20例溶血尿毒症综合征病因和临床特征

目的 分析溶血尿毒症综合征（HUS）病因和临床特点，探讨改善预后的方法。 方法 收集我科1998年7月至2004年12月20例HUS患者的临床资料，分析病因、临床表现、个体化治疗方案和预后。 结果 20例HUS占同期急性肾衰竭（ARF）的2.48%（18/726），女性16例，男性4例，平均年龄（49.11±19.85）岁。5例为特发性HUS，其余15例为继发性，包括狼疮肾炎10例，产后2例，抗磷脂综合征（APS）、恶性肾小动脉硬化、药物性各1例。表现为ARF 18例，肾病综合征15例, 高血压17例 (恶性高血压4例)。12例患者为肉眼血尿或酱油色尿，余8例均为镜下血尿, 仅1例腹泻。20例HUS发病时Scr为（504.40±381.10） μmol/L，尿蛋白量（24 h）（5.0±2.6） g 。16例接受肾活检。14例接受血液净化治疗，包括2例血浆置换、8例血浆置换+连续性血液透析滤过或单纯血透、4例连续性血液透析滤过+单纯血透。7例采用静脉丙种球蛋白。对继发性HUS的原发病加用皮质激素和免疫抑制剂治疗；对特发性HUS以中、小剂量皮质激素治疗；抗磷脂综合征以低分子量肝素抗凝。1例失随访，其余19例平均随访（46.0±32.8）月，其中4例死亡；11例治愈且肾功能恢复正常；4例肾功能未恢复，其中2例维持透析。系统性红斑狼疮（SLE）和非SLE合并HUS的存活比例分别为70%和90%；肾存活比例为50%和60%，差异均无统计学意义。 结论 本组HUS以继发性为主，以SLE占首位，SLE并发HUS的临床表现危重，预后较差。本组HUS存活比例为80%，肾脏治愈比例为55%。血浆置换和静脉注射丙种球蛋白为主要治疗方法。小剂量皮质激素治疗能减少复发。对有治疗指征的原发病，免疫抑制剂能改善预后。

Causes and clinical features of 20 patients with hemolytic uremic syndrome

Objective To analyze the causes and clinical features of 20 patients with hemolytic uremic syndrome (HUS) in order to improve the prognosis. Methods Twenty patients with HUS hospitalized in our department during July 1998 to December 2004 were enrolled in this study. The etiology, clinical features, individualized therapy and prognosis were retrospectively analyzed. Results These 20 HUS patients (18 HUS patients complicated with ARF) accounted for 2.48% of total patients with acute renal failure (ARF) in our hospital. There were 16 females and 4 males with mean age of (49.11±19.85) years. Five patients were idiopathic HUS and the other 15 were secondary HUS (10 SLE-associated HUS, 2 pregnancy-associated HUS, 1 APS-associated HUS, 1 renal arterioles sclerosis-associated HUS and 1 drug-associated HUS). Eighteen cases had ARF and 15 had nephrotic syndrome. Hypertension was found in 17 patients, among them 4 had malignant hypertension. Twelve patients had gross hematuria and the other 8 had microscopic hematuria. Diarrhea was found only in 1 patient. At onset, mean serum creatinine was (504.40±381.10) μmol/L and 24-h proteinuria was (5.0±2.6) g. Renal biopsy was pedormed in 16 patients. Fourteen patients received hemopurification therapy: 2 patients plasma exchange (PE); 8 patients PE combined with CVVHDF and /or HD; 4 patients CVVHDF and HD. Seven cases were treated with intravenous immunoglobulin (IVIg). Patients with SLE-associated HUS received the corticosteroids and immunosuppressants. Low or middle dosage of corticosteroids( 10-40 mg/d) was administered in patients with idiopathic HUS. For patients with APS, low molecular weight heparin was used. HUS patients were followed-up for average (46.0±32.8) months. During follow-up, 4 patients died, 11 recovered from renal insufficiency, 4 progressed to end stage renal failure of whom 2 depended on dialysis and 1 lost. The survival rates of SLE-associated HUS and none-SLE-associated HUS were 70% and 90%, and renal survival rates were 50% and 60% respectively, which were not significantly different between these two groups. Conclusions Most of the patients are secondary HUS. SLE-associated HUS is the main type of secondary HUS. The prognosis of SLE-associated HUS is poor. PE and IVIg are main therapy. Low dosage of corticosteroids can reduce relapse of HUS. Immunosuppressants can improve the prognosis.