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以多器官功能衰竭为表现的妊娠急性重症脂肪肝:病例系列

Fulminant acute fatty liver of pregnancy presenting with multi-organ failure: A case series.

作者信息

Saran Sai, Misra Saumitra, Siddiqui Suhail Sarwar, Agrawal Avinash, Gurjar Mohan, Patwa Ajay Kumar, Muzaffar Syed Nabeel

机构信息

Department of Critical Care Medicine, King George's Medical University, Lucknow, Uttar Pradesh, India.

Department of Critical Care Medicine, Sanjay Gandhi Postgraduate Institute of Medical Sciences, Lucknow, Uttar Pradesh, India.

出版信息

Int J Crit Illn Inj Sci. 2022 Oct-Dec;12(4):239-243. doi: 10.4103/ijciis.ijciis_31_22. Epub 2022 Dec 26.

Abstract

Liver disease in pregnancy can be classified into pregnancy-related, liver disease coincident with pregnancy or preexisting liver disease. Acute fatty liver of pregnancy (AFLP) is a rare liver disorder that is caused by defects in mitochondrial beta (β) oxidation of fatty acids. In view of its fulminant presentation and rapid progression to multiple organ failure (MOF), AFLP carries high maternal and fetal mortality. These patients are commonly present in the third trimester of pregnancy with gastrointestinal symptoms and complications such as hypoglycemia, lactic acidosis, hyperammonemia, leukocytosis, liver dysfunction, coagulopathy, and renal dysfunction. Diagnosis is mostly based on the Swansea diagnostic criteria and by excluding other etiologies of liver dysfunction. Liver biopsy is rarely performed owing to underlying coagulopathy and thrombocytopenia. In this case series, we intend to share our experience of managing four cases of AFLP that were admitted to the intensive care unit with fetal demise and MOF.

摘要

妊娠期肝病可分为与妊娠相关的肝病、与妊娠同时发生的肝病或既往存在的肝病。妊娠急性脂肪肝(AFLP)是一种罕见的肝脏疾病,由脂肪酸线粒体β(β)氧化缺陷引起。鉴于其暴发性表现和迅速进展为多器官功能衰竭(MOF),AFLP导致母婴死亡率很高。这些患者通常在妊娠晚期出现胃肠道症状以及低血糖、乳酸性酸中毒、高氨血症、白细胞增多、肝功能障碍、凝血障碍和肾功能障碍等并发症。诊断主要基于斯旺西诊断标准并排除肝功能障碍的其他病因。由于存在潜在的凝血障碍和血小板减少症,很少进行肝活检。在本病例系列中,我们打算分享我们管理4例因胎儿死亡和MOF入住重症监护病房的AFLP患者的经验。

https://cdn.ncbi.nlm.nih.gov/pmc/blobs/23b6/9910109/439da6d8714b/IJCIIS-12-239-g001.jpg

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