Objective To explore the clinical features and therapeutic strategy for adult with atypical hemolytic uremic syndrome (Ahus). Methods A retrospective analysis of clinical features, therapeutic strategy and outcome was made for 5 adult suffering from Ahus in our hospital from 2005 to 2011. Results Among the five adults with Ahus, 2 cases were cured, 1 case died and 2 cases had renal insufficiency, of which, one was mild and the other was developing into ESRD relying on dialysis ultimately. 3 cases were associated to pregnancy. The key points in the treatment and nursing for Ahus case were early diagnosis and managemant with plasma exchange. The efficiency of glucocorticoid was not clearly. The severity of renal pathological involvement, especially the degree of vascular damage, was a good predictor of long-term outcome of patient with atypical HUS. Conclusion Ahus is caused by complicated factors and has a poor prognosis. Early diagnosis and proper treatments are the key factors to improve prognosis.%目的 探讨成人不典型溶血尿毒综合征的临床特点及诊治经验.方法 回顾性分析2005～2011年我院诊断成人不典型溶血尿毒综合征患者共5例,总结分析其临床表现、辅助检查、治疗方法及疗效.结果 5例患者中,治愈2例,死亡1例,遗留肾功能不全2例,其中1例最终走向透析,1例肾功能长期保持稳定.其中,与妊娠相关者共3例.早期血浆置换可有效控制病情,改善预后,糖皮质激素的效果尚不确定.肾脏病理改变是估计预后的重要指标.以小动脉改变为主的预后差.结论 成人不典型溶血尿毒综合征病因多样、预后较差.早期诊断、早期治疗是改善预后的关键因素.
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