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再探HELLP综合征。

Revisiting HELLP syndrome.

作者信息

Dusse Luci Maria, Alpoim Patrícia Nessralla, Silva Juliano Teixeira, Rios Danyelle Romana Alves, Brandão Augusto Henriques, Cabral Antônio Carlos Vieira

机构信息

Faculdade de Farmácia, Universidade Federal de Minas Gerais, Brazil.

Faculdade de Farmácia, Universidade Federal de Minas Gerais, Brazil.

出版信息

Clin Chim Acta. 2015 Dec 7;451(Pt B):117-20. doi: 10.1016/j.cca.2015.10.024. Epub 2015 Oct 23.

Abstract

HELLP syndrome was first described in 1982 by Weinstein et al. and the term HELLP refers to an acronym used to describe the clinical condition that leads to hemolysis, elevated liver enzymes and low platelets. The syndrome frequency varies from 0.5 to 0.9% pregnancies and manifests preferentially between the 27th and 37th week of gestation. Approximately 30% of cases occur after delivery. Although the etiopathogenesis of this syndrome remains unclear, histopathologic findings in the liver include intravascular fibrin deposits that presumably may lead to hepatic sinusoidal obstruction, intrahepatic vascular congestion, and increased intrahepatic pressure with ensuing hepatic necrosis, intraparenchymal and subcapsular hemorrhage, and eventually capsular rupture. Typical clinical symptoms of HELLP syndrome are pain in the right upper quadrant abdomen or epigastric pain, nausea and vomiting. However, this syndrome can present nonspecific symptoms and the diagnosis may be difficult to be established. Laboratory tests and imaging exams are essential for differential diagnosis with other clinical conditions. Treatment of HELLP syndrome with corticosteroids, targeting both lung maturation of the fetus is still an uncertain clinical value. In conclusion, three decades after the tireless efforts of Dr. Weinstein to characterize HELLP syndrome, it remains a challenge to the scientific community and several questions need to be answered for the benefit of pregnant women.

摘要

HELLP综合征于1982年由温斯坦等人首次描述,HELLP这个术语是一个首字母缩略词,用于描述导致溶血、肝酶升高和血小板减少的临床病症。该综合征在妊娠中的发生率为0.5%至0.9%,且优先在妊娠第27周至37周之间出现。约30%的病例发生在分娩后。尽管该综合征的病因仍不清楚,但肝脏的组织病理学发现包括血管内纤维蛋白沉积,这可能会导致肝窦阻塞、肝内血管充血以及肝内压力升高,继而出现肝坏死、实质内和包膜下出血,最终导致包膜破裂。HELLP综合征的典型临床症状是右上腹疼痛或上腹部疼痛、恶心和呕吐。然而,该综合征可能表现出非特异性症状,诊断可能难以确立。实验室检查和影像学检查对于与其他临床病症进行鉴别诊断至关重要。使用皮质类固醇治疗HELLP综合征,同时针对胎儿肺成熟,其临床价值仍不确定。总之,在温斯坦博士为描述HELLP综合征付出不懈努力的三十年后,它仍然是科学界面临的一个挑战,为了孕妇的利益,仍有几个问题需要解答。

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