Familial Mediterranean fever in children: the expanded clinical profile

The clinical picture of familial Mediterranean fever (FMF) has been appreci ably expanded in the last 10 years. Over 8 years, we studied the expanded c linical profile of FMF in 476 children. Of these, 81% had abdominal pain, 4 1% chest pain, 42% arthritis, 12% severe myalgia, 12% skin manifestations, 4% scrotal swelling, 3% recurrent episodic fever, and one child (0.2%) deve loped recurrent hyperbilirubinaemia. Two (0.4%) children developed renal co mplications which were reversed by colchicine; however of 19 probands, 36 f amily members suffered from chronic renal failure. Our study indicates a fa milial predisposition to nephropathy in certain families with FMF. This stu dy is the first to report the expanded clinical profile of FMF in a large g roup of Arab children, giving an opportunity to compare the findings with t hose in children with FMF in other ethnic groups, and to help in the study of genotype-phenotype correlation.