Atypical Hemolytic Uremic Syndrome

**Definition:** An hereditary hemolytic uremic syndrome associated with variations in the gene that encodes COMPLEMENT FACTOR H, or the related proteins CFHR1 and CFHR3. Disease often progresses to CHRONIC KIDNEY FAILURE without the prodromal symptoms of ENTEROCOLITIS and DIARRHEA that characterize typical hemolytic uremic syndrome.

**Tree numbers:** - C12.050.351.968.419.936.463.500 - C12.200.777.419.936.463.500 - C12.950.419.936.463.500 - C15.378.050.141.610.500 - C15.378.140.855.925.500.500 - C15.378.243.937.925.500.500

**Synonyms:** - Atypical Hemolytic-Uremic Syndrome - Non-Stx-Hus - Hemolytic Uremic Syndrome, Atypical - Nonenteropathic HUS - Non-Shiga-Like Toxin-Associated HUS