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"lead": "постепенное увеличение объема конечности вследствие нарушения оттока лимфы и ее последующего скопления в межклеточном пространстве",
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"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;\">Первичный лимфостаз нижних конечностей обусловлен врожденными пороками развития лимфатической системы.</span></p>\r\n<p><span id=\"docs-internal-guid-8c1314e4-7fff-c9fc-fb8b-b928ac24c4ad\"> </span></p>",
"diagnostics": "<p><span style=\"background-color: transparent; font-family: Verdana; font-size: 7pt; white-space: pre-wrap;\">Чтобы установить и подтвердить диагноз, врач анализирует клиническую картину, изучает семейную историю болезни, проводит физикальный осмотр и направляет пациента на дополнительные обследования. </span></p>\r\n<p><span style=\"background-color: transparent; font-family: Verdana; font-size: 7pt; white-space: pre-wrap;\">В целях диагностики выполняют ультразвуковую допплерографию, компьютерную томографию, лимфангиографию и молекулярно-генетические исследования, методом секвенирования генов GJC2 и VEGF3. </span></p>\r\n<p><span style=\"background-color: transparent; font-family: Verdana; font-size: 7pt; 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: 6.999999999999999pt; font-family: Verdana; color: #000000; background-color: transparent; font-weight: 400; font-style: normal; font-variant: normal; text-decoration: none; vertical-align: baseline; white-space: pre-wrap;\">Терапия наследственной лимфедемы сводится к нескольким принципам – улучшению оттока лимфы, снижению задержки тканевой жидкости и устранению вторичных нарушений, обусловленных отечным синдромом. </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;\">Для улучшения оттока применяют разнообразные лимфодренажные массажи (как ручные, так и аппаратные) и особый компрессионный трикотаж, иногда используют местные лекарственные средства. </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;\">Для некоторых форм наследственной лимфедемы могут быть эффективными хирургические методики, создающие более полноценные пути эвакуации тканевой жидкости в лимфатическую или кровеносную системы. </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;\">Для снижения задержки лимфы в тканях назначают специальные диеты с пониженным содержанием белков и солей – эти нутриенты способны значительно усугублять течение лимфатического отека.</span></p>\r\n<p> </p>\r\n<p style=\"line-height: 1.38; margin-top: 0pt; margin-bottom: 0pt;\" dir=\"ltr\"><span style=\"font-size: 6.999999999999999pt; font-family: Verdana; color: #000000; background-color: transparent; font-weight: 400; font-style: normal; font-variant: normal; text-decoration: none; vertical-align: baseline; white-space: pre-wrap;\">При запущенных формах наследственной лимфедемы с выраженными вторичными нарушениями (слоновостью, трофическими язвами, инфекцией) проводят лечение осложнений. Для борьбы с инфекцией назначают антибиотики, трофические нарушения лечат при помощи витаминотерапии, регенеративных средств и биогенных стимуляторов. При выраженной слоновости иногда производится хирургическое удаление лишнего объема тканей (резекционное оперативное вмешательство), что приводит к улучшению как внешнего вида, так и состояния больного наследственной лимфедемой.</span></p>",
"prevention": "<p><span id=\"docs-internal-guid-5b5d8d0f-7fff-dcec-1082-a6276ac506b5\"><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;\">Специфические методы профилактики не разработаны. Парам, планирующим беременность, рекомендована консультация генетика. </span></span></p>\r\n<p><span id=\"docs-internal-guid-5b5d8d0f-7fff-dcec-1082-a6276ac506b5\"><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;\">Если у пациента выявлена наследственная лимфедема, ему необходимо соблюдать диету, отказаться от длительных физических нагрузок на конечности, а также придерживаться медицинских рекомендаций.</span></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;\">Первичная лимфедема развивается из-за врожденной аплазии или гипоплазии лимфатических сосудов.</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;\">Компенсированная первичная лимфедема нередко быстро прогрессирует после беременности или травмы. </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;\">Жалобы: боли, слабость, ощущение распирания, тяжесть в пораженной конечности, ограничение подвижности суставов. Кожные покровы в пораженной области бледные, рисунок сети подкожных вен не определяется, кожная складка утолщена. Вначале поражаются дистальные отделы конечностей (стопы или кисти). </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;\">Припухлость может быть уже при рождении, но обычно появляется позже, по мере того как объем лимфы увеличивается и превышает возможности малого количества лимфатических сосудов. У пациентов появляется безболезненный плотный отек пальцев, распространяющийся на стопу и голеностопный сустав (при поражении верхней конечности – на кисть и лучезапястный сустав). Постепенно отек распространяется на голень и бедро. Ноги больного становятся похожими на колонны. В области суставов со временем образуются складки из отечных мягких тканей. Складки на тыле стопы не выражены. Кожа напоминает апельсиновую корку. </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;\">Наследственная лимфедема со временем не исчезает полностью даже после ночного отдыха. Нарушается питание тканей. Возникают участки гиперкератоза. Измененная кожа трескается, появляются язвы. Иногда развиваются лимфатические свищи между пальцами. Часто осложняется рожистым воспалением, которое может стать причиной флегмоны, вызывает облитерацию сохранных лимфатических сосудов и приводит к быстрому прогрессированию заболевания.</span></p>",
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