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},
"code": "E23.2",
"name": "Несахарный диабет",
"icd_name": "Несахарный диабет",
"gender": 0,
"age_min": 1,
"age_max": 50,
"cause": [
"0"
],
"periodicity": 3,
"slug": "e23.2_nesaharnyy_diabet",
"lead": "эндокринное заболевание, связанное с неспособностью почек концентрировать мочу и реабсорбировать воду",
"description": "<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: #ffffff; font-weight: 400; font-style: normal; font-variant: normal; text-decoration: none; vertical-align: baseline; white-space: pre-wrap;\">Несахарный диабет – хроническая патология, развивающаяся в результате дефицита антидиуретического гормона (АДГ) или снижении чувствительности к нему почечной ткани. Характеризуется полиурией и сильной жаждой. </span></p>",
"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;\">Врожденный:</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: 6.999999999999999pt; font-family: Verdana; color: #243178; 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;\">аутосомно-доминантный;</span></p>\r\n</li>\r\n<li style=\"list-style-type: disc; font-size: 6.999999999999999pt; font-family: Verdana; color: #243178; 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;\">сочетание сахарного диабета и несахарного диабета, атрофии дисков зрительных нервов и нейросенсорной тугоухости.</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;\"> </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;\">Приобретенный:</span></p>\r\n<p> </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: 6.999999999999999pt; font-family: Verdana; color: #243178; 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;\">травма (нейрохирургические операции, черепно-мозговая травма);</span></p>\r\n</li>\r\n<li style=\"list-style-type: disc; font-size: 6.999999999999999pt; font-family: Verdana; color: #243178; 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;\">опухоли (краниофарингиома, герминома, глиома и др.);</span></p>\r\n</li>\r\n<li style=\"list-style-type: disc; font-size: 6.999999999999999pt; font-family: Verdana; color: #243178; 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;\">метастазы в гипофиз опухолей других локализаций;</span></p>\r\n</li>\r\n<li style=\"list-style-type: disc; font-size: 6.999999999999999pt; font-family: Verdana; color: #243178; 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;\">гипоксическое/ишемическое повреждение мозга;</span></p>\r\n</li>\r\n<li style=\"list-style-type: disc; font-size: 6.999999999999999pt; font-family: Verdana; color: #243178; 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;\">лимфоцитарный нейрогипофизит;</span></p>\r\n</li>\r\n<li style=\"list-style-type: disc; font-size: 6.999999999999999pt; font-family: Verdana; color: #243178; 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;\">гранулема (туберкулез, саркоидоз, гистиоцитоз);</span></p>\r\n</li>\r\n<li style=\"list-style-type: disc; font-size: 6.999999999999999pt; font-family: Verdana; color: #243178; 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;\">инфекции (врожденные цитомегаловирусная инфекция, токсоплазмоз, энцефалит, менингит);</span></p>\r\n</li>\r\n<li style=\"list-style-type: disc; font-size: 6.999999999999999pt; font-family: Verdana; color: #243178; 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;\">сосудистая патология (аневризма, мальформации сосудов);</span></p>\r\n</li>\r\n<li style=\"list-style-type: disc; font-size: 6.999999999999999pt; font-family: Verdana; color: #243178; 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;\">идиопатический.</span></p>\r\n</li>\r\n</ul>",
"pathogenesis": "<p><span id=\"docs-internal-guid-cb12b8e1-7fff-8608-3ac0-68847f7b6772\"><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;\">Механизм развития несахарного диабета связан с недостаточностью вазопрессина, что приводит к нарушению концентрации мочи на уровне дистальных канальцев нефрона, в результате чего выделяется большой объем мочи с низким удельным весом; развивается полидипсия. Если прием жидкости строго ограничивать, формируется гиперосмолярная дегидратация; избыток жидкости может спровоцировать синдром раздраженного кишечника, опущение желудка и другими заболеваниями.