Автор Тема: Общая анестезия ребенку с митохондр. заб.  (Прочитано 4526 раз)

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Оффлайн Syndi

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Добрый день!
Ребенку 5 лет, ЛОР рекомендовал выполнение аденотомии.
Сопутствующий диагноз: Митохондриальная энцефаломиопатия (мутация в гене COX3, кодирующем цитохромоксидазу четвертого комплекса дыхательной цепи митохондрий).
Первые проявления заболевания у ребенка около 2-х лет назад, около года назад нарастание клиники, диагноз уточнен в феврале 2014, назначено лечение (постоянный прием L-карнитина, курсовой прием Q10, витаминов группы В). На фоне лечения уменьшение выраженности проявлений, снижение частоты эпизодов "обострения" заболевания.
Жалобы в настоящее время: повышенная утомляемость; трудности при подъеме тела из положения лежа на спине (может встать, только перевернувшись на живот); различные по длительности и частоте эпизоды (провоцируемые чаще всего ОРВИ или переутомлением или стрессом, иногда возникающие без видимой причины): одно- или двухсторонний птоз, нечеткая "смазанная" речь, выраженное усиление утомляемости.
В неврологическом статусе умеренное диффузное снижение мышечного тонуса.
На ЭМГ умеренно выраженные первично-мышечные изменения. КФК, ЛДГ - незначительное околограничное повышение.

Возможно ли выполнение этому ребенку аденотомии в условиях общей анестезии (с учетом представленной информации)? Есть ли какие-то особенности анестезии и периода после нее у ребенка с данным диагнозом? Нужны ли какие-то дополнительные исследования/заключения специалистов для допуска к операции в условиях общей анестезии (кроме того, что требуется всем пациентам)?


Оффлайн rudenkogo

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Re: Общая анестезия ребенку с митохондр. заб.
« Ответ #1 : 11 Октябрь 2014, 22:09:39 »
"Именное" название у вашего синдрома есть? Или по международной классификации: MELAS; NARP; MILS? Сравнивать все генетические дефекты в дыхательной цепи по всем митохондриальным болезням очень не продуктивно и долго. Что еще в клинике: слепота, снижение слуха, судороги, эндокринные нарушения, кардиомиопатия?
 Операция в целом возможна.
Требуется индивидуальный подход и обсуждение всех нюансов. Однако операцию в амбулаторном режиме выполнить (прооперироваться и выписаться в один день) не удасться. 


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Vigo, Aidan, Irmak and Curtis Italy
« Ответ #2 : 05 Июль 2017, 14:24:56 »
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Bandaro, Vasco, Bradley and Tyler Russian federation
« Ответ #3 : 05 Июль 2017, 15:55:27 »
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