The changing face of medicine... and the future of the TMA?
Brenda M Williams
Tennessee medicine : journal of the Tennessee…
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HYSTORY: The Registry was established in 1996 under the coordination of the Clinical Research Center for Rare Diseases Aldo e Cele Daccò. AIMS OF THE REGISTRY: - understanding the pathogenesis of HUS/TTP - studying the genetic and biochemical abnormalities of HUS/TTP - collecting clinical and genetic data of patients and their families - finding the best therapeutic approach for patients - giving up-to-date information to physicians and families INTERNATIONAL REGISTRY of RECURRENT and FAMILIAL HEMOLYTIC UREMIC SYNDROME (HUS) and THROMBOTIC THROMBOCYTOPENIC PURPURA (TTP) THE THROMBOTIC MICROANGIOPATHY The term “thrombotic microangiopathy” (TMA) defines a lesion of vessel wall thickening (mainly arterioles and capillaries), intraluminal platelet thrombosis and partial or complete obstruction of the vessel lumina. HUS microangiopathic haemolysis, thrombocytopenia and renal failure TTP microangiopathic hemolysis, thrombocytopenia and cerebral lesions HUS and TTP are two clinically defined syndromes with shared pathology of thrombotic microangiopathy FORMS OF HUS/TTP:
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