</span></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;\">Для диагностики несахарного диабета специалисты Клинического госпиталя на Яузе проводят следующие лабораторные исследования:</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: 6.999999999999999pt; font-family: Verdana; color: #2d3e50; 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;\">общий анализ крови</span></p>\r\n</li>\r\n<li style=\"list-style-type: disc; font-size: 6.999999999999999pt; font-family: Verdana; color: #2d3e50; 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;\">биохимический анализ крови с определением уровня глюкозы, креатинина, билирубина, холестерина, электролитов крови, ACT и АЛТ</span></p>\r\n</li>\r\n<li style=\"list-style-type: disc; font-size: 6.999999999999999pt; font-family: Verdana; color: #2d3e50; 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;\">гормональные анализы крови</span></p>\r\n</li>\r\n<li style=\"list-style-type: disc; font-size: 6.999999999999999pt; font-family: Verdana; color: #2d3e50; 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;\">гликемический профиль</span></p>\r\n</li>\r\n<li style=\"list-style-type: disc; font-size: 6.999999999999999pt; font-family: Verdana; color: #2d3e50; 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;\">общий анализ мочи</span></p>\r\n</li>\r\n<li style=\"list-style-type: disc; font-size: 6.999999999999999pt; font-family: Verdana; color: #2d3e50; 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;\">проба по Зимницкому</span></p>\r\n</li>\r\n<li style=\"list-style-type: disc; font-size: 6.999999999999999pt; font-family: Verdana; color: #2d3e50; 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;\">проба Реберга</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;\">Также предусмотрена инструментальная диагностика, которая включает МРТ головного мозга, ЭКГ, Rg-графию грудной клетки, УЗИ органов малого таза, ЭЭГ.</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;\">Лечение симптоматического несахарного диабета начинают с устранения причины (например, опухоли). При всех формах несахарного диабета назначают заместительную терапию синтетическим аналогом АДГ - десмопрессином. Препарат применяется внутрь или интраназально (путем закапывания в нос). </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;\">Назначают также пролонгированный препарат из масляного раствора питуитрина. При центральной форме несахарного диабета назначают хлорпропамид, карбамазепин, стимулирующие секрецию антидиуретического гормона.</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;\">Проводится коррекция водно-солевого баланса путем инфузионного введения солевых растворов в больших объемах. </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;\">Значительно уменьшают диурез при несахарном диабете сульфаниламидные диуретики (гипохлоротиазид). </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-style: normal; font-variant-numeric: normal; font-variant-east-asian: normal; vertical-align: baseline; white-space: pre-wrap;\">Питание при несахарном диабете строится на ограничении белка (для уменьшения нагрузки на почки) и достаточном потреблении углеводов и жиров, частом приеме пищи, увеличении количества овощных и фруктовых блюд. Из напитков утолять жажду рекомендуется соками, морсами, компотами.</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;\">Методов первичной профилактики не разработано, поскольку единой причины патологии нет. Врачи рекомендуют отказаться от вредных привычек и регулярно проходить профилактические осмотры.</span></p>",
"clinical_picture": "<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;\">Основные проявления несахарного диабета — выраженная полиурия (выделение мочи более 2 л/м2 в сутки или 40 мл/кг в сутки у старших детей и взрослых), полидипсия (порядка 3–18 л/сут) и связанные с ними нарушения сна. </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;\">К проявлениям заболевания у взрослых относятся следующие:</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: 6.999999999999999pt; 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, снижение работоспособности, похудение;</span></p>\r\n</li>\r\n<li style=\"list-style-type: disc; font-size: 6.999999999999999pt; 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: 6.999999999999999pt; 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;\">изменение секреции слюны;</span></p>\r\n</li>\r\n<li style=\"list-style-type: disc; font-size: 6.999999999999999pt; 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;\">сухость кожных покровов;</span></p>\r\n</li>\r\n<li style=\"list-style-type: disc; font-size: 6.999999999999999pt; 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 размеров мочевого пузыря, желудка из-за растяжения этих органов;</span></p>\r\n</li>\r\n<li style=\"list-style-type: disc; font-size: 6.999999999999999pt; 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;\">нарушение секреции пищеварительных ферментов;</span></p>\r\n</li>\r\n<li style=\"list-style-type: disc; font-size: 6.999999999999999pt; 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;\">пониженное кровяное давление;</span></p>\r\n</li>\r\n<li style=\"list-style-type: disc; font-size: 6.999999999999999pt; 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 частоты сердечных сокращений;</span></p>\r\n</li>\r\n<li style=\"list-style-type: disc; font-size: 6.999999999999999pt; 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;\">бессонница, раздражительность;</span></p>\r\n</li>\r\n<li style=\"list-style-type: disc; font-size: 6.999999999999999pt; 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;\">нарушение менструального цикла у женщин.