Summarized clinical course in the various subgroups of aHUSa
| Parameter | Mutations | |||
|---|---|---|---|---|
| CFH | IF | MCP | No | |
| n | 10 | 6 | 7 | 22 |
| Age at onset (median [range])b | 6 mo(3 d to 3 yr, 6 mo) | 2 mo(1 d to 3 yr, 8 mo) | 4 yr, 6 mo(1 yr, 6 mo to 11 yr, 3 mo) | 1 yr, 1 mo(15 d to 15 yr) |
| Newborn (<3 mo) | 3 | 4 | 0 | 1 |
| Male/femalec | 4/6 | 5/1 | 3/4 | 13/9 |
| Familial HUS (no. of pedigree)d | 4 (3)g | 1 (1)g | 2 (1) | 7 (4) |
| Relapsing HUS (%)e | 3 (30) | 2 (33) | 6 (86) | 13 (59) |
| CNS involvement | 1 | 1 | 0 | 3 |
| Plasmatherapy (efficient/not efficient) | 2/4 | 2/1 | 0/4 | 3/5 |
| Outcomef | ||||
| death | 2 | 1 | 0 | 1 |
| ESRD (as soon as first flare) | 6 (4) | 2 (2) | 2 (0) | 6 (4) |
↵a CNS, central nervous system.
↵b P = 0.02; age at onset is significantly different among the four groups, with the youngest being in the CHF and IF mutation groups.
↵c P = 0.37; gender ratio is not different among the four groups.
↵d P = 0.85; distribution of familial HUS is not different among the four groups.
↵e P = 0.10; proportion of patients with relapsing HUS is not different among the four groups.
↵f Outcome within the 10 yr after onset is indicated. One patient in the CFH mutation group had ESRD at death.
↵g Brother of patients 4 and 16 were not included in the study (aHUS after the end of the recruitment of patients). They have no mutation of CFH or IF, suggesting the presence of another genetic unknown factor.