成人非典型溶血尿毒综合征1例诊治分析并文献复习  

Diagnosis and treatment of atypical hemolytic uremic syndrome:a case report and literature review

作　　者：雷海燕 陈雅波 兰成佃 刘瑶 Lei Haiyan;Chen Yabo;Lan Chengdian;Liu Yao(Department of General Practice Medicine,Xiamen Branch,Zhongshan Hospital,Fudan University,Xiamen 361015,China;Department of General Practice,Zhongshan Hospital,FudanUniveversity,Shanghai 200032,China)

机构地区：[1]复旦大学附属中山医院厦门医院全科医学科,厦门361015 [2]复旦大学附属中山医院全科医学科,上海200032

出　　处：《中华全科医师杂志》2024年第7期734-737,共4页Chinese Journal of General Practitioners

摘　　要：回顾分析复旦大学附属中山医院厦门医院收治的1例成人非典型溶血尿毒综合征(aHUS)患者的诊治经过,并进行文献复习。本例患者以“腹胀、腹泻、水肿、少尿”起病,迅速进展至急性肾功能衰竭,完善相关检查明确诊断为aHUS,及时行血液透析、输注血浆、免疫球蛋白、血浆置换等治疗后血液学和肾功能指标改善;后患者1年内出现多次aHUS复发,予规律使用依库珠单抗治疗后获得长期缓解。检索万方数据知识服务平台和中国知网数据库自2000年1月至2024年1月研究对象为非妊娠且符合aHUS诊断的文献4篇,结合本例共65例患者,男女比例为1.2∶1,年龄20~86岁,平均34.8岁;常见的临床表现有发热(24例,36.9%)、肾功能不全(27例,41.5%)、神经系统症状(15例,23.1%)。40例(61.5%)合并高血压。36例(55.4%)行血液透析治疗,31例(47.7%)行血浆置换或血浆输注,29例(44.6%)使用糖皮质激素治疗,3例(4.6%)使用依库珠单抗治疗。65例患者中,19例(29.2%)治疗后肾功能完全恢复,30例(46.2%)发展为终末期肾病,4例(6.2%)死亡。总之,aHUS临床表现多样、涉及多个系统,目前治疗仍以血浆置换为主,早期正确诊断和及时有效的治疗可影响患者预后。A 79-year male presenting with abdominal distension,diarrhea,edema and oliguria,which soon progressed to acute renal failure,was admitted in Xiamen Branch of Zhongshan Hospital.The diagnosis of atypical hemolytic uremic syndrome(aHUS)was confirmed after admission.The hematological parameters and renal function were improved after prompt hemodialysis,plasma exchange,plasma and immunoglobulin transfusion.The patient experienced multiple recurrences of aHUS within 1 year,but achieved long-term remission with regular use of eculizumab.The reported cases of aHUS were searched from Wanfang Data Knowledge Service Platform and CNKI Database from January 2000 to January 2024,and 64 non-pregnant adult aHUS cases were retrieved from literature.The analysis of 65 cases showed a male-to-female ratio was 1.2∶1 and an average age of 34.8 years ranging from 20 to 86 years.Common clinical manifestations included fever(24 cases,36.9%),renal dysfunction(27 cases,41.5%),and neurological symptoms(15 cases,23.1%).Additionally,40 cases(61.5%)had concomitant hypertension.Among the 65 patients,36(55.4%)received hemodialysis,31(47.7%)underwent plasma exchange or plasma transfusion,29(44.6%)were treated with glucocorticoids,and only 3(4.6%)were treated with eculizumab.In terms of treatment outcomes,29.2%(19 cases)of the patients achieved complete renal function recovery,46.2%(30 cases)developed end-stage renal disease,and 4 patients died.aHUS presents with a variety of clinical manifestations involving multiple systems.Plasma exchange remains the primary treatment method at present.Early and accurate diagnosis,prompt and effective treatment can significantly improve patient prognosis.

关 键 词：溶血尿毒症综合征 补体途径 旁路 血小板减少 肾功能不全 

分 类 号：R692.5[医药卫生—泌尿科学]