</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;\">Несахарный диабет может спровоцировать у женщин развитие бесплодия, выкидыши, а у мужчин - импотенцию.</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;\">Несахарный диабет у мужчин характеризуется слабым половым влечением и импотенцией, у женщин может привести к прерыванию беременности, бесплодию и нарушениям менструаций.</span></p>\r\n<p style=\"line-height: 1.38; margin-top: 0pt; margin-bottom: 0pt;\" dir=\"ltr\"><strong id=\"docs-internal-guid-5c5260d5-7fff-6a7b-e538-1f408d5dd4c3\" style=\"font-weight: normal;\"> </strong></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 случае наличия заболевания у ребенка до 3 лет или у подростка ход заболевания будет таким же, что и у взрослых, однако симптомы при этом могут быть менее ярко выраженными. </span></p>\r\n<p style=\"line-height: 1.38; margin-top: 0pt; margin-bottom: 0pt;\" dir=\"ltr\"><strong style=\"font-weight: normal;\"> </strong></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;\">Как правило, у детей ухудшается аппетит, повышается вес и возникает рвота при употреблении еды. Поздний период сопровождается констипацией, энурезом и болезненными ощущениями в суставах. Помимо этого, бывают задержка полового созревания и роста.</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 ребенка возрастом до одного года отмечаются такие симптомы:</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: 6.999999999999999pt; font-family: Verdana; color: #424242; 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;\">Постоянно меняющаяся температура тела;</span></p>\r\n</li>\r\n<li style=\"list-style-type: disc; font-size: 6.999999999999999pt; font-family: Verdana; color: #424242; 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 частое мочеиспускание, причем в больших количествах;</span></p>\r\n</li>\r\n<li style=\"list-style-type: disc; font-size: 6.999999999999999pt; font-family: Verdana; color: #424242; 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;\">Очень малое число слез;</span></p>\r\n</li>\r\n<li style=\"list-style-type: disc; font-size: 6.999999999999999pt; font-family: Verdana; color: #424242; 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;\">Отсутствие жажды или ребенок начинает употреблять воду чаще и не хочет пить грудное молоко;</span></p>\r\n</li>\r\n<li style=\"list-style-type: disc; font-size: 6.999999999999999pt; font-family: Verdana; color: #424242; 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;\">Сниженный вес тела и учащенные рвотные позывы;</span></p>\r\n</li>\r\n<li style=\"list-style-type: disc; font-size: 6.999999999999999pt; 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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>",
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"code": "E24.1",
"name": "Синдром Нельсона",
"icd_name": "Синдром Нельсона",
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"slug": "e24.1_sindrom_nelsona",
"lead": "нейроэндокринная патология, характеризующаяся хронической надпочечниковой недостаточностью, гиперпигментацией кожных покровов и слизистых оболочек и наличием аденомы гипофиза",
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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;\">Синдром Нельсона возникает при абсолютном выпадении функции коры надпочечников в результате лечения болезни Иценко-Кушинга. Причинами 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;\">Двусторонняя адреналэктомия. Хирургическое удаление двух надпочечников – наиболее распространенный фактор возникновения синдрома. Выработка кортикостероидов прекращается, синтез АКТГ значительно увеличивается.</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;\">Односторонняя адреналэктомия, терапия митотаном. Реже патология развивается после удаления одного надпочечника, на фоне угнетения функций второго препаратами группы ингибиторов синтеза стероидов. Патологические процессы аналогичны таковым при билатеральной адреналэктомии, но медленнее прогрессируют.