title: Familial Mediterranean fever in children: the expanded clinical profile.
abstract: The clinical picture of familial Mediterranean fever (FMF) has been appreciably expanded in the last 10 years. Over 8 years, we studied the expanded clinical profile of FMF in 476 children. Of these, 81% had abdominal pain, 41% chest pain, 42% arthritis, 12% severe myalgia, 12% skin manifestations, 4% scrotal swelling, 3% recurrent episodic fever, and one child (0.2%) developed recurrent hyperbilirubinaemia. Two (0.4%) children developed renal complications which were reversed by colchicine; however of 19 probands, 36 family members suffered from chronic renal failure. Our study indicates a familial predisposition to nephropathy in certain families with FMF. This study is the first to report the expanded clinical profile of FMF in a large group of Arab children, giving an opportunity to compare the findings with those in children with FMF in other ethnic groups, and to help in the study of genotype-phenotype correlation.
authors.affiliation: Department of Pediatrics, Faculty of Medicine, University of Jordan, Amman, Jordan. pal@go.com.jo
authors.forename: H A
authors.initials: HA
authors.lastname: Majeed
authors.forename: M
authors.initials: M
authors.lastname: Rawashdeh
authors.forename: H
authors.initials: H
authors.lastname: el-Shanti
authors.forename: H
authors.initials: H
authors.lastname: Qubain
authors.forename: N
authors.initials: N
authors.lastname: Khuri-Bulos
authors.forename: H M
authors.initials: HM
authors.lastname: Shahin
mesh_terms: D015746:Abdominal Pain / Q000209:etiology; D017668:Age of Onset; D000686:Amyloidosis / Q000209:etiology; D002648:Child; D002675:Child, Preschool; D005006:Ethnicity; D010505:Familial Mediterranean Fever / Q000150:complications / Q000175:diagnosis* / Q000208:ethnology; D005260:Female; D006801:Humans; D007072:Immunoglobulin D / Q000032:analysis; D007223:Infant; D007231:Infant, Newborn; D007597:Jordan; D008297:Male; D010375:Pedigree; D015995:Prevalence
chemical_list: D007072:Immunoglobulin D