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[Familial Mediterranean fever: not to be missed].
- Source :
-
Nederlands tijdschrift voor geneeskunde [Ned Tijdschr Geneeskd] 2013; Vol. 157 (18), pp. A5784. - Publication Year :
- 2013
-
Abstract
- Familial Mediterranean fever (FMF) is common among Turkish and Moroccan migrants. We describe three patients with FMF. A 3-year-old girl with recurrent fever and abdominal pain who was diagnosed early with FMF and treated effectively with colchicine. An adolescent girl who required interleukin (IL)-1 blockade to achieve disease remission. And a 37-year-old woman in whom the attacks of FMF had not been recognised, but who developed end-stage kidney failure due to AA amyloidosis. Mutations in the MEFV gene underlie the disease in most but not all patients. Therefore, FMF remains a clinical diagnosis. FMF patients suffer recurrent bouts of inflammation, often with fever, serositis or arthritis. The major complication is AA amyloidosis. The inflammatory process is mediated by IL-1β. When started early, colchicine prophylaxis can prevent amyloidosis. When colchicine fails, IL-1 blockade has shown promising results. Timely diagnosis and treatment can make the difference between near normal health and end-stage kidney failure.
- Subjects :
- Adolescent
Adult
Amyloidosis genetics
Amyloidosis therapy
Child, Preschool
Familial Mediterranean Fever complications
Familial Mediterranean Fever genetics
Female
Humans
Inflammation drug therapy
Inflammation etiology
Interleukin-1 antagonists & inhibitors
Morocco ethnology
Mutation
Netherlands
Turkey ethnology
Amyloidosis etiology
Colchicine therapeutic use
Familial Mediterranean Fever diagnosis
Familial Mediterranean Fever drug therapy
Tubulin Modulators therapeutic use
Subjects
Details
- Language :
- Dutch; Flemish
- ISSN :
- 1876-8784
- Volume :
- 157
- Issue :
- 18
- Database :
- MEDLINE
- Journal :
- Nederlands tijdschrift voor geneeskunde
- Publication Type :
- Academic Journal
- Accession number :
- 23635502