</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;\">Длительное лечение митотаном. Препарат относится к противоопухолевым средствам, ингибиторам производства гормонов надпочечников. Он разрушает опухолевую и нормальную ткань надпочечниковой коры. При длительном приеме возможна полная утрата гормоносекретирующей функции.</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>\r\n<p style=\"line-height: 1.38; margin-top: 0pt; margin-bottom: 0pt;\" dir=\"ltr\"> </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;\">Ключевые патогенетические процессы синдрома Нельсона – недостаток ингибирующего влияния кортизола на АКТГ-производящие клетки, возобновление роста и быстрое прогрессирование гипофизарной опухоли, сформировавшейся при болезни Кушинга. После резекции надпочечников или при продолжительном приеме митотана нарушается физиологическая регуляция функций надпочечников гипоталамо-гипофизарным комплексом. Это ведет к значительному повышению количества АКТГ, формированию гипофизарной аденомы.</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;\">Усиление пигментации объясняется вненадпочечниковым воздействием АКТГ на меланоциты – клетки кожи, производящие пигмент мелатонин черно-коричневого и желто-красного цвета. Кроме этого, гормон стимулирует липолиз и секрецию инсулина, оказывая гипогликемический эффект. У лиц с синдромом Нельсона он усиливает метаболизм кортикоидных гормонов, поэтому для устранения признаков надпочечниковой недостаточности требуется назначение высоких доз их синтетических аналогов.</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;\">Диагностирование синдрома Нельсона основано на результатах лабораторно-инструментального обследования:</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;\">Магниторезонансной и компьютерной томографии гипофиза – для визуализации микро-аденом.</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;\">Клинических анализов крови и мочи – для оценивания общего состояния пациента.</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;\">Биохимического анализа крови – для измерения концентрации АКТГ, кортизола, глюкозы.</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;\">Рентгенографии турецкого седла – для обнаружения признаков неоплазии (расширения входа и укорочения клиновидных отростков).</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;\">Нейроофтальмологического обследования – для выявления изменений на глазном дне, снижения остроты зрения, опущения верхнего века.</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;\">Электроэнцефалографии – для регистрации биоэлектрических потенциалов коры головного мозга и оценивания состояния нервной системы.</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;\">Флебографии кавернозного синуса.</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;\">Для достижения положительного результата и предупреждения рецидивов используются комбинированные методы лечения, включающие следующие процедуры в различных сочетаниях:</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;\">Прием медикаментов. Медикаментозная коррекция целесообразна при новообразовании, которое не выходит за пределы турецкого седла, при рецидиве синдрома. Используются препараты, снижающие уровень АКТГ: серотониновые блокаторы, стимуляторы дофаминовых рецепторов. Для компенсации ХНН проводится заместительная терапия минерало- и глюкокортикоидами.</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;\">Радиационная терапия. Курс процедур рекомендуется пациентам с аденомами маленьких размеров, а также после проведения резекции опухоли, при рецидивах синдрома. Применяется методика облучения γ-лучами и протонными пучками, введения в ткани гипофиза радиоактивных изотопов иттрия и серебра.</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;\">Хирургическая операция. Метод показан при аденомах маленьких, средних и больших размеров. Микрохирургическое оборудование позволяет полностью удалить образование, но не исключает развития рецидива, поэтому после оперативного вмешательства назначается курс лучевого лечения.</span></li>\r\n<li><span id=\"docs-internal-guid-38df3fa7-7fff-8b5b-98d7-1e28bbc91d9d\"><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;\">Криотерапия. Низкотемпературный метод воздействия эффективен при опухолях небольших размеров. Жидкий азот стереотаксически через трансназальный транссфеноидальный доступ подводится к аденоме и разрушает ее ткани.</span></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>",
