Table 2.

Summarized clinical course in the various subgroups of aHUSa

ParameterMutations
CFHIFMCPNo
n106722
Age at onset (median [range])b6 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)3401
Male/femalec4/65/13/413/9
Familial HUS (no. of pedigree)d4 (3)g1 (1)g2 (1)7 (4)
Relapsing HUS (%)e3 (30)2 (33)6 (86)13 (59)
CNS involvement1103
Plasmatherapy (efficient/not efficient)2/42/10/43/5
Outcomef
    death2101
    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.