妊娠相关非典型溶血性尿毒症综合征1例报告及文献复习

Y. Chen, Qin Xue, N. Wang
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引用次数: 0

摘要

非典型溶血性尿毒症综合征(aHUS)是一种罕见的替代途径补体激活不受控制的疾病,妊娠相关的aHUS (P-aHUS)是一种更罕见但危及生命的疾病。除基因筛选外,ADAMTS-13检测和肾活检有助于肾脏病变的诊断和预后评估。除了治疗性血浆交换和血浆输注外,肝肾联合移植和eculizumab治疗通常是该疾病治疗的初始方法。尽管移植和eculizumab疗法可能比传统疗法更有效,但它要昂贵得多,而且不易获得。在此,我们报告了一例P-aHUS,我们的病例表明血浆置换和血液透析可能对那些不适合/无法进行埃珠单抗治疗或移植的P-aHUS有效。
本文章由计算机程序翻译,如有差异,请以英文原文为准。
Pregnancy-associated atypical hemolytic uremic syndrome: A case report and literature review
Atypical hemolytic uremic syndrome (aHUS) is a rare disease of uncontrolled alternative pathway complement activation and pregnancy-associated aHUS (P-aHUS) is a more rare but life-threatening disorder. Besides gene screening, ADAMTS-13 detection and renal biopsy is helpful to establish diagnosis and prognosis assessment when kidney is involved. Combined liver-kidney transplant and eculizumab therapy has generally been the initial approach to this disease management besides therapeutic plasma exchange and plasma infusion. Though transplant and eculizumab therapy may be more effective than traditional therapy, it is much more expensive and not easily available. Herein we report a case of P-aHUS and our case indicates that plasma exchange and hemodialysis may be effective to P-aHUS for those not suitable/available for eculizumab treatment or transplant.
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