https://scholars.lib.ntu.edu.tw/handle/123456789/627475
標題: | Atypical hemolytic uremic syndrome: Consensus of diagnosis and treatment in taiwan | 作者: | Tseng, Min-Hua Lin, Shih-Hua Tsai, Jeng-Daw Wu, Mai-Szu I-JUNG TSAI Chen, Yeu-Chin Chang, Min-Chih WEN-CHIEN CHOU Chiou, Yee-Hsuan Huang, Chiu-Ching |
關鍵字: | Atypical hemolytic uremic syndrome; Coagulation; Complement | 公開日期: | 30-十月-2022 | 來源出版物: | Journal of the Formosan Medical Association = Taiwan yi zhi | 摘要: | Atypical hemolytic uremic syndrome (aHUS), characterized by microangiopathic hemolytic anemia, thrombocytopenia, and acute kidney injury, is a rare but life-threatening systemic disorder caused by the dysregulation of the complement pathway. Current advances in molecular analysis and pathogenesis have facilitated the establishment of diagnosis and development of effective complement blockade. Based on this recent consensus, we provide suggestions regarding the diagnosis and management of aHUS in Taiwan. The diagnosis of aHUS is made by the presence of TMA with normal ADAMTS13 activity without known secondary causes. Although only 60% of patients with aHUS have mutations in genes involving the compliment and coagulation systems, molecular analysis is suggestive for helping establish diagnosis, clarifying the underlying pathophysiology, guiding the treatment decision-making, predicting the prognosis, and deciding renal transplantation. Complement blockade, anti-C5 monoclonal antibody, is the first-line therapy for patients with aHUS. Plasma therapy should be considered for removing autoantibody in patients with atypical HUS caused by anti-CFH or complement inhibitor is unavailable. |
URI: | https://scholars.lib.ntu.edu.tw/handle/123456789/627475 | ISSN: | 0929-6646 | DOI: | 10.1016/j.jfma.2022.10.006 |
顯示於: | 醫學系 |
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