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Hemolytic-uremic syndrome
In medicine, Hemolytic-uremic syndrome (or haemolytic-uraemic syndrome,
abbreviated HUS) is a disease characterised by microangiopathic
hemolytic anemia, acute renal failure and a low platelet count (thrombopenia).
Signs and symptoms
The classic case of HUS occurs after bloody diarrhea caused by E. coli
O157:H7, a strain of E. coli that expresses verotoxin (also called Shiga
toxin). The toxin enters the bloodstream, attaches to renal endothelium
and initiates an inflammatory reaction leading to acute renal failure (ARF)
and diffuse intravascular coagulation (DIC). The fibrin mesh destroys
red blood cells and captures thrombocytes, leading to a decrease of both
on full blood count.
HUS occurs after 2-7% of all E. coli O157:H7 infections.
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Diagnosis
Clinically, HUS can be very hard to distinguish from Thrombotic
Thrombocytopenic Purpura (TTP). The laboratory features are almost
identical, and not every case of HUS is preceded by diarrhea. The only
distinguishing feature is that in TTP, neurological symptoms occur more
often, but this is not always the case.
Treatment
Treatment is with dialysis and blood and platelet transfusion. Mortality
of HUS is 3-5%.
Often, plasmapheresis is also initiated, when there is diagnostic
uncertainty between HUS and TTP.
Prognosis
About one-third of persons with hemolytic-uremic syndrome have abnormal
kidney function many years later, and a few require long-term dialysis.
Another 8% of persons with hemolytic uremic syndrome have other lifelong
complications, such as high blood pressure, seizures, blindness,
paralysis, and the effects of having part of their bowel removed.
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