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比较妊娠急性脂肪肝与溶血、肝酶升高和血小板减少综合征。

Comparing acute fatty liver of pregnancy from hemolysis, elevated liver enzymes, and low platelets syndrome.

作者信息

Byrne John J, Seasely Angela, Nelson David B, Mcintire Donald D, Cunningham F Gary

机构信息

Department of Obstetrics and Gynecology, University of Texas, Southwestern Medical Center, Parkland Health and Hospital Systems, Dallas, TX, USA.

出版信息

J Matern Fetal Neonatal Med. 2022 Apr;35(7):1352-1362. doi: 10.1080/14767058.2020.1754790. Epub 2020 Apr 19.

Abstract

Acute fatty liver of pregnancy (AFLP) and hemolysis, elevated liver enzymes, and low platelets (HELLP) syndrome are both associated with significant maternal and perinatal morbidity and mortality. Because of the overlap of several clinical and laboratory findings differentiation can sometimes be difficult. Both disorders have been of interest for more than 100 years, however they were not completely characterized until the early 1980s. It was not until the 1980s that AFLP and HELLP syndrome, and more specifically their clinical, laboratory, and pathologic findings, were further differentiated in the literature. More recently, the pathophysiologic mechanisms have been elucidated. In this review, we outline the similarities and differences in the clinical presentation, laboratory findings, maternal and perinatal outcomes, and clinical recovery for women diagnosed with these two syndromes. From our observations, we suggest that levels of fibrinogen, creatinine, cholesterol, and total bilirubin be used to assist with differentiating AFLP from HELLP syndrome upon admission in women presenting with either suspected disease. The rationale for identifying the specific conditions is that clinical consequences for recovery vary considerably. Specifically, AFLP is associated with significantly more hepatic and renal dysfunction as well as coagulopathy. Fortunately, both conditions can be managed with supportive measures with overall improved perinatal outcomes including morbidity and mortality.

摘要

妊娠急性脂肪肝(AFLP)和溶血、肝酶升高及血小板减少(HELLP)综合征均与孕产妇及围产期的高发病率和死亡率相关。由于一些临床和实验室检查结果存在重叠,有时难以进行鉴别。这两种病症已被关注超过100年,但直到20世纪80年代初才得到全面描述。直到20世纪80年代,AFLP和HELLP综合征,尤其是它们的临床、实验室及病理表现,才在文献中得到进一步区分。最近,其病理生理机制已被阐明。在本综述中,我们概述了诊断为这两种综合征的女性在临床表现、实验室检查结果、孕产妇及围产期结局以及临床恢复方面的异同。根据我们的观察,我们建议在疑似患有这两种疾病的女性入院时,使用纤维蛋白原、肌酐、胆固醇和总胆红素水平来辅助鉴别AFLP和HELLP综合征。明确具体病症的理由是,恢复的临床后果差异很大。具体而言,AFLP与更严重的肝肾功能障碍以及凝血病相关。幸运的是,通过支持性措施可以对这两种病症进行管理,从而总体改善包括发病率和死亡率在内的围产期结局。

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