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Atypical hemolytic-uremic syndrome
rareA rare, genetic thrombotic microangiopathy due to dysregulation of the alternative complement pathway and characterized by the triad of hemolytic anemia, thrombocytopenia, and acute renal dysfunction.
- Identifiers
- MONDO:0016244 Orphanet:2134
- Part of
- Complement deficiencyHereditary hemolytic uremic syndromeSyndromic diseaseThrombotic microangiopathy
- Also known as
- aHUS · Atypical Hemolytic Uremic Syndrome · atypical HUS · D-HUS · D-minus hemolytic uremic syndrome (D-HUS)
Subtypes
Disease Rare Not rare Subtypes
- Atypical hemolytic uremic syndrome with complement gene abnormality MONDO:0035290 5
- Atypical hemolytic-uremic syndrome with DGKE deficiency MONDO:0018159
- Atypical hemolytic-uremic syndrome with H factor anomaly MONDO:0019738
- Atypical hemolytic-uremic syndrome with anti-factor H antibodies MONDO:0019739
Projects
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