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Atypical Hemolytic Uremic Syndrome

Abstract

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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

  • aHUS
  • familial hemolytic-uremic syndrome
  • hereditary hemolytic-uremic syndrome

Disorder Subdivisions

  • No subdivisions found.

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 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.

Organizations related to Atypical Hemolytic Uremic Syndrome

NORD offers an online community for this rare disease. RareConnect was created by EURORDIS (European Rare Disease Organisation) and NORD (National Organization for Rare Disorders) to provide a safe space where individuals and families affected by rare diseases can connect with each other, share vital experiences, and find helpful information and resources. You can view these international, rare disease communities at www.rareconnect.org.

The information in NORD’s Rare Disease Database is for educational purposes only. It should never be used for diagnostic or treatment purposes. If you have questions regarding a medical condition, always seek the advice of your physician or other qualified health professional. NORD’s reports provide a brief overview of rare diseases. For more specific information, we encourage you to contact your personal physician or the agencies listed as “Resources” on this report.

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