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"lead": "дефект мягких тканей верхней губы с одной или двух сторон от средней линии в виде щели той или иной степени выраженности",
"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: transparent; font-weight: 400; font-style: normal; font-variant: normal; text-decoration: none; vertical-align: baseline; white-space: pre-wrap;\">Заячья губа - это врожденный дефект, образованный несросшимися во внутриутробном периоде тканями носовой полости и верхней челюсти и проявляющийся расщелиной губы. </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;\">С дефектом «заячья губа» причины необходимо искать на генетическом уровне. Ткани челюстно-лицевой области закладываются и формируются в период внутриутробного развития до 8 недель. Если в период беременности женщины было влияние определенных негативных факторов, это также оказывает воздействие на плод. Для формирования патологии могут быть и следующие причины:</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;\">возраст матери и отца, превышающий 40 лет;</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;\">генетическая предрасположенность;</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;\">плохие экологические условия;</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;\">вредные привычки (курение, наркотики, алкоголь);</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;\">сильный авитаминоз во время беременности;</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;\">инфекционные заболевания в анамнезе;</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;\">гипоксия плода на ранних сроках беременности.</span></li>\r\n</ul>",
"pathogenesis": "<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: 8pt; font-family: Verdana; color: #181d21; 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;\">Теория слияния отростков. Согласно этой теории, расщелина появляется в результате несращения лицевых отростков у плода.</span></p>\r\n</li>\r\n<li style=\"list-style-type: disc; font-size: 8pt; font-family: Verdana; color: #181d21; 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;\">Теория миграции мезодермы. На 7 неделе эмбриогенеза в губы проникают клетки мезодермы (среднего зародышевого листка) — миобласты, которые в будущем станут круговой мышцей рта. В соответствии с этой теорией врождённые расщелины появляются из-за недостаточно быстрого прорастания (миграции) мезодермы между слоями эктодермы и эндодермы. В результате этого при быстром росте зародыша происходит разрыв (расщепление) тонкой мембраны экто- и эндодермы с формированием расщелины верхней губы и нёба.</span></p>\r\n</li>\r\n</ul>",
"diagnostics": "<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;\">Диагностика расщелины основывается в первую очередь на осмотре лица ребёнка. Однако такие дети должны быть тщательно обследованы на предмет синдромальной расщелины. Т. е. необходимо проверить, нет ли у ребёнка сопутствующих тяжёлых патологий: сердечно-сосудистой (коарктация дуги аорты, общий артериальный ствол и др.) и нервной (гипотония мышц, эпилепсия и др.) систем. Для этого проводят ЭКГ, ЭЭГ, КТ и МРТ.</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;\">Коррекция заячьей губы проводится в ходе одной или нескольких реконструктивных пластических операций с учетом особенностей каждого варианта дефекта. Проведение пластической коррекции врожденных расщелин губы показано детям, рожденным в срок и не имеющим противопоказаний (сочетанных пороков жизненно важных органов, родовых травм, приобретенных заболеваний, физиологической желтухи и др.). </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;\">Оперативное устранение заячьей губы – хейлопластику лучше проводить в 3-6-месячном возрасте. В случае тяжелого дефекта, возможно ее осуществление в первые дни или месяц жизни ребенка при условии достаточной прибавки в весе, отсутствии анемии, патологии кишечника, сердечно-сосудистой, эндокринной или нервной систем.</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;\">Проведение хейлопластики в первые две недели после рождения благоприятно влияет на развитие губы и носа в дальнейшем, а также уменьшает психическую тревожность родителей. Однако, у детей в этом возрасте анатомические размеры губы еще очень малы, некоторые физиологические функции несовершенны, повышена склонность к кровопотере. При хирургическом устранении расщелины губы достигается восстановление ее анатомической структуры и целостности, устранение деформаций нёба, носа, других лицевых дефектов, а также создание предпосылок для правильного развития челюстно-лицевой системы в детском возрасте.</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;\">Коррекция заячьей губы должна быть завершена к 3-м годам, когда начинается становление речи. В более позднем возрасте проводится логопедическое лечение, направленное на устранение речевых дефектов, и косметическое лечение по устранению послеоперационного рубца. С учетом имеющихся дефектов губы, носа, нёба и альвеолярного отростка в качестве реконструктивных операций выполняют хейлопластику, ринохейлопластику или ринохейлогнатопластику.</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 качестве профилактики беременным женщинам необходимо исключить влияние на плод вредных факторов, особенно на ранних сроках: не допускать травм, отказаться от алкоголя и наркотических средств, не использовать агрессивную бытовую химию. </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-style: normal; font-variant-numeric: normal; font-variant-east-asian: normal; vertical-align: baseline; white-space: pre-wrap;\">Здоровье отца также имеет значение.</span></p>",
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"lead": "дефект мягких тканей верхней губы с одной или двух сторон от средней линии в виде щели той или иной степени выраженности",
