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Меня постоянно волновало бцж, но ответы врачей были - заживет; никто не будет делать ринактивацию; чем страшнее тем лучше, формируется иммунитет. Особенное «спасибо» хочу сказать врачу иммунологу-аллергологу-педиатру с вольской 6, Рамазановой Ю.В., как иммунолог может пропустить и закрыть глаза на все как мы узнали симптомы?! На тот момент доченьке был 1 год и месяц, когда мы попали к ней, и если б она была квалифицорованый врач, то хотя бы предположила и дала направление на анализ(иммунограмму). У нас бы было больше шансов чтоб для того чтоб выжить и провести операцию по пересадке костного мозга. Но .... увы узнали мы об этом диагнозе поздно. По поводу стационара Вольской 6, хочу отметить очень безобразное отношение, долго рассказывать, перейду к сути. Пролечившись в инфекционной больнице по кишечной инфекции и пневмонии, нас выписали(сама настояла), нужно было доп. обследование и мы поехали на Вольскую 6, мало того что меня пытались с ребёнком грубо говоря выгнать, в итоге 5 часов держали в холле с больными детьми, решая в какое отделение нас положить. Бог знает что мы без иммунитета могли там нацеплять(но на тот момент о диагнозе ТКИН мы не знали), вечером уже пошли симптомы ротовирусной инфекции, на утро подтвердили что у дочки ротовирус и нас отправляют обратно в инфекцию, ее капали всю ночь, она была в тяжелом состоянии, перед отправкой температура поднялась 39,9...и это никого не остановило, меня попросили быстро собрать вещи и одеть малышку, тут же приехала скорая и нас забрали. Вот такие прекрасные врачи!!!!! В итоге доченька умерла в Рогачевской больнице из-за того что поздно приехали! 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Характеризуется нарушением работы одного или нескольких компонентов иммунной системы [2](https://klinikarassvet.ru/patients/zabolevanija/pervichnyy-immunodefitsit/). Такие нарушения приводят к повышенной уязвимости организма к патогенам, выражающейся в частых и рецидивирующих инфекционных заболеваниях, развитии хронических и системных болезней (аутоиммунных, онкологических) [2](https://klinikarassvet.ru/patients/zabolevanija/pervichnyy-immunodefitsit/). \n\nПервичные иммунодефициты проявляются в раннем возрасте и требуют поддерживающей терапии на протяжении всей жизни [3](https://yandex.ru/medicine/disease/pervichnye-immunodefitsity)[4](https://www.mechnikoff.ru/novosti-i-stati/chto-takoe-sindrom-immunodefitsita-i-kak-ego-lechit/).\n\n\n\n## Причины\nБольшинство случаев ПИД имеют **наследственный характер** — мутации передаются от родителей к детям [7](https://nadejdamed.ru/blog/pervichnyy-immunodeficit/). Однако иногда мутации возникают спонтанно, без семейного анамнеза [7](https://nadejdamed.ru/blog/pervichnyy-immunodeficit/). Это может произойти из-за ошибок при копировании ДНК во время деления клеток или под воздействием внешних факторов, таких как радиация или химические вещества [7](https://nadejdamed.ru/blog/pervichnyy-immunodeficit/). \n\nНекоторые группы первичных иммунодефицитов: \n* **Дефициты клеточного иммунитета** — состояния, обусловленные недостаточной активностью или низким уровнем Т-лимфоцитов [3](https://yandex.ru/medicine/disease/pervichnye-immunodefitsity)[9](https://www.KrasotaiMedicina.ru/diseases/immune/primary-immunodeficiency). \n* **Дефициты гуморального иммунитета** — группа состояний, при которых понижена функция преимущественно В-лимфоцитов, нарушен синтез иммуноглобулинов [3](https://yandex.ru/medicine/disease/pervichnye-immunodefitsity)[9](https://www.KrasotaiMedicina.ru/diseases/immune/primary-immunodeficiency). \n* **Комбинированные первичные иммунодефициты** — обширная группа заболеваний с пониженной активностью как клеточных, так и гуморальных звеньев иммунитета [3](https://yandex.ru/medicine/disease/pervichnye-immunodefitsity)[9](https://www.KrasotaiMedicina.ru/diseases/immune/primary-immunodeficiency).