиной развития является продолжительная терапия аутоиммунных заболеваний с применением больших дозировок препаратов, содержащих глюкокортикоидные гормоны.</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>",
"pathogenesis": "<p style=\"line-height: 1.38; margin-top: 0pt; margin-bottom: 0pt;\" dir=\"ltr\"><span style=\"font-size: 7.5pt; font-family: Verdana; font-variant-numeric: normal; font-variant-east-asian: normal; vertical-align: baseline; white-space: pre-wrap;\">Гиперсекреция кортизола при синдроме Иценко-Кушинга вызывает катаболический эффект - распад белковых структур костей, мышц (в том числе и сердечной), кожи, внутренних органов и т. д., со временем приводя к дистрофии и атрофии тканей. Усиление глюкогенеза и всасывания в кишечнике глюкозы вызывает развитие стероидной формы диабета. Нарушения жирового обмена при синдроме Иценко-Кушинга характеризуется избыточным отложением жира на одних участках тела и атрофией на других ввиду их разной чувствительности к глюкокортикоидам. Влияние избыточного уровня кортизола на почки проявляется электролитными расстройствами – гипокалиемией и гипернатриемией и, как следствие, повышением артериального давления и усугублением дистрофических процессов в мышечной ткани. В наибольшей степени от гиперкортицизма страдает сердечная мышца, что проявляется в развитии кардиомиопатии, сердечной недостаточности и аритмий. Кортизол оказывает угнетающее действие на иммунитет, вызывая у пациентов с синдромом Иценко-Кушинга склонность к инфекциям.</span></p>\r\n<p><span style=\"font-size: 7.5pt; font-family: Verdana; 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: 7pt; font-family: Verdana; background-color: transparent; font-variant-numeric: normal; font-variant-east-asian: normal; vertical-align: baseline; white-space: pre-wrap;\">Диагностика синдрома Иценко-Кушинга включает:</span></p>\r\n<ul>\r\n<li><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;\">Анализ мочи на определение суточного выделения свободного кортизола.</span></li>\r\n<li><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;\">Дексаметазоновые пробы: малая и большая. Малая проба заключается в поэтапном определении показателя кортизола в крови до приема глюкокортикостероида и после приема. В качестве препарата используется дексаметазон – гормональное средство с антигистаминным и противовоспалительным эффектом. После приема дексаметазона концентрация кортизола в норме снижается в два раза и более. При заболевании уровень кортизола повышается либо остается неизменным. Большая дексаметазоновая проба проводится аналогично, разница только в дозе медикаментов. Цель – дифференциальная диагностика синдрома Иценко-Кушинга и определение его конкретного вида.</span></li>\r\n<li><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;\">МР-томография головного мозга для исключения гипофизарной аденомы.</span></li>\r\n<li><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;\">Компьютерное или магнитно-резонансное обследование надпочечников.</span></li>\r\n<li><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;\">Рентгенологические методы диагностики позволяют выявить осложнения болезни, компрессионные переломы позвоночного столба.</span></li>\r\n<li><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;\">Анализ крови общий и биохимия крови для выявления сахарного диабета стероидного генеза и электролитных нарушений.</span></li>\r\n</ul>\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>",
"treatment": "<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;\">При ятрогенной (лекарственной) природе синдрома Иценко-Кушинга необходима постепенная отмена глюкокортикоидов и замена их на другие иммунодепрессанты. При эндогенной природе гиперкортицизма назначаются препараты, подавляющие стероидогенез (аминоглютетимид, митотан).</span></p>\r\n<p><span><span> </span></span></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;\">При наличии опухолевого поражения надпочечников, гипофиза, легких проводится хирургическое удаление новообразований, а при невозможности – одно- или двусторонняя адреналэктомия (удаление надпочечника) или лучевая терапия гипоталамо-гипофизарной области. Лучевую терапию часто проводят в комбинации с хирургическим или медикаментозным лечением для усиления и закрепления эффекта.</span></p>\r\n<p><span><span> </span></span></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;\">Симптоматическое лечение при синдроме Иценко-Кушинга включает применение гипотензивных, мочегонных, сахароснижающих препаратов, сердечных гликозидов, биостимуляторов и иммуномодуляторов, антидепрессантов или седативных средств, витаминотерапию, лекарственную терапию остеопороза. Проводится компенсация белкового, минерального и углеводного обмена. Послеоперационное лечение пациентов с хронической надпочечниковой недостаточностью, перенесших адреналэктомию, состоит в постоянной заместительной гормональной терапии.</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>",
