【摘要】 目的分析非典型溶血尿毒症综合征(aHUS)患者的病因、临床特点,探讨改善预后的方法。方法回顾性分析我院2003年1月至2009年12月26例aHUS患者病因、临床特点、治疗和预后。结果 26例aHUS患者中,家族性HUS1例,特发性HUS18例,继发性7例(妊娠相关HUS4例,系统性红斑狼疮1例,恶性高血压1例,外伤后HUS1例)。aHUS前驱症状包括恶心/呕吐(73%)、肉眼血尿(65%)、发热(50%)、皮肤粘膜出血(46%)、上呼吸道感染(35%)、少尿或无尿(31%)、腹痛(31%)等。15例aHUS行血浆置换(PE)治疗,其中6例PE+血液透析(HD),1例行PE+连续性静脉血液滤过(CVVH)+HD;4例单纯HD,1例行HD+CVVH;8例aHUS患者给予血浆输注。aHUS18例纳入预后分析,完全有效14例,部分有效4例;肾脏预后肾功能正常16例,ESRD2例,其中1例肾移植。结论本组aHUS以特发性为主,继发原因有妊娠、系统性红斑狼疮,恶性高血压和外伤,临床表现多样且危重,PE和血浆输注为主要治疗,预后较差。更多还原

【Abstract】 Objective To analyze the etiological and clinical characteristics of 26 patients with atypical hemolytic-uremic syndrome (aHUS) in order to improve prognosis. Methods Twenty-six patients with aHUS in our hospital from January 2003 to December 2009 were enrolled in this study. Etiology,clinical features and therapeutic response were retrospectively analyzed. Results Among the 26 patients,there were 18 patients with idiopathic HUS,1 with familial HUS and 7 with secondary HUS (4 pregnancy-associated HUS,1 SLE-associated HUS,1 malignant hypertension-associated HUS and 1 post-trauma-associated HUS). Prodromes of the patients included nausea and vomiting (73%),gross hematuria (65%),fever (50%),skin or mucosa hemorrhage (46%),upper respiratory infection (35%),oliguresis or anuresis (31%),and abdominal pain (31%). 15 patients were treated with plasma exchange(PE),among them 6 with PE and HD,and 1 with PE plus CVVH and HD. 4 patients were treated with HD alone,and 1 patient was treated with HD and CVVH. 8 patients received plasma infusion. Among the 18 patients who were included in the outcome analysis,14 achieved complete response and 4 had partial response; 16 had normal renal function,and 2 progressed to ESRD(end stage renal disease). Conclusions Most of the patients suffered from idiopathic HUS. Secondary causes include pregnancy,SLE,malignant hypertension and trauma. aHUS represents a syndrome with variable and critical clinical manifestations. Main therapies include plasma exchange and plasma infusion,with poor prognosis.更多还原