title: [Turkish children with recurrent abdominal pain and fever: familial Mediterranean fever].
abstract: Four children of Turkish origin, three boys aged 12, 8 and 7 years, and a girl aged 5 years, presented with clinical symptoms of familial Mediterranean fever. They had the characteristic episodes of fever combined with abdominal pain, thoracic pain, general malaise or arthralgia. Familial Mediterranean fever is an autosomal recessive genetic disorder restricted to people originating from the Middle East. The causative gene (MEFV) and many missense mutations have been identified. The clinical syndrome is characterised by self-limiting febrile episodes accompanied by inflammation of the serous membranes, resulting in peritonitis, pleuritis or synovitis. In untreated patients systemic amyloidosis may develop, which manifests as renal insufficiency. The diagnosis is based on the characteristic medical history and is confirmed by DNA analysis. Meanwhile, treatment with colchicine can be started. This is effective in 90% of affected patients. Being aware of the prevalence of familial Mediterranean fever in immigrant populations can improve the quality of life and prevent long-term complications.
authors.affiliation: Medisch Centrum Rijnmond-Zuid, locatie Zuider, Postbus 9119, 3007 AC Rotterdam. hulsmanna@mcrz.nl
authors.forename: A R
authors.initials: AR
authors.lastname: Hulsmann
authors.forename: W B
authors.initials: WB
authors.lastname: Hofstra
authors.forename: J G
authors.initials: JG
authors.lastname: Brinkman
authors.forename: M J
authors.initials: MJ
authors.lastname: van der Wielen
authors.forename: E
authors.initials: E
authors.lastname: Bakker
authors.forename: A M
authors.initials: AM
authors.lastname: Oudesluys-Murphy
mesh_terms: D015746:Abdominal Pain / Q000209:etiology*; D000686:Amyloidosis / Q000209:etiology / Q000235:genetics; D002648:Child; D002675:Child, Preschool; D003078:Colchicine / Q000627:therapeutic use; D010505:Familial Mediterranean Fever / Q000175:diagnosis* / Q000188:drug therapy / Q000453:epidemiology / Q000235:genetics; D005260:Female; D006801:Humans; D008297:Male; D020125:Mutation, Missense; D015995:Prevalence; D012008:Recurrence; D014421:Turkey / Q000453:epidemiology
chemical_list: D003078:Colchicine