\n\n## Симптомы\nПроявления ПИД разнообразны, но есть ряд общих признаков, характерных для большинства патологий из этой группы [12](https://MedAboutMe.ru/zdorove/spravochnik/bolezni/pervichnye_immunodefitsity/)[13](https://www.invitro.ru/library/bolezni/35529/). Некоторые из них: \n* повышенная восприимчивость к вирусным и грибковым инфекциям [12](https://MedAboutMe.ru/zdorove/spravochnik/bolezni/pervichnye_immunodefitsity/)[13](https://www.invitro.ru/library/bolezni/35529/); \n* рецидивирующие гнойничковые поражения кожи [12](https://MedAboutMe.ru/zdorove/spravochnik/bolezni/pervichnye_immunodefitsity/)[13](https://www.invitro.ru/library/bolezni/35529/); \n* частые осложнённые бактериальные инфекции [13](https://www.invitro.ru/library/bolezni/35529/); \n* патологические разрастания лимфоидной ткани [13](https://www.invitro.ru/library/bolezni/35529/); \n* стойкая диарея [13](https://www.invitro.ru/library/bolezni/35529/); \n* длительное повышение температуры тела [13](https://www.invitro.ru/library/bolezni/35529/); \n* гранулематозные (узелковые) изменения в органах и тканях [13](https://www.invitro.ru/library/bolezni/35529/).\n\n## Диагностика\nДиагностикой ПИД занимается иммунолог [7](https://nadejdamed.ru/blog/pervichnyy-immunodeficit/). Для диагностики используют: \n* **Лабораторные методы**: анализ крови, тесты на функциональность иммунных клеток, генетические тесты [7](https://nadejdamed.ru/blog/pervichnyy-immunodeficit/). \n* **Инструментальную диагностику**: рентгенографию и КТ — для оценки состояния органов и тканей, ультразвуковое исследование — для обнаружения аномалий в лимфатической системе и других органах [7](https://nadejdamed.ru/blog/pervichnyy-immunodeficit/).\n\n## Лечение\nЕдиных принципов лечения ПИД не существует, поскольку причины и механизмы их развития отличаются [3](https://yandex.ru/medicine/disease/pervichnye-immunodefitsity). Некоторые направления терапии: \n* **Профилактика и контроль рецидивирующих инфекций** — назначается курс антибиотикотерапии (только инфекции, вызванные бактериями или грибками), прицельная противовирусная терапия и симптоматическая терапия [2](https://klinikarassvet.ru/patients/zabolevanija/pervichnyy-immunodefitsit/). \n* **Коррекция иммунитета** — могут назначаться иммуноглобулинотерапия (восполнение дефицита конкретных антител), интерферон-гамма-терапия, терапия факторами роста (при ПИД, вызванном недостатком лейкоцитов) [2](https://klinikarassvet.ru/patients/zabolevanija/pervichnyy-immunodefitsit/). \n* **В тяжёлых случаях** — трансплантация костного мозга, которая позволяет восстановить нормальную функцию иммунной системы [4](https://www.mechnikoff.ru/novosti-i-stati/chto-takoe-sindrom-immunodefitsita-i-kak-ego-lechit/). \n\n**Самолечение** при ПИД не рекомендуется — оно может усугубить течение заболевания [4](https://www.mechnikoff.ru/novosti-i-stati/chto-takoe-sindrom-immunodefitsita-i-kak-ego-lechit/).","linksData":[{"sourceId":1,"url":"https://polyclinika.ru/tech/immunodefitsit-prichiny-simptomy-tipy-lechenie/","shownUrl":"https://polyclinika.ru/tech/immunodefitsit-prichiny-simptomy-tipy-lechenie/","title":"Иммунодефицит - причины, как