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Colegio de Medicina Interna de México.

2014, Number 5

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Med Int Mex 2014; 30 (5)

Atypical hemolytic uremic syndrome

Buelna-Cano C, Gálvez-Acosta S, Ornelas-Velázquez A

Language: Spanish
References: 12
Page: 622-628
PDF size: 440.64 Kb.


ABSTRACT

Hemolytic uremic syndrome is a clinical entity defined by the triad of non-immune microangiopathic hemolytic anemia (negative direct Coombs), thrombocytopenia and acute renal failure. Histological lesions of hemolytic uremic syndrome are characterized by systemic thrombotic microangiopathy, affecting preferently renal vessels, with wall thickening, thrombosis and vascular light obstruction. Atypical hemolytic uremic syndrome is a subtype in which phenomena of thrombotic microangiopathy are consequence of lost of regulation of alternative way of complement on cell surfaces of genetic cause in most cases with a predominantly infectious triggering. Near 10% of cases of hemolytic uremic syndrome are classified as atypical and have a poor prognosis, with mortality of 25% and progression to terminal renal failure in 50%. This paper reports the case of a 15-year-old female patient with nonimmune hemolytic anemia, thrombocytopenia and acute renal failure.


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