Form of aHUS | Complement abnormalities |
---|---|
Familial | Mutations in factor H, 40–45% |
In factor I, 5–10% | |
In C3, 8–10% | |
In membrane cofactor protein, 7–15% | |
In thrombomodulin, 9% | |
In factor B, 1–2% | |
Sporadic | Mutations in factor H, 15–20% |
In factor I, 3–6% | |
In C3, 4–6% | |
In membrane cofactor protein, 6–10% | |
In thrombomodulin, 2% | |
In factor B, 2 cases | |
Anti-Factor H antibodies, 6–10% |