Complement-Mediated Atypical Hemolytic Uremic Syndrome

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Published: May 28, 2024
Keywords:
glomerulonephritis HUS acute kidney injury AKI acute renal failure

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Saranchana Jiampochaman
Division of Nephrology, Department of Medicine, Faculty of Medicine, Chulalongkorn University, Bangkok Thailand
Wonngarm Kittanamongkolchai
Division of Nephrology, Department of Medicine, Faculty of Medicine, Chulalongkorn University, Bangkok Thailand

Abstract

Atypical hemolytic uremic syndrome (aHUS) is a kidney disease caused by dysregulation of the  alternative complement pathway. Uncontrolled complement activation resulted in endothelial injury, platelet activation and consumption, and thrombus formation, leading to kidney injury. Clinical features of aHUS include microangiopathic hemolytic anemia, thrombocytopenia, and acute kidney injury. Atypical HUS occurs in genetically susceptible individuals who have an acquired precipitating event that unmasks a complement regulatory deficiency. The incidence of aHUS is 0.5 per million per year. Histopathological features include endothelial swelling, fibrin thrombi, and fragmented red blood cells. Current treatment options are complement-inhibiting therapy, plasma exchange, immunosuppressive medication, and kidney and/or liver transplantation. The choice of treatment is based on the underlying complement regulation defect.

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How to Cite
Jiampochaman, S. ., & Kittanamongkolchai, W. (2024). Complement-Mediated Atypical Hemolytic Uremic Syndrome. Journal of the Nephrology Society of Thailand, 30(2), 110–121. Retrieved from https://he01.tci-thaijo.org/index.php/JNST/article/view/269500
Issue
Vol. 30 No. 2 (2024): April - June
Section
Review Article
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