Table 1 Demographic data, duration of treatment, schedule (dose and frequency) of canakinumab and response to treatment at the beginning of the follow-up period
Patient number | Phenotype | NLRP3 mutation | Disease onset | Duration of treatment (months) | Body weight (Kg) | Dose (mg/kg) | Dose at each Administration, mg | Canakinumab frequency, weeks | Response |
|---|---|---|---|---|---|---|---|---|---|
1 | CINCA | N477K | birth | 5 | 65 | 4.6 | 300 | 8 | partial |
2 | CINCA | F573S | birth | 5 | 38 | 4.0 | 152 | 8 | inadequate |
3 | CINCA | Negative | 2 months | 5 | 50 | 6.0 | 300 | 8 | partial |
4 | CINCA | Negative | birth | 6 | 54 | 2.8 | 150 | 8 | partial |
5 | MWS/CINCA | D303N/V198M | 1 week | 12 | 29 | 2.0 | 72 | 8 | complete |
6 | MWS/CINCA | T348M | 6 months | 11 | 85 | 3.5 | 300 | 8 | partial |
7 | CINCA | E304K | birth | 12 | 78 | 3.8 | 300 | 8 | partial |
8 | MWS | T348M | birth | 12 | 63 | 2.4 | 150 | 8 | partial |
9 | MWS | E525K | 15 months | 17 | 62 | 2.4 | 150 | 8 | complete |
10 | MWS | D303N | 3 weeks | 20 | 37 | 2.0 | 74 | 8 | complete |
11 | CINCA | T348M | birth | 15 | 23 | 2.0 | 46 | 8 | partial |
12 | CINCA | Negative | birth | 7 | 61 | 2.5 | 150 | 8 | complete |
13 | MWS | V198M | 9 years | - | 38 | 2.0 | 76 | 8 | complete |