A refractory heterogeneous Cryopyrin-Associated Periodic Syndrome (CAPS) phenotype related to V198M mutation responds to canakinumab - a case report
نویسندگان
چکیده
Methods A 9-year old boy, diagnosed with symptomatic MWS and evidence of a V198M mutation (father also V198M), only partially responsive to anakinra, participated in a phase III study of canakinumab in CAPS patients (N=166). Study treatment was 8-weekly administration of canakinumab by s.c. injection, 150 mg to adults and 2 mg/kg to patients ≤40 kg for up to 2 years. Administration frequency and dose (maximum 600 mg or 8 mg/kg [≤40 kg]) were increased in case of residual symptoms.
منابع مشابه
Canakinumab (ILARIS®) in cryopyrin-associated periodic syndrome (CAPS) patients below 2 years of age: Review of four cases and literature.
Curr Pediatr Res 2017 Volume 21 Issue 4 646 Cryopyrin-Associated Periodic Syndrome (CAPS) is combination of different auto-inflammatory hereditary disorders with same genetic basis. CAPS caused mostly due to mutation of NLRP3 gene (nucleotide-binding domain, leucine-rich family (NLR), pyrin domain containing 3), which is an early onset in children. There are many anti-INF agents, anti-IL blocke...
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