Hemolytic-uremic syndrome is a predominantly pediatric condition that consists of the simultaneous triad of acute renal failure, thrombocytopenia, and microangiopathic hemolytic anemia associated with distorted erythrocytes. It is classified as either D+ (typical) when it is associated with a preceding diarrheal illness, which in most people is caused by infection with verotoxin-producing E. coli, is self-limiting and nonrecurring, with complete recovery in about 90% of cases, or less commonly non-diarrheal associated D- (atypical), often recurrent and sporadic, and generally with a poor outcome. The syndrome may be sporadic or familial. The familial form of hemolytic-uremic syndrome is generally considered to be an autosomal recessive disorder, but dominant pedigrees have also been reported. Mortality rates are greater than 90% in patients with autosomal dominant disease and 70% in patients with the autosomal recessive form.
Although there are new therapeutic modalities in the horizon for D+HUS, present recommended therapy is merely symptomatic. Parenteral volume expansion may counteract the effect of thrombotic process before development of HUS and attenuate renal injury. Use of antibiotics, antimotility agents, narcotics and non-steroidal anti-inflammatory drugs should be avoided during the acute phase. Prevention is best done by preventing primary STEC infection.