"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: transparent; font-weight: 400; font-style: normal; font-variant: normal; text-decoration: none; vertical-align: baseline; white-space: pre-wrap;\">Заячья губа - это врожденный дефект, образованный несросшимися во внутриутробном периоде тканями носовой полости и верхней челюсти и проявляющийся расщелиной губы. </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;\">С дефектом «заячья губа» причины необходимо искать на генетическом уровне. Ткани челюстно-лицевой области закладываются и формируются в период внутриутробного развития до 8 недель. Если в период беременности женщины было влияние определенных негативных факторов, это также оказывает воздействие на плод. Для формирования патологии могут быть и следующие причины:</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;\">возраст матери и отца, превышающий 40 лет;</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;\">генетическая предрасположенность;</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;\">плохие экологические условия;</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;\">вредные привычки (курение, наркотики, алкоголь);</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;\">сильный авитаминоз во время беременности;</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;\">инфекционные заболевания в анамнезе;</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;\">гипоксия плода на ранних сроках беременности.</span></li>\r\n</ul>",
"pathogenesis": "<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: 8pt; font-family: Verdana; color: #181d21; 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;\">Теория слияния отростков. Согласно этой теории, расщелина появляется в результате несращения лицевых отростков у плода.</span></p>\r\n</li>\r\n<li style=\"list-style-type: disc; font-size: 8pt; font-family: Verdana; color: #181d21; 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;\">Теория миграции мезодермы. На 7 неделе эмбриогенеза в губы проникают клетки мезодермы (среднего зародышевого листка) — миобласты, которые в будущем станут круговой мышцей рта. В соответствии с этой теорией врождённые расщелины появляются из-за недостаточно быстрого прорастания (миграции) мезодермы между слоями эктодермы и эндодермы. В результате этого при быстром росте зародыша происходит разрыв (расщепление) тонкой мембраны экто- и эндодермы с формированием расщелины верхней губы и нёба.</span></p>\r\n</li>\r\n</ul>",
"diagnostics": "<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;\">Диагностика расщелины основывается в первую очередь на осмотре лица ребёнка. Однако такие дети должны быть тщательно обследованы на предмет синдромальной расщелины. Т. е. необходимо проверить, нет ли у ребёнка сопутствующих тяжёлых патологий: сердечно-сосудистой (коарктация дуги аорты, общий артериальный ствол и др.) и нервной (гипотония мышц, эпилепсия и др.) систем. Для этого проводят ЭКГ, ЭЭГ, КТ и МРТ.</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;\">Коррекция заячьей губы проводится в ходе одной или нескольких реконструктивных пластических операций с учетом особенностей каждого варианта дефекта. Проведение пластической коррекции врожденных расщелин губы показано детям, рожденным в срок и не имеющим противопоказаний (сочетанных пороков жизненно важных органов, родовых травм, приобретенных заболеваний, физиологической желтухи и др.). </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;\">Оперативное устранение заячьей губы – хейлопластику лучше проводить в 3-6-месячном возрасте. В случае тяжелого дефекта, возможно ее осуществление в первые дни или месяц жизни ребенка при условии достаточной прибавки в весе, отсутствии анемии, патологии кишечника, сердечно-сосудистой, эндокринной или нервной систем.</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;\">Проведение хейлопластики в первые две недели после рождения благоприятно влияет на развитие губы и носа в дальнейшем, а также уменьшает психическую тревожность родителей. Однако, у детей в этом возрасте анатомические размеры губы еще очень малы, некоторые физиологические функции несовершенны, повышена склонность к кровопотере. При хирургическом устранении расщелины губы достигается восстановление ее анатомической структуры и целостности, устранение деформаций нёба, носа, других лицевых дефектов, а также создание предпосылок для правильного развития челюстно-лицевой системы в детском возрасте.</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;\">Коррекция заячьей губы должна быть завершена к 3-м годам, когда начинается становление речи. В более позднем возрасте проводится логопедическое лечение, направленное на устранение речевых дефектов, и косметическое лечение по устранению послеоперационного рубца. С учетом имеющихся дефектов губы, носа, нёба и альвеолярного отростка в качестве реконструктивных операций выполняют хейлопластику, ринохейлопластику или ринохейлогнатопластику.</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 качестве профилактики беременным женщинам необходимо исключить влияние на плод вредных факторов, особенно на ранних сроках: не допускать травм, отказаться от алкоголя и наркотических средств, не использовать агрессивную бытовую химию. </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-style: normal; font-variant-numeric: normal; font-variant-east-asian: normal; vertical-align: baseline; white-space: pre-wrap;\">Здоровье отца также имеет значение.</span></p>",
