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Haemolytic uraemic syndrome: prognostic factors

Haemolytic uraemic syndrome: prognostic factors. Green DA, Murphy WG, Uttley WS. Clinical and Laboratory Haematology 2000;22(1):11-14.


Haemolytic uraemic syndrome (HUS) associated with Escherichia coli O157:H7 is the most common cause of acute renal failure (ARF) in childhood. Production of verotoxin by the organism is pivotal in the pathogenesis of the disease. Verotoxin binds to a receptor on blood and endothelial cells, expressed as the P1 blood group antigen on red blood cells. A protective effect of the P1 phenotype has been proposed in this disease. This study investigated diarrhea-associated HUS as part of a larger outbreak investigation of E. Coli in Scotland in 1994. Ten children developed HUS compared to no adults. An additional 12 subsequent HUS cases, plus five previous cases at the same hospital since 1999 were also examined. Prognostic factors and the relationship between outcome and P1 phenotype in 27 pediatric cases of diarrhoea-associated HUS were assessed. A poor outcome, defined by the presence of chronic renal failure (CRF), hypertension or proteinuria on six-month follow-up, was associated with younger age at presentation and with the following clinical markers: greater maximum white blood cell count (WBC) and duration of raised WBC, greater duration of anuria, and longer duration of need for dialysis. None of these outcome measures or prognostic factors, and no extra-renal manifestations of the disease, were associated with P1 phenotype.