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ahus
Atypical HUS
Atypical hemolytic uremic syndrome (aHUS) is a complement-mediated thrombotic microangiopathy (MAHA + thrombocytopenia + AKI); Factor H mutations are most common (20-30%); uncontrolled alternative pathway at renal endothelium → microthrombi. Eculizumab is standard of care.
Entry Metadata
| Field | Value |
|---|---|
| ID | ahus |
| Name | Atypical HUS |
| Status | draft |
| Last reviewed | 2026-06-08 |
| Atlas | 01-human |
| Scale | 07-system |
Cross-Atlas Connections
Sources
- Fakhouri F, Zuber J, Frémeaux-Bacchi V, Loirat C. Haemolytic uraemic syndrome. Lancet. 2017;390(10095):681-696. · PubMed 28242109
- Legendre CM, Licht C, Muus P, et al. Terminal complement inhibitor eculizumab in atypical hemolytic-uremic syndrome. N Engl J Med. 2013;368(23):2169-2181. · PubMed 23738544
- Goodship TH, Cook HT, Fakhouri F, et al. Atypical hemolytic uremic syndrome and C3 glomerulopathy: conclusions from a 'Kidney Disease: Improving Global Outcomes' (KDIGO) Controversies Conference. Kidney Int. 2017;91(3):539-551. · PubMed 28062089