FAMILIAL MEDITERRANEAN FEVER IN 2 ITALIAN BROTHERS

Citation
L. Breda et al., FAMILIAL MEDITERRANEAN FEVER IN 2 ITALIAN BROTHERS, Panminerva Medica, 40(2), 1998, pp. 157-160
Citations number
24
Categorie Soggetti
Medicine, General & Internal
Journal title
ISSN journal
00310808
Volume
40
Issue
2
Year of publication
1998
Pages
157 - 160
Database
ISI
SICI code
0031-0808(1998)40:2<157:FMFI2I>2.0.ZU;2-Z
Abstract
Familial Mediterranean fever (FMF) is an autosomal recessive disease o f unknown etiology, characterized by recurrent self limited episodes o f fever and polyserositis, Some patients develop generalized amyloidos is, which can be fatal, Colchicine therapy modifies the natural histor y of the disease by decreasing the attack frequency and preventing amy loid deposition. The disease is common among Sephardic Jews, Arabs, Ar menians and has also been sporadically found in other ethnic groups of Mediterranean origin. We report two cases of FR IF in brothers living in Abruzzo, Italy. They were born from