"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 профилактики синдрома Иценко-Кушинга для недопущения необратимых последствий, необходимо регулярно проходить скрининговые тесты и наблюдаться у специалистов, не работать на вредном производстве, особенно в ночные смены, избегать физических нагрузок. </span></p>",
"clinical_picture": "<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;\">Симптомы синдрома Иценко-Кушинга следующие:</span></p>\r\n<ul style=\"margin-top: 0; margin-bottom: 0; padding-inline-start: 48px;\">\r\n<li><span id=\"docs-internal-guid-a6472c3c-7fff-df19-005a-9ad9901b77fb\"><span style=\"background-color: transparent; font-size: 7pt; white-space: pre-wrap; font-family: Verdana;\">Ожирение — отложение жировой ткани происходит в верхней части тела, конечности при этом остаются худыми.</span></span></li>\r\n<li><span id=\"docs-internal-guid-a6472c3c-7fff-df19-005a-9ad9901b77fb\"><span style=\"background-color: transparent; font-size: 7pt; white-space: pre-wrap; font-family: Verdana;\">Появление на коже багрово-розовых полос вследствие распада коллагена и перерастяжения ее жиром.</span></span></li>\r\n<li><span id=\"docs-internal-guid-a6472c3c-7fff-df19-005a-9ad9901b77fb\"><span style=\"background-color: transparent; font-size: 7pt; white-space: pre-wrap; font-family: Verdana;\">Нарушение цикла у женщин, развитие бесплодия.</span></span></li>\r\n<li><span id=\"docs-internal-guid-a6472c3c-7fff-df19-005a-9ad9901b77fb\"><span style=\"background-color: transparent; font-size: 7pt; white-space: pre-wrap; font-family: Verdana;\">Образование на локтях и в кожных складках пигментных пятен, акне, появление избыточного количества волос на теле.</span></span></li>\r\n<li><span id=\"docs-internal-guid-a6472c3c-7fff-df19-005a-9ad9901b77fb\"><span style=\"background-color: transparent; font-size: 7pt; white-space: pre-wrap; font-family: Verdana;\">Снижение либидо и эректильная дисфункция у мужчин.</span></span></li>\r\n<li><span id=\"docs-internal-guid-a6472c3c-7fff-df19-005a-9ad9901b77fb\"><span style=\"background-color: transparent; font-size: 7pt; white-space: pre-wrap; font-family: Verdana;\">Прогрессирующий остеопороз и, как следствие, переломы костей.</span></span></li>\r\n<li><span id=\"docs-internal-guid-a6472c3c-7fff-df19-005a-9ad9901b77fb\"><span style=\"background-color: transparent; font-size: 7pt; white-space: pre-wrap; font-family: Verdana;\">Снижение иммунной защиты организма, что вызывает развитие инфекционных заболеваний кожного покрова и ряда других органов.</span></span></li>\r\n<li><span id=\"docs-internal-guid-a6472c3c-7fff-df19-005a-9ad9901b77fb\"><span style=\"background-color: transparent; font-size: 7pt; white-space: pre-wrap; font-family: Verdana;\">Сердечная аритмия, артериальная гипертензия.</span></span></li>\r\n<li><span id=\"docs-internal-guid-a6472c3c-7fff-df19-005a-9ad9901b77fb\"><span style=\"background-color: transparent; font-size: 7pt; white-space: pre-wrap; font-family: Verdana;\">Нарушение углеводного обмена и развитие стероидного диабета.</span></span></li>\r\n<li><span id=\"docs-internal-guid-a6472c3c-7fff-df19-005a-9ad9901b77fb\"><span style=\"background-color: transparent; font-size: 7pt; white-space: pre-wrap; font-family: Verdana;\">Слабость мышц (в том числе и миокарда), возникающая в результате распада белков.</span></span></li>\r\n</ul>\r\n<p><span> </span></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;\">При заболевании нарушается глюкогенез. Избыточное количество глюкозы всасывается кишечником, что приводит к развитию стероидного диабета. Нарушение липидного обмена проявляется в виде жировых отложений в одних зонах тела и атрофии других участков из-за разницы в чувствительности органов и тканей к глюкокортикоидам. Нарушение электролитного обмена проявляется гипокалиемией (пониженное содержание калия в крови) и гипернатриемией (повышенная концентрация натрия в крови). При этом может развиваться гипертоническая болезнь, непроходимость кишечника, мочекаменная болезнь, дыхательные расстройства, слабость.