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高危妊娠合并线粒体脑肌病伴乳酸血症和卒中样发作(MELAS综合征)的麻醉管理:一例报告

Anesthetic Management of Mitochondrial Encephalopathy With Lactic Acidosis and Stroke-Like Episodes (MELAS Syndrome) in a High-Risk Pregnancy: A Case Report.

作者信息

Bell Josh D, Higgie Kushlin, Joshi Mital, Rucker Joshua, Farzi Sahar, Siddiqui Naveed

机构信息

From the *Department of Anesthesiology, University of Toronto; and †Department of Anesthesia and Pain Management, Mount Sinai Hospital, Toronto, Ontario, Canada.

出版信息

A A Case Rep. 2017 Jul 15;9(2):38-41. doi: 10.1213/XAA.0000000000000520.

Abstract

MELAS syndrome (mitochondrial encephalopathy, lactic acidosis, and stroke-like symptoms) is a rare and complex mitochondrial disorder. We present the in-hospital course of a 36-year-old gravida 2, para 0 with MELAS syndrome and severe preeclampsia, complicated by hyponatremia, hyperkalemia, and diabetes. A retained placenta with postpartum hemorrhage required urgent instrumental delivery under spinal anesthesia, transfusion, and intensive care unit admission for pulmonary edema, effusions, and atelectasis. Postpartum endometritis and sepsis also were encountered. This is to our knowledge the first case report of obstetric complications in MELAS syndrome and highlights the salient metabolic sequelae of this syndrome.

摘要

线粒体脑肌病伴乳酸酸中毒及卒中样发作综合征(MELAS)是一种罕见且复杂的线粒体疾病。我们报告了一名36岁、孕2产0、患有MELAS综合征及重度子痫前期的患者的住院病程,该患者还并发低钠血症、高钾血症及糖尿病。胎盘滞留伴产后出血需要在脊髓麻醉下紧急器械助产,输血,并因肺水肿、胸腔积液和肺不张入住重症监护病房。还出现了产后子宫内膜炎和败血症。据我们所知,这是首例关于MELAS综合征产科并发症的病例报告,并突出了该综合征显著的代谢后遗症。

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