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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;\">Развитие заболевания связывают с генетическим дефектом, проявляющимся на этапе внутриутробного формирования органов плода. У страдающих декстрокардией выявлены специфические мутации генов. Наибольшее признание получила теория аутосомно-рецессивного наследования порока: аномальное положение сердца носит семейный характер, повторяется через 1-2 поколения.</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;\">Помимо семейной предрасположенности, в процессе мутаций генетического материала доказано участие тератогенных факторов. Экзогенные тератогены подразделяются на 3 основных группы по характеру воздействия:</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;\">физические (ионизирующее излучение);</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;\">химические (алкоголь, лекарственные препараты);</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;\">биологические (бактериальные и вирусные инфекции).</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\"><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;\">Эндогенные причины включают гинекологические и экстрагенитальные заболевания беременной, тяжелые токсикозы.</span></p>",
"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 основе декстрокардии лежит нарушение процессов эмбриогенеза на ранних стадиях внутриутробного развития. В норме на 1-2 месяце беременности формирующаяся сердечная трубка начинает изгибаться влево. При наличии типичных для декстрокардии мутаций происходит аномальное искривление этой трубки в правую сторону. При этом нарушается дальнейшее формирование эмбриона, может изменяться позиция других висцеральных органов, чаще всего печени и селезенки.</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;\">Опытный терапевт-кардиолог может заподозрить декстрокардию уже на первичном приеме при физикальном исследовании. В ходе пальпации предсердечной области верхушечный толчок обнаруживается справа от грудины. Перкуторно врач отмечает смещение границ сердечной тупости вправо, при аускультации выслушиваются изменения сердечных тонов. При подозрении на декстрокардию назначают полное обследование, в которое включены следующие методы:</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;\">ЭКГ. Электрокардиография является наиболее простым и информативным исследованием для подтверждения декстрокардии. На кардиограмме отмечаются отклонение электрической оси сердца вправо, отрицательные зубцы Р и Т, высокоамплитудные зубцы R в правых грудных отведениях.</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;\">Рентгенография ОГК. На рентгенограмме визуализируется смещение сердечной тени вправо, при комбинированных аномалиях изменяются контуры магистральных сосудов. Чтобы подтвердить транспозицию проводится рентгенография 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;\">УЗИ сердца и сосудов. Эхокардиография выполняется для оценки функциональной способности аномально расположенного сердца. На ЭхоКГ видна структура сердечной стенки, скорость кровотока, фракция выброса.</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. В качестве подготовки к хирургической коррекции пороков развития показаны ангиокардиография или катетеризация сердечных полостей. Для выявления сопутствующих патологий зеркально расположенных внутренних органов рекомендуют КТ брюшной полости.</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;\">Консервативная терапия</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;\">Аномальное расположение сердца без дополнительных заболеваний не сопровождается расстройствами гемодинамики и не требует лечения. При развитии сердечно-сосудистых заболеваний терапию проводят так же, как и для больных с отсутствием декстрокардии. При сочетании аномалии с синдромом Картагенера назначается симптоматическая терапия, предполагающая прием ингаляционных и топических назальных кортикостероидов, бета-адреномиметиков.</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;\">Хирургическое лечение</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-style: normal; font-variant-numeric: normal; font-variant-east-asian: normal; vertical-align: baseline; white-space: pre-wrap;\">Оперативное вмешательство показано при сочетанных пороках развития сердца, транспозиции магистральных сосудов. Срок выполнения операции устанавливают с учетом вида порока и тяжести нарушений гемодинамики. Если угрожающие жизни состояния отсутствуют, хирургическую коррекцию откладывают до 3-6-летнего возраста ребенка. Когда декстрокардия сочетается с тяжелыми пороками (например, тетрадой Фалло), вмешательство проводят в максимально ранние сроки.</span></p>\r\n<p><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>",
"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;\">Профилактические мероприятия направлены на устранение тератогенных факторов, медико-генетическое консультирование беременных.</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;\">Изолированная форма, которая не сочетается с другими сердечными аномалиями, не имеет клинических проявлений. Специфические жалобы обычно отсутствуют. Состояние обнаруживается в детском или взрослом возрасте при проведении профилактических осмотров, обращении за врачебной помощью по другой причине. Люди с декстрокардией чаще других страдают болезнями нижних дыхательных путей: трахеитами, бронхитами.</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;\">Клинические симптомы возникают при наличии других аномалий висцеральных органов или возникновении на фоне декстрокардии кардиальной патологии. Пациенты ощущают постоянную слабость и повышенную утомляемость, не проходящую после сна. Их периодически беспокоят приступы учащенного сердцебиения, головокружения. Кожные покровы становятся бледными, иногда возникает синеватый оттенок кожи вокруг рта, на пальцах рук.</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;\">Сочетание декстрокардии и врожденных сердечных пороков проявляется яркой клинической картиной уже в раннем детском возрасте. Для «белых» пороков характерна постоянная бледность кожи, при «синих» формах наблюдаются акроцианоз или тотальный цианоз. Появляются одышечно-цианотические приступы: ребенок становится беспокойным, кожа холодная, дыхание хриплое и прерывистое.</span></p>",
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