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7% vs 8.8%, p less then 0.001). Children carrying homozygous M694V mutation had significantly earlier age of disease onset and severe disease course (p less then 0.001). Forty-four patients (2.6%) were colchicine resistant and most of them were carrying homozygous M694V mutation. Sixteen colchicine-resistant patients were treated with anakinra while 28 received canakinumab. Juvenile idiopathic arthritis (JIA) and immunoglobulin A vasculitis were the most commonly seen associated diseases and the patients with a concomitant disease d