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

机译:妊娠诱导的非典型溶血性尿毒症综合征:新时代用生态蛋白

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摘要

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.
机译:怀孕是一种公认​​的非典型溶解综合征(P-Ahus)的触发器,并且经常发生在Partum期间。类似于非典型溶血性尿毒症综合征,它具有较差的预后,具有较高的发病率,特别是肾功能衰竭的形式。早期识别和干预在其管理中至关重要,特别是由于近期生态蛋白可用,一种人源化单克隆抗体,以补充组分C5,这表明了预后的剧烈改善。然而,对于P-Ahus的临床和生化表现形式,HellP综合征(溶血酶和低血小板计数低)和其他高血压障碍的临床和生化表现相当多的重叠进行了及时诊断,鉴于相当数量的诊断。我们提出了一个案例报告和文献综述,突出了到达诊断的临床难题。我们还强调了早期管理P-Ahus与生态灭绝的重要性及其对提高发病率的影响。

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