ATYPICAL HEMOLYTIC-UREMIC SYNDROME IN HUMAN IMMUNODEFICIENCY VIRUS-1-INFECTED CHILDREN

We describe the clinical and pathological findings of the hemolytic ur emic syndrome (HUS) in two children with human immunodeficiency virus (HIV) infection. Both patients presented with microangiopathic hemolyt ic anemia, thrombocytopenia, and subsequently developed renal failure. The diagnosis of HUS was confirmed by renal histopathology in both pa tients. None of these children presented with bloody diarrhea, evidenc e of circulating antibody response to Escherichia coli O157 lipopolysa ccharide, or other known risk factors for HUS, except for the presence of HIV infection. Each patient was treated with intravenous plasma in fusion and renal replacement therapy. Their clinical course was charac terized by non-oliguria and lack of significant hypertension throughou t the acute phase of the disease. Despite these favorable clinical par ameters, both patients developed endstage renal failure. The etiology of this atypical HUS characterized by poor renal survival remains unkn own and the role of HIV infection in its pathogenesis, although possib le, is unclear.