Journal of Clinical Medicine, Journal Year: 2025, Volume and Issue: 14(7), P. 2527 - 2527

Published: April 7, 2025

Atypical hemolytic uremic syndrome (aHUS) is a rare, life-threatening thrombotic microangiopathy (TMA) characterized by complement dysregulation, leading to microvascular thrombosis and multi-organ injury. TMAs are defined thrombocytopenia, microangiopathic anemia organ dysfunction caused small-vessel thrombosis. Unlike thrombocytopenic purpura, which results from severe ADAMTS13 deficiency, aHUS driven uncontrolled activation of the alternative pathway. While kidneys most frequently affected, other vital organs can also be involved. Genetic susceptibility contributes significantly disease risk, but trigger such as infection, pregnancy or autoimmune usually required. Diagnosis challenging due overlapping features with relies on exclusion testing. C5 inhibitors, eculizumab ravulizumab, have revolutionized treatment necessitate prophylactic vaccination ongoing clinical surveillance. these therapies provide effective control, discontinuing remains complex, especially in patients gene mutations. New targeting various points cascade under investigation may offer safer, more cost-effective options. Progress genetic profiling biomarker discovery essential for earlier diagnosis, individualized therapy relapse prevention. This review highlights recent advances understanding pathophysiology, evolving therapeutic strategies aimed at improving patient outcomes.

Language: Английский