Abstract:
Pregnancy-associated atypical hemolytic uremic syndrome (P-aHUS) is a rare disease. There are only few reports in the literature, and most are in the puerperium period. It is a thrombotic microangiopathy (TMA) characterized for microangiopathic hemolytic anemia, thrombocytopenia, and renal dysfunction. We report the case of a pregnant patient at 26.3 gestation weeks, who developed clinical features of TMA, neurological alterations, and septic shock; then after fetus and placental delivery, no clinical improvement was observed; a diagnostic protocol was performed due to suspicion of P-aHUS, showing improvement after the plasma exchange sessions and eculizumab. We present here a brief review of the case since it is an entity that needs to be suspected during pregnancy when TMA features and requires an immediate diagnosis to provide timely treatment.
摘要:
妊娠相关非典型溶血性尿毒综合征(P-aHUS)是一种罕见疾病。文献中只有很少的报道,大多数都在产褥期。它是一种血栓性微血管病(TMA),以微血管病性溶血性贫血为特征,血小板减少症,肾功能不全.我们报告了一名妊娠26.3周的孕妇,发展了TMA的临床特征,神经系统改变,和感染性休克;然后在胎儿和胎盘分娩后,未观察到临床改善;由于怀疑P-aHUS而进行了诊断方案,血浆置换和依库珠单抗后显示改善。我们在此简要回顾该病例,因为当TMA特征时,它是需要在怀孕期间怀疑的实体,并且需要立即诊断以提供及时治疗。