</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;\">Наиболее серьезное осложнение синдрома Иценко-Кушинга — это нарушение работы миокарда. При дисфункции сердечной мышцы развивается аритмия, декомпенсированная сердечная недостаточность, кардиомиопатия. Также гиперкортизолизм угнетает иммунитет, поэтому больные с синдромом Иценко-Кушинга склонны к инфекционным заболеваниям, фурункулезу, грибковым поражениям кожи. </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 тяжелых случаях развивается:</span></p>\r\n<ul style=\"margin-top: 0; margin-bottom: 0; padding-inline-start: 48px;\">\r\n<li><span id=\"docs-internal-guid-a6472c3c-7fff-df19-005a-9ad9901b77fb\"><span style=\"background-color: transparent; font-size: 7pt; white-space: pre-wrap; font-family: Verdana;\">Инсульт.</span></span></li>\r\n<li><span id=\"docs-internal-guid-a6472c3c-7fff-df19-005a-9ad9901b77fb\"><span style=\"background-color: transparent; font-size: 7pt; white-space: pre-wrap; font-family: Verdana;\">Параличи и парезы.</span></span></li>\r\n<li><span id=\"docs-internal-guid-a6472c3c-7fff-df19-005a-9ad9901b77fb\"><span style=\"background-color: transparent; font-size: 7pt; white-space: pre-wrap; font-family: Verdana;\">Пиелонефрит.</span></span></li>\r\n<li><span id=\"docs-internal-guid-a6472c3c-7fff-df19-005a-9ad9901b77fb\"><span style=\"background-color: transparent; font-size: 7pt; white-space: pre-wrap; font-family: Verdana;\">Декомпенсированная почечная недостаточность.</span></span></li>\r\n<li><span id=\"docs-internal-guid-a6472c3c-7fff-df19-005a-9ad9901b77fb\"><span style=\"background-color: transparent; font-family: Verdana; font-size: 7pt; white-space: pre-wrap;\">Сепсис.</span></span></li>\r\n<li><span id=\"docs-internal-guid-a6472c3c-7fff-df19-005a-9ad9901b77fb\"><span style=\"background-color: transparent; font-size: 7pt; white-space: pre-wrap; font-family: Verdana;\">Остеопороз, характеризующийся множественными переломами.</span></span></li>\r\n<li><span id=\"docs-internal-guid-a6472c3c-7fff-df19-005a-9ad9901b77fb\"><span style=\"background-color: transparent; font-size: 7pt; white-space: pre-wrap; font-family: Verdana;\">Симпатоадреналовый криз (рвота, гипотензия, спутанность сознания, боль в животе, ацидоз).</span></span></li>\r\n</ul>",
"image": "http://api.symptomd.ru/storage/971a3ca3bdc1aeec97f3c3ca3c01c4f6_8iWOf3H.jpg",
"image_alt": "Синдром Иценко-Кушинга",
"standard_type": 1,
"danger": 70,
"published": 1,
"parent": null,
"block_rubric": 48,
"standards": []
},
{
"id": 6235,
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"alternative_names": [
{
"name": "Гиперфункцией коры надпочечников"
}
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"complications": [],
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"pathogenesis": "<p>Сущность патологии состоит в том, что вследствие дефекта в энзимной системе коры надпочечников (врожденного или приобретенного) нарушается способность последней синтезировать гидрокортизон и кортизон из 17-λ-гидроксипрогестерона. Недостаточное образование гидрокортизона корой надпочечников обусловливает увеличение секреции АКТГ гипофизом, что вызывает еще большее образование 17-λ-гидрокортизона. Избыток последнего ведет к усиленному образованию различных андрогенов (мужских половых гормонов). Большое количество андрогенов угнетает гонадотропную функцию гипофиза, что ведет к снижению функции яичников.</p>",
"diagnostics": "<p>Диагностирование основано на наличии характерных клинических симптомов, исследовании экскреции 17-кетостероидов с мочой – при адреногенитальном синдроме она увеличивается в 2–3 раза и составляет 25–40 мг/сут., при опухолях надпочечников она увеличивается до 200 мг/сут.</p>",
"treatment": "<p>При опухолях коры надпочечников проводят удаление опухоли, после операции проявления вирильного синдрома исчезают. При врожденном адреногенитальном синдроме огромную роль играет возраст, в котором начато лечение. Чем раньше начата терапия, тем она эффективнее.<br /><br />Лечение заключается в применении кортизона или преднизолона, они тормозят темп роста и процесс окостенения. Тело приобретает черты женского организма, формируются молочные железы, появляются вторичные половые признаки, присущие женскому организму. Необходимо оперативное вмешательство – коррекция наружных половых органов (удаление пенисообразного клитора и формирование входа во влагалище).<br /><br />Прогноз<br /><br />При своевременно начатой адекватной терапии и успешной операции благоприятный, однако такие девочки остаются бесплодными.</p>",
"prevention": "",
