Table 3.
Main clinical characteristics of patients with atypical hemolytic uremic syndrome according to complement abnormality
| Gene or subgroup | Frequency in aHUS | Minimal age at onset | Risk of death or ESRD at 1st episode or within < 1 y | Risk of relapses | Risk of recurrence after renal transplantation | Plasma therapy indicated | |
|---|---|---|---|---|---|---|---|
| Children | Adults | ||||||
| CFH | 20-30% | Birth | any age | 50-70% | 50% | 75-90% | Yes |
| CFI | 4 -10% | Birth | any age | 50% | 10-30% | 45-80% | Yes |
| MCP | 5 -15% | > 1 y | any age | 0-6% | 70-90% | < 20% | Questionable |
| C3 | 2 -10% | 7 m | any age | 60% | 50% | 40-70% | Yes |
| CFB | 1-4% | 1 m | any age | 50% | 3/3 not in ESRD | 100% | Yes |
| THBD | 3 -5% | 6 m | rare | 50% | 30% | 1 patient | Yes |
| Anti-CFH Ab | 6% | Mostly 7-11 y | 30-40% | 40-60% | Yes if high Ab titer | Yes (+ IS) | |
CFH: factor H; CFI: factor I; MCP: membrane cofactor protein; CFB: factor B; THBD: thrombomodulin; Ab, antibodies; ESRD: end stage renal disease; IS: immunosuppressive treatment.