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危及生命的妊娠相关非典型溶血性尿毒症综合征及其对依库珠单抗的反应。

Life-threatening pregnancy-associated atypical haemolytic uraemic syndrome and its response to eculizumab.

作者信息

Gately Ryan, San Aye, Kurtkoti Jagadeesh, Parnham Alan

机构信息

Nephrology Department, Gold Coast University Hospital, Southport, Australia.

出版信息

Nephrology (Carlton). 2017 Feb;22 Suppl 1:32-35. doi: 10.1111/nep.12938.

Abstract

Pregnancy-associated atypical haemolytic uraemic syndrome (P-aHUS) is a rare, potentially lethal condition that can complicate pregnancy in up to 1 in 25 000 cases. Without prompt diagnosis and initiation of appropriate treatment, this condition can lead to disastrous consequences for both mother and child. Given the broad spectrum of conditions that can present similarly in the peripartum period, it is often difficult to establish the correct diagnosis in a timely manner. Recently, the terminal complement cascade inhibitor eculizumab has been used with considerable success in non-pregnancy HUS; however, its use in P-aHUS is limited to isolated case reports. Here, we present a case of fulminant P-aHUS in the postpartum period that was successfully treated with eculizumab resulting in significant recovery of renal function.

摘要

妊娠相关非典型溶血尿毒综合征(P-aHUS)是一种罕见的、潜在致命的疾病,每25000例妊娠中可能有1例并发该疾病。如果不及时诊断并开始适当治疗,这种疾病会给母婴带来灾难性后果。鉴于围产期可能出现多种表现相似的疾病,往往难以及时做出正确诊断。最近,终末补体级联抑制剂依库珠单抗在非妊娠型溶血尿毒综合征的治疗中取得了显著成功;然而,其在P-aHUS中的应用仅限于个别病例报告。在此,我们报告1例产后暴发性P-aHUS病例,该病例经依库珠单抗成功治疗后肾功能显著恢复。

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