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妊娠诱发的非典型溶血性尿毒症综合征:依库珠单抗开启的新时代。

Pregnancy-induced atypical haemolytic uremic syndrome: A new era with eculizumab.

作者信息

Shanmugalingam Renuka, Hsu Danny, Makris Angela

机构信息

Department of Renal Medicine, Liverpool Hospital, NSW, Australia.

School of Medicine, Western Sydney University, NSW, Australia.

出版信息

Obstet Med. 2018 Mar;11(1):28-31. doi: 10.1177/1753495X17704563. Epub 2017 May 18.

Abstract

Pregnancy is a well-recognised trigger of atypical haemolytic syndrome (P-aHUS) and often occurs in the post-partum period. Similar to atypical haemolytic uremic syndrome, it carries a poor prognosis with high morbidity particularly in the form of renal failure. Early recognition and intervention is crucial in its management particularly with the recent availability of eculizumab, a humanized monoclonal antibody to complement component C5, which has demonstrated drastic improvement in prognosis. The issue, however, is arriving at a timely diagnosis given the considerable amount of overlap in the clinical and biochemical manifestation of P-aHUS, HELLP syndrome (haemolysis, elevated liver enzyme and low platelet count) and other hypertensive disorders of pregnancy. We present a case report and literature review that highlights the clinical conundrum of arriving at the diagnosis. We also highlight the importance of early management of P-aHUS with eculizumab and its impact on improving morbidity.

摘要

妊娠是公认的非典型溶血性尿毒综合征(P-aHUS)的诱发因素,且常发生在产后阶段。与非典型溶血性尿毒症综合征相似,其预后较差,发病率高,尤其是肾衰竭形式。早期识别和干预对其治疗至关重要,特别是鉴于近期可获得的依库珠单抗,一种针对补体成分C5的人源化单克隆抗体,已证明其可显著改善预后。然而,鉴于P-aHUS、HELLP综合征(溶血、肝酶升高和血小板计数降低)及其他妊娠高血压疾病在临床和生化表现上有相当多的重叠,及时诊断是个问题。我们呈现了一份病例报告和文献综述,突出了诊断方面的临床难题。我们还强调了用依库珠单抗对P-aHUS进行早期治疗的重要性及其对降低发病率的影响。

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