Diagnosis and treatment of atypical hemolytic uremic syndrome: a case report and literature review
10.3760/cma.j.cn114798-20231010-00224
- VernacularTitle:成人非典型溶血尿毒综合征1例诊治分析并文献复习
- Author:
Haiyan LEI
1
;
Yabo CHEN
;
Chengdian LAN
;
Yao LIU
Author Information
1. 复旦大学附属中山医院厦门医院全科医学科，厦门361015
- Keywords:
Hemolytic-uremic syndrome;
Complement pathway, alternative;
Thrombocytopenia;
Renal insufficiency
- From:
Chinese Journal of General Practitioners
2024;23(7):734-737
- CountryChina
- Language:Chinese
-
Abstract:
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.
