The hemolytic-uremic syndrome.

P A Rodrigues, J Machado, O Cardoso, J Cruz, F Carvalho, M da Costa, R Proença

Abstract


The microangiopathic thrombotic syndromes--thrombotic thrombocytopenic purpura (TTP) and hemolytic uremic syndrome (HUS)--are characterized by microangiopathic hemolytic anemia, thrombocytopenia, renal dysfunction, fever and central nervous system abnormalities. Today they are considered as two extremes of a continuous spectrum named TTP--HUS. The syndrome is an uncommon disease with a high mortality rate, despite treatment. The authors describe a case of hemolytic uremic syndrome in a young adult patient. Initially the clinical course and the first biopsy suggested a favourable prognosis, but the early recurrence with severe hypertension was followed by a fatal outcome 6 months later. Concerning this clinical case, the authors present a review of the most recent aspects of the pathogenesis and treatment of this syndrome.

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