Table 1.
Clinical and genetic data available for patients in acute phase of atypical haemolytic uraemic syndrome (aHUS)
| Patient no. | aHUS pathogenic change | Gender | Age at onset/time of study* | Treatment | Outcome |
|---|---|---|---|---|---|
| P1a† | C3: p.Arg161Trp 15,16 | Male | 1st episode: 6 years;2nd episode: 12 years | Plasma therapy | Remission |
| P2a | None | Female | 7 years | Plasma therapy | Remission |
| P3a | Anti-CFH autoantibodies 19 | Female | 11 years | Plasma therapy, haemodialysis | Remission |
| P4a | MCP: p.Asp271_Ser272del 10 | Male | 1st episode: 4 years;2nd episode: 8 years | Plasma therapy | Remission |
| P5a† | None | Male | 1st episode: 7 months;2nd episode: 2 years | Plasma therapy, eculizumab | Remission under eculizumab |
| P6a | None | Male | 5 years | Plasma therapy, CVVHDF | Remission |
Age at onset of the latest episode for these patients is equal to the age at the time of the study. First episodes of patients P1a, P4a and P5a resolved in (almost) complete remission without need of a renal transplantation.
Familial aHUS. CVVHDF = continuous veno-venous haemodiafiltration; CFH = complement factor H; MCP = membrane co-factor protein.