Atypical Hemolytic Uremic Syndrome

National Organization for Rare Disorders, Inc.

Important
It is possible that the main title of the report Atypical Hemolytic Uremic Syndrome is not the name you expected. Please check the synonyms listing to find the alternate name(s) and disorder subdivision(s) covered by this report.

Synonyms

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

Disorder Subdivisions

  • None

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.

Resources

American Kidney Fund, Inc.
6110 Executive Boulevard
Suite 1010
Rockville, MD 20852
USA
Tel: (301)881-3052
Fax: (301)881-0898
Tel: (800)638-8299
Email: helpline@kideyfund.org
Internet: http://www.kidneyfund.org

National Kidney Foundation
30 East 33rd Street
New York, NY 10016
Tel: (212)889-2210
Fax: (212)689-9261
Tel: (800)622-9010
Email: info@kidney.org
Internet: http://www.kidney.org

Foundation for Children with Atypical HUS
c/o Bill Biermann
7018 Forest Oak Drive
Barnhart, MO 63012
USA
Tel: (636)942-4425
Fax: (314)429-3790
Email: bbiermann@premier-ks.com
Internet: http://www.atypicalhus.net/

Genetic and Rare Diseases (GARD) Information Center
PO Box 8126
Gaithersburg, MD 20898-8126
Tel: (301)251-4925
Fax: (301)251-4911
Tel: (888)205-2311
TDD: (888)205-3223
Email: ordr@od.nih.gov
Internet: http://rarediseases.info.nih.gov/Default.aspx

For a Complete Report

For a Complete Report

This is an abstract of a report from the National Organization for Rare Disorders, Inc. ® (NORD). A copy of the complete report can be obtained for a small fee by visiting the NORD website. The complete report contains additional information including symptoms, causes, affected population, related disorders, standard and investigational treatments (if available), and references from medical literature. For a full-text version of this topic, see http://www.rarediseases.org/search/rdblist.html

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