"clinical_picture": "<p>При врожденном адреногенитальном синдроме сразу же после рождения обращает на себя внимание нарушение строения наружных половых органов. Нарушения выражаются в увеличении клитора различной степени (от гипертрофии до пенисообразного), наличии урогенитального синуса, углублении преддверья влагалища, высокой промежности, недоразвитии малых и больших половых губ. Если вирилизация значительно выражена, то нередко возникают затруднения в определении пола ребенка. Соматическое развитие детей с адреногенитальным синдромом характеризуется резко выраженным ускорением темпа роста в первое десятилетие жизни (в 2–3 раза по сравнению со здоровыми девочками). К 12 годам рост прекращается, окостенение почти завершено. Телосложение в связи с этим носит диспластический характер: рост ниже среднего, широкие плечи, узкий таз, короткие конечности, массивные трубчатые кости. Период полового созревания происходит рано, в 6–7 лет, и протекает по гетеросексуальному типу. Молочные железы и менструальная функция отсутствуют. У половозрелых женщин (с приобретенным адреногенитальным синдромом) наблюдается вирилизация различной степени. Она проявляется чрезмерным гирсутизмом (оволосением), олигоменореей или аменореей, уменьшением размеров матки и яичников, бесплодием, атрофией молочных желез. Голос становится низким, кожа становится пористой, начинают расти волосы на подбородке и щеках.</p>",
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},
"code": "D45",
"name": "Полицитемия истинная",
"icd_name": "Полицитемия истинная",
"gender": 0,
"age_min": 1,
"age_max": 100,
"cause": [
"0"
],
"periodicity": 3,
"slug": "D45_policitemiya_istinnaya",
"lead": "хроническое заболевание, при котором костный мозг вырабатывает избыточное количество эритроцитов",
"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;\">В зависимости от причин патологию делят на истинную (первичную) и вторичную.</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;\">Истинная полицитемия всегда является проявлением опухолеобразного разрастания клеток, предшественников нормальных эритроцитов.</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;\">Вторичная полицитемия формируется под воздействием разных заболеваний, вызывающих «сгущение» крови. Это могут быть:</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;\">фактор обезвоживания (потеря жидкости от частой и обильной рвоты при холере, отравлениях, диареи, большой ожоговой поверхности);</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;\">гипоксия (недостаток кислорода), вызванная подъемом в горы, жарой, повышенной потливостью при лихорадке.</span></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;\">Вторичное состояние является клиническим проявлением другой болезни, например, сальмонеллеза или дизентерии. При этом общая масса эритроцитов в крови у пациентов остается нормальной.</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;\">На развитие заболевания могут влиять различные факторы, например: </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;\">увеличение селезенки, </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;\">повышенное артериальное давление, </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;\">наследственная предрасположенность.</span></li>\r\n</ul>",
"pathogenesis": "<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 патогенезе полицитемии ведущая роль принадлежит усиленному эритропоэзу, следствием которого служит абсолютный эритроцитоз, нарушение реологических и свертывающих свойств крови, миелоидная метаплазия селезенки и печени. Высокая вязкость крови обусловливает склонность к сосудистым тромбозам и гипоксическому повреждению тканей, а гиперволемия вызывает повышенное кровенаполнение внутренних органов. В финале полицитемии отмечается истощение кроветворения и миелофиброз.</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;\">Диагноз помогают поставить:</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;\">Врачебный осмотр</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;\">Общий и биохимический анализы крови</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;\">Определение концентрации эритропоэтина в крови</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;\">Морфологическое и гистологическое исследование костного мозга</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;\">Цитогенетическое исследование</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;\">УЗИ органов брюшной полости</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;\">Дополнительные исследования (по показаниям)</span></li>\r\n</ul>\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;\">В лечебные мероприятия полицитемии обычно входит кровопускание, позволяющее снизить количество красных кровяных телец. Для этого с интервалом в 1-2 дня забирают по 500 мл крови пока количество эритроцитов не упадет. Процедуру кровопускания гематологи иной раз заменяют проведением эритроцитофереза, когда после забора методом центрифугирования или сепарирования отделяется красная кровь, а плазма возвращается больному.