Familial Mediterranean fever in children: the expanded clinical profile.
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.
Majeed
H A
HA
Department of Pediatrics, Faculty of Medicine, University of Jordan, Amman, Jordan. pal@go.com.jo
Rawashdeh
M
M
el-Shanti
H
H
Qubain
H
H
Khuri-Bulos
N
N
Shahin
H M
HM
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
D007072:Immunoglobulin D