Shanmugalingam Renuka, Hsu Danny, Makris Angela
Department of Renal Medicine, Liverpool Hospital, NSW, Australia.
School of Medicine, Western Sydney University, NSW, Australia.
Obstet Med. 2018 Mar;11(1):28-31. doi: 10.1177/1753495X17704563. Epub 2017 May 18.
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.
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。
