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妊娠急性脂肪肝与溶血、肝酶升高和血小板减少综合征的鉴别。

Differentiation of acute fatty liver of pregnancy from syndrome of hemolysis, elevated liver enzymes and low platelet counts.

作者信息

Minakami Hisanori, Morikawa Mamoru, Yamada Takahiro, Yamada Takashi, Akaishi Rina, Nishida Ryutaro

机构信息

Department of Obstetrics, Hokkaido University Graduate School of Medicine, Sapporo, Japan.

出版信息

J Obstet Gynaecol Res. 2014 Mar;40(3):641-9. doi: 10.1111/jog.12282. Epub 2014 Jan 15.

Abstract

As proposed criteria (Swansea criteria) for the diagnosis of acute fatty liver of pregnancy (AFLP) do not include antithrombin (AT) activity, diagnosis of AFLP may be delayed. The aim of this review is to underscore problems in the differential diagnosis of AFLP and the syndrome of hemolysis, elevated liver enzymes and low platelet counts (HELLP syndrome) and to facilitate prompt diagnosis of AFLP. Published works dealing with liver dysfunction in pregnancy, HELLP syndrome and AFLP were reviewed. AFLP and HELLP syndrome shared common clinical, laboratory, histological and genetic features, and differential diagnosis between them was often difficult. However, HELLP syndrome was likely to occur in patients with hypertension, but AFLP occurred often in the absence of hypertension. In addition, AFLP was exclusively associated with pregnancy-induced antithrombin deficiency (PIATD). Approximately 50% of patients with AFLP did not have thrombocytopenia at presentation. As the Swansea criteria for AFLP did not include PIATD, diagnosis of AFLP was delayed until manifestation of life-threatening complications; 60% of women were admitted to intensive care and 15% to a specialist liver unit. In conclusion, incorporation of AT activity of less than 65% into the diagnostic criteria for AFLP may facilitate suspicion and prompt diagnosis of AFLP, decrease uncertainty regarding the diagnosis of AFLP, and contribute to better investigation and understanding of the process leading to the development of liver dysfunction.

摘要

由于目前用于诊断妊娠急性脂肪肝(AFLP)的标准(斯旺西标准)未纳入抗凝血酶(AT)活性,AFLP的诊断可能会延迟。本综述的目的是强调AFLP与溶血、肝酶升高和血小板计数降低综合征(HELLP综合征)鉴别诊断中的问题,并促进AFLP的及时诊断。对已发表的关于妊娠肝功能障碍、HELLP综合征和AFLP的研究进行了综述。AFLP和HELLP综合征具有共同的临床、实验室、组织学和遗传学特征,两者之间的鉴别诊断往往很困难。然而,HELLP综合征可能发生在高血压患者中,而AFLP常发生在无高血压的情况下。此外,AFLP仅与妊娠诱导的抗凝血酶缺乏症(PIATD)相关。大约50%的AFLP患者在就诊时没有血小板减少症。由于AFLP的斯旺西标准未包括PIATD,AFLP的诊断一直延迟到出现危及生命的并发症;60%的女性被收入重症监护病房,15%被收入专科肝病科。总之,将AT活性低于65%纳入AFLP的诊断标准可能有助于对AFLP的怀疑和及时诊断,减少AFLP诊断的不确定性,并有助于更好地研究和理解导致肝功能障碍发生的过程。

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