Atypical Hemolytic Uremic Syndrome
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NORD is very grateful to Giuseppe Remuzzi, MD, Mario Negri Institute for Pharmacological Research, Italy, for assistance in the preparation of this report.
Synonyms of Atypical Hemolytic Uremic Syndrome
- familial hemolytic-uremic syndrome
- hereditary hemolytic-uremic syndrome
- No subdivisions found.
Atypical hemolytic uremic syndrome (aHUS) is an extremely rare disease characterized by hemolytic anemia, low platelet count (thrombocytopenia) and acute renal failure. It is a distinctly different illness from hemolytic uremic syndrome caused by particular strains of the bacterium E.coli producing Shiga toxins, most frequently the 0157:H7 strain (Stx HUS). While Stx HUS typically is preceded by a gastroenteritis and is associated with infection by Shiga toxin producing-E.coli, there is substantial evidence that aHUS is a genetic disorder.
Atypical hemolytic uremic syndrome may become a chronic condition, and patients with aHUS may experience repeated attacks of the disorder. When children with Stx HUS recover from the life-threatening initial episode, they are likely to respond well to supportive treatment and to make a good recovery. Children with aHUS are much more likely to develop chronic serious complications such as kidney failure and severe high blood pressure.
Atypical Hemolytic Uremic Syndrome Resources
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