"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 качестве профилактической меры больным, перенесшим билатеральную адреналэктомию, рекомендуется ежегодно назначать обследования, которые позволяют визуализировать область расположения гипофизарной аденомы, контролировать содержание АКТГ. Кроме того, пациентам показаны регулярные офтальмологические осмотры.</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;\">Синдром Нельсона проявляется в следующих симптомах: прогрессирующий рост опухоли в гипофизе и сдавливание соседних структур головного мозга; передозировка препаратами, заменяющими гормоны удаленных надпочечников (врачам очень сложно угадать индивидуальную дозу); недостаток поступления препаратов, которые вырабатывали удаленные надпочечники.</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;\">Диагностировать синдром Нельсона можно по следующим симптомам и жалобам больного:</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;\">частые и сильные головные боли;</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;\">уменьшение остроты зрения и прочие нарушения;</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;\">снижение обоняния;</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;\">диэнцефальные нарушения;</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;\">изменение вкусовых ощущений;</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;\">повышенная пигментация (потемнение) открытых частей тела.</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\"><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 передозировках препаратов, заменяющих гормоны надпочечника, наблюдаются такие симптомы: </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;\">неравномерное ожирение;</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;\">округление лица и его покраснение; </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;\">учащенное сердцебиение; </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;\">боль в спине; </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;\">слабость в ногах; </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;\">артериальная гипертензия.</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;\">В случае недостаточного поступления препаратов, которых пациент принимает для замены гормонов надпочечников, могут проявиться следующие симптомы: </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;\">плохой аппетит; </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;\">частая тошнота и рвота; </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;\">не проходящая, нарастающая общая слабость; </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;\">повышенная утомляемость; </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;\">боли в суставах; </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;\">низкое артериальное давление.</span></li>\r\n</ul>",
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"etiology": "<p>Этот патологический синдром обусловлен гиперфункцией коры надпочечников, которая может быть вызвана:<br /><br />1) генетически обусловленной недостаточностью ферментной системы 21-гидроксилазы в коре надпочечников (врожденный адреногенитальный синдром);<br /><br />2) гиперплазией или опухолью коры надпочечников (приобретенный адреногенитальный синдром).</p>",
"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>",
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"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": "E26",
"name": "Гиперальдостеронизм",
"icd_name": "Гиперальдостеронизм",
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"slug": "e26_giperaldosteronizm",
"lead": "заболевание, обусловленное повышенным содержанием альдостерона",
"description": "Гиперальдостеронизм – патологическое состояние, обусловленное повышенной продукцией альдостерона - основного минералокортикоидного гормона коры надпочечников.\r\n\r\n",
"etiology": "<p>Чаще всего первичный гиперальдостеронизм наблюдается при следующих патологических состояниях:</p>\r\n<ul>\r\n<li>доброкачественные и злокачественные опухоли надпочечников (прежде всего, аденома коры надпочечников);</li>\r\n<li>гиперплазия коры надпочечников.</li>\r\n</ul>\r\n<p> Вторичный гиперальдостеронизм развивается на фоне:</p>\r\n<ul>\r\n<li>избыточного поступления калия;</li>\r\n<li>повышенной потери натрия;</li>\r\n<li>гиперсекреции ренина;</li>\r\n<li>сердечной недостаточности;</li>\r\n<li>злокачественной артериальной гипертензии;</li>\r\n<li>циррозе печени;</li>\r\n<li>почечной недостаточности;</li>\r\n<li>резкого уменьшения объема циркулирующей жидкости;</li>\r\n<li>беременности;</li>\r\n<li>перераспределения внеклеточной жидкости, что ведет к снижению наполнения кровью крупных сосудов;</li>\r\n<li>длительный прием некоторых лекарственных средств (диуретиков, слабительных).</li>\r\n</ul>",