передается, симптомы, признаки, типы, диагностика, лечение, профилактика","shortUrl":"polyclinika.ru","isTranslated":false,"description":"","greenUrl":"polyclinika.ru"},{"sourceId":2,"url":"https://klinikarassvet.ru/patients/zabolevanija/pervichnyy-immunodefitsit/","shownUrl":"https://klinikarassvet.ru/patients/zabolevanija/pervichnyy-immunodefitsit/","title":"Первичный иммунодефицит: симптомы и лечение | Клиника Рассвет","shortUrl":"klinikarassvet.ru","isTranslated":false,"description":"","greenUrl":"klinikarassvet.ru"},{"sourceId":3,"url":"https://yandex.ru/medicine/disease/pervichnye-immunodefitsity","shownUrl":"https://yandex.ru/medicine/disease/pervichnye-immunodefitsity","title":"Первичные иммунодефициты - симптомы, причины и лечение","shortUrl":"yandex.ru","isTranslated":false,"description":"","greenUrl":"yandex.ru"},{"sourceId":4,"url":"https://www.mechnikoff.ru/novosti-i-stati/chto-takoe-sindrom-immunodefitsita-i-kak-ego-lechit/","shownUrl":"https://www.mechnikoff.ru/novosti-i-stati/chto-takoe-sindrom-immunodefitsita-i-kak-ego-lechit/","title":"Синдром иммунодефицита: причины, симптомы и лечение","shortUrl":"mechnikoff.ru","isTranslated":false,"description":"","greenUrl":"mechnikoff.ru"},{"sourceId":5,"url":"https://gemotest.ru/info/spravochnik/zabolevaniya/pervichnyy-immunodefitsit/","shownUrl":"https://gemotest.ru/info/spravochnik/zabolevaniya/pervichnyy-immunodefitsit/","title":"Первичный иммунодефицит — что это, признаки у детей и взрослых, классификация, лечение","shortUrl":"gemotest.ru","isTranslated":false,"description":"","greenUrl":"gemotest.ru"},{"sourceId":6,"url":"https://korilip-altpharm.ru/articles/immunodeficit/","shownUrl":"https://korilip-altpharm.ru/articles/immunodeficit/","title":"Иммунодефицит - причины, симптомы и лечение","shortUrl":"korilip-altpharm.ru","isTranslated":false,"description":"","greenUrl":"korilip-altpharm.ru"},{"sourceId":7,"url":"https://nadejdamed.ru/blog/pervichnyy-immunodeficit/","shownUrl":"https://nadejdamed.ru/blog/pervichnyy-immunodeficit/","title":"Иммунодефицит: симптомы, причины и диагностика — Блог \"Надежда\"","shortUrl":"nadejdamed.ru","isTranslated":false,"description":"","greenUrl":"nadejdamed.ru"},{"sourceId":8,"url":"https://detskiy-medcentr-spb.ru/disease/immunodefitsit/","shownUrl":"https://detskiy-medcentr-spb.ru/disease/immunodefitsit/","title":"Иммунодефицит у детей и новорожденных: что такое, симптомы, признаки, как определить","shortUrl":"detskiy-medcentr-spb.ru","isTranslated":false,"description":"","greenUrl":"detskiy-medcentr-spb.ru"},{"sourceId":9,"url":"https://www.KrasotaiMedicina.ru/diseases/immune/primary-immunodeficiency","shownUrl":"https://www.KrasotaiMedicina.ru/diseases/immune/primary-immunodeficiency","title":"Первичные иммунодефициты - причины, симптомы, диагностика и лечение","shortUrl":"KrasotaiMedicina.ru","isTranslated":false,"description":"","greenUrl":"KrasotaiMedicina.ru"},{"sourceId":10,"url":"https://ru.wikipedia.org/wiki/%D0%98%D0%BC%D0%BC%D1%83%D0%BD%D0%BE%D0%B4%D0%B5%D1%84%D0%B8%D1%86%D0%B8%D1%82","shownUrl":"https://ru.wikipedia.org/wiki/%D0%98%D0%BC%D0%BC%D1%83%D0%BD%D0%BE%D0%B4%D0%B5%D1%84%D0%B8%D1%86%D0%B8%D1%82","title":"Иммунодефицит — Википедия","shortUrl":"ru.wikipedia.org","isTranslated":false,"description":"","greenUrl":"ru.wikipedia.org"},{"sourceId":11,"url":"https://ProBolezny.ru/pervichnyj-immunodeficit/","shownUrl":"https://ProBolezny.ru/pervichnyj-immunodeficit/","title":"Первичный иммунодефицит: причины, симптомы и лечение в статье аллерголога Сараева Ю. 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