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Genetic and Rare Diseases Information Center (GARD)

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Hemolytic uremic syndrome, atypical, childhood


Other Names for this Disease

  • Atypical childhood HUS
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Overview

Hemolytic uremic syndrome, atypical, childhood is a disease that causes abnormal blood clots to form in small blood vessels in the kidneys. These clots can cause serious medical problems if they restrict or block blood flow, including hemolytic anemia, thrombocytopenia, and kidney failure. It is often caused by a combination of environmental and genetic factors. Genetic factors involve genes that code for proteins that help control the complement system (part of your body’s immune system). Environmental factors include viral or bacterial infections, certain medications (such as anticancer drugs), chronic diseases, cancers, and organ transplantation.  Most cases are sporadic. Less than 20 percent of all cases have been reported to run in families. When the disorder is familial, it can have an autosomal dominant or an autosomal recessive pattern of inheritance.[1][2]

Atypical hemolytic-uremic syndrome differs from a more common condition called typical hemolytic-uremic syndrome. The two disorders have different causes and symptoms.[2]

Last updated: 9/27/2010

References

  1. Alpers CE. The Kidney. In: Kumar ed. Robbins and Cotran Pathologic Basis of Disease, Professional Edition , 8th ed. Philadelphia, PA: Saunders; 2009;
  2. Atypical hemolytic-uremic syndrome. Genetics Home Reference. 2010; http://ghr.nlm.nih.gov/condition/atypical-hemolytic-uremic-syndrome. Accessed 9/27/2010.
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Basic Information

  • The Merck Manual provides information on the complement system. The Merck Manuals are a series of healthcare books for medical professionals and consumers.
  • The Foundation for Children with Atypical HUS provides further information on atypical HUS on their Web site. Click on the link above to view the information page.
Other Names for this Disease
  • Atypical childhood HUS
See Disclaimer regarding information on this site. Some links on this page may take you to organizations outside of the National Institutes of Health.