"pathogenesis": "<p>Основу патогенеза первичного гиперальдостеронизма составляет влияние избытка альдостерона на водно-электролитный баланс: повышение всасывания ионов натрия и воды в почечных канальцах и усиленное выведение ионов калия с мочой, приводящее к задержке жидкости и гиперволемии (увеличению объема циркулирующей крови), метаболическому алкалозу (повышению pH крови), снижению выработки и активности ренина плазмы крови. Отмечается нарушение гемодинамики - повышение чувствительности сосудистой стенки к действию внутренних прессорных факторов и сопротивления периферических сосудов току крови. При первичном гиперальдостеронизме выраженный и длительный низкий уровень калия приводит к дистрофическим изменениям в почечных канальцах и мышцах.</p>\r\n<p>Вторичный гиперальдостеронизм возникает компенсаторно, в ответ на снижение объема почечного кровотока при различных заболеваниях почек, печени, сердца. Вторичный гиперальдостеронизм развивается за счет активации ренин-ангиотензиновой системы и усиления продукции ренина почками. Характерные для первичного гиперальдостеронизма выраженные электролитные нарушения при вторичной форме не возникают.</p>",
"diagnostics": "<p>Рутинный метод выявления пациентов из группы риска – измерение уровня артериального давления. Его повышенные значения – показание к проведению скринингового обследования (определения концентрации калия в плазме крови).</p>\r\n<p>Снижение концентрации калия в крови менее 2,7 мэкв/л предполагает дальнейшее обследование для уточнения клинического диагноза.</p>\r\n<p>Основной диагностический метод при данном синдроме – определение уровня альдостерона в плазме крови и выявление повышенной суточной экскреции его метаболита (альдостерон-18-глюкуронида) с мочой.</p>\r\n<p>Дополнительно используются следующие методы визуализации:</p>\r\n<ul>\r\n<li>ультразвуковое сканирование надпочечников;</li>\r\n<li>сцинтиграфия надпочечников;</li>\r\n<li>компьютерная или магнитно-резонансная томография надпочечников.</li>\r\n</ul>\r\n<p>Генетическая диагностика позволяет выявить псевдогиперальдостеронизм и семейные формы первичного заболевания.</p>",
"treatment": "<p>Тактика терапии недуга диктуется его разновидностью, однако есть несколько методик лечения, присущих всем формам гиперальдостеронизма. К ним стоит отнести:</p>\r\n<ul>\r\n<li>соблюдение щадящего рациона, направленного на снижение потребления поваренной соли и обогащение меню продуктами, обогащёнными калием;</li>\r\n<li>приём калийсберегающих мочегонных средств;</li>\r\n<li>инъекционное введение препаратов калия.</li>\r\n</ul>\r\n<p>Лечение гиперальдостеронизма, вызванного формированием альдостеромы или онкологическим поражением надпочечников, только хирургическое. Операция предполагает иссечение поражённого сегмента, что предварительно требует восстановления водно-электролитного баланса.</p>\r\n<p>Лечение вторичного гиперальдостеронизма заключается в патогенетической и симптоматической терапии основного заболевания.</p>\r\n<p>Больных с двусторонней гиперплазией коры надпочечников обычно лечат консервативно калийсберегающими диуретиками в комбинации с ингибиторами АПФ, антагонистами кальциевых каналов.</p>",
"prevention": "<p>С целью профилактики гиперальдостеронизма необходимо постоянное диспансерное наблюдение лиц с артериальной гипертензией, заболеваниями печени и почек; соблюдение медицинских рекомендаций относительно приема медикаментов и характера питания.</p>",
"clinical_picture": "<p>У пациентов с первичным гиперальдостеронизмом возникает выраженная или умеренная артериальная гипертензия, головные боли, ноющие боли в области сердца (кардиалгия), нарушения сердечного ритма, изменения глазного дна с ухудшением зрительной функции (гипертоническая ангиопатия, ангиосклероз, ретинопатия).</p>\r\n<p>Дефицит калия приводит к появлению быстрой утомляемости, мышечной слабости, парестезий, приступов судорог в различных группах мышц, периодических псевдопараличей; в тяжелых случаях – поражению сердца, почек, развитию несахарного диабета.</p>\r\n<p>При вторичном гиперальдостеронизме наблюдается высокий уровень артериального давления, постепенно приводящий к поражению сосудистой стенки и ишемии тканей, ухудшению функции почек и развитию хронической почечной недостаточности, изменениям глазного дна. Наиболее частым признаком вторичного гиперальдостеронизма являются отеки. Вторичный гиперальдостеронизм может протекать без артериальной гипертензии, у некоторых пациентов наблюдается малосимптомное течение гиперальдостеронизма.</p>",
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}