A question of burning importance! Hemolytic and uremic syndrome in children and adults

Abstract

The hemolytic uremic syndrome (HUS) is related to a thrombotic microangiopathy in the kidney, leading to hypertension and acute renal failure. In the post-infectious, typical form, its pathogenesis includes a lesion/activation of endothelial cells, mainly in the kidney vasculature, induced by bacterial enterotoxins known as the shiga-toxins. Haemolytic anemia with schizocytes, thrombocytopenia without intravascular coagulation and renal failure are present. In typical HUS, a prodromic hemorragic diarrhea is observed due to an infection by an enteropathogenic bacteria, mainly the O157:H7 Escherichia coli. Recently a very large epidemic of diarrhea complicated by HUS in about 25% of the cases has been observed in Germany. Prognosis has improved greatly, and the patient survival rate is higher than 85% in a year. Chronic renal failure may result in 10 to 20% of the cases. Fresh plasma infusion or plasma exchanges are recommended in most of the cases of typical HUS, especially in severe forms in both children and adults. However, eculizumab, an anti-C5 antibody which is now recommended in complement deficiency-associated HUS, has been shown recently to be effective in post-infectious HUS as well. It may become the firstline treatment of infectious HUS in the near future.