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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

From: The schedule of administration of canakinumab in cryopyrin associated periodic syndrome is driven by the phenotype severity rather than the age

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
  1. CINCA: chronic infantile neurological cutaneous articular syndrome; MWS: Muckle-Wells syndrome; MWS/CINCA: overlap syndrome.