Atypical hemolytic uremic syndrome: Consensus of diagnosis and treatment in taiwan

Min Hua Tseng, Shih Hua Lin, Jeng Daw Tsai, Mai Szu Wu, I. Jung Tsai, Yeu Chin Chen, Min Chih Chang, Wen Chien Chou, Yee Hsuan Chiou, Chiu Ching Huang

研究成果: 雜誌貢獻文章同行評審

4 引文 斯高帕斯(Scopus)

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.
原文英語
頁(從 - 到)366-375
頁數10
期刊Journal of the Formosan Medical Association
122
發行號5
DOIs
出版狀態已發佈 - 5月 2023

ASJC Scopus subject areas

  • 一般醫學

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