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Atypical Hemolytic Uremic Syndrome

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Copyright 2004, 2009

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
  • aHUS
  • Familial Hemolytic-Uremic Syndrome
  • Hereditary Hemolytic-Uremic Syndrome

Disorder Subdivisions



General Discussion
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 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.

Organizations related to Atypical Hemolytic Uremic Syndrome
  • American Kidney Fund, Inc.
    6110 Executive Boulevard
    Suite 1010
    Rockville MD 20852
    Phone #: 301-881-3052
    800 #: 800-638-8299
    e-mail: mdemelo@kidneyfund.org
    Home page: http://www.kidneyfund.org
  • Foundation for Children with Atypical HUS
    c/o Bill Biermann
    7018 Forest Oak Drive
    Barnhart MO 63012
    Phone #: 636-942-4425
    800 #: --
    e-mail: bbiermann@premier-ks.com
    Home page: http://www.atypicalhus.net/
  • Genetic and Rare Diseases (GARD) Information Center
    PO Box 8126
    Gaithersburg MD 20898-8126
    Phone #: 301-251-4925
    800 #: 888-205-2311
    e-mail: http://rarediseases.info.nih.gov/GARD/EmailForm.aspx
    Home page: http://rarediseases.info.nih.gov/GARD
  • National Kidney Foundation
    30 East 33rd Street
    New York NY 10016
    Phone #: 212-889-2210
    800 #: 800-622-9010
    e-mail: info@kidney.org
    Home page: http://www.kidney.org

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Last modified Wednesday, November 26, 2008