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- W2293342860 abstract "HUS and TTP are two different diseases resulting from arterial microthrombosis. For HUS, thrombotic microangiopathy lesions predominate in the kidney and the main expression is renal failure. For TTP the lesions predominate in the brain and main signs are neurological. Cases with HUS and TTP features are reported. The recent progress in the knowledge of the physiopathology of these syndromes may help for diagnosis and treatment. In TTP, there often is a constitutive or an acquired deficiency in the metalloprotease cleaving the von Willebrand factor, which is not observed in HUS. In familial HUS, the main abnormality is factor H deficiency, which leads to complement activation and endothelial cell lesions. Some retrospective series suggest that plasma exchanges or plasma infusions may be as effective in HUS as in TTP but their efficiency have not been demonstrated in prospective trials. The prognosis is good if treatment is started at the early phase with mortality lower than 10%. Chronic renal failure, sometimes requiring chronic dialysis, may complicate the evolution of HUS in 25 to 50% of the cases." @default.
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- W2293342860 date "2002-09-12" @default.
- W2293342860 modified "2023-09-24" @default.
- W2293342860 title "The thrombotic microangiopathy: the nephrologist point of view" @default.
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