</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;\">Также лечение заключается в назначении цитостатической терапии и антиагрегантов. Схема лечения эритремии расписывается врачом для каждого случая индивидуально.</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;\">Немалая роль в лечении полицитемии отводится режиму труда (снижение физических нагрузок), отдыха и питания. На начальной стадии пациенту не рекомендуют употреблять продукты, усиливающие кроветворение (печень, например) и предлагают пересмотреть рацион, отдавая предпочтение молочным и растительным продуктам.</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;\">Во второй стадии болезни пациенту ограничивают или вовсе исключают рыбные и мясные блюда, бобовые и щавель. Выписавшись из стационара, больной должен придерживаться данных врачом рекомендаций и при амбулаторном наблюдении или лечении.</span></p>",
"prevention": "<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 настоящее время не разработаны специфические методы профилактики этого заболевания ввиду отсутствия достаточной информации о причинах его возникновения. Важно соблюдать общие рекомендации:</span></p>\r\n<ul style=\"margin-top: 0; margin-bottom: 0; padding-inline-start: 48px;\">\r\n<li><span style=\"font-size: 7pt; color: #000000; background-color: transparent; font-style: normal; font-variant-numeric: normal; font-variant-east-asian: normal; vertical-align: baseline; white-space: pre-wrap;\">Вести здоровый образ жизни, отказавшись от вредных привычек.</span></li>\r\n<li><span style=\"font-size: 7pt; color: #000000; background-color: transparent; font-style: normal; font-variant-numeric: normal; font-variant-east-asian: normal; vertical-align: baseline; white-space: pre-wrap;\">Правильно и полноценно питаться.</span></li>\r\n<li><span style=\"font-size: 7pt; color: #000000; background-color: transparent; font-style: normal; font-variant-numeric: normal; font-variant-east-asian: normal; vertical-align: baseline; white-space: pre-wrap;\">Исключить контакты с радиацией и вредными веществами.</span></li>\r\n<li><span style=\"font-size: 7pt; color: #000000; background-color: transparent; font-style: normal; font-variant-numeric: normal; font-variant-east-asian: normal; vertical-align: baseline; white-space: pre-wrap;\">Регулярно проходить профилактические осмотры.</span></li>\r\n</ul>",
"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;\">В два синдрома объединены симптомы истинной полицитемии: плеторический и миелопролиферативный.</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;\">Плеторический (или полнокровный) синдром связан с усилением кровенаполнения органов. К нему относятся:</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;\">головные боли и головокружение;</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;\">при физической нагрузке давящая боль за грудиной;</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;\">тяжесть в голове;</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;\">эритроцианоз (покраснение с синюшным оттенком кожных покровов);</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;\">покраснение глаз;</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;\">из-за увеличения селезенки появляется тяжесть в верхней части живота;</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;\">кожный зуд, который увеличивается после водных процедур;</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;\">повышенное артериальное давление;</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;\">эритромелалгия (острые боли на кончиках пальцев, которые после приема препаратов, разжижающих кровь, уменьшаются).</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 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 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\"><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 повышенным образованием эритроцитов, тромбоцитов и лейкоцитов связан миелопролиферативный синдром, который характеризуется:</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;\">болями в костях;</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;\">тяжестью в левой части живота из-за увеличения селезенки;</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;\">тяжестью в правой части живота из-за увеличения печени;</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;\">повышением температуры.</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> </p>\r\n<p style=\"line-height: 1.38; margin-top: 0pt; margin-bottom: 0pt;\" dir=\"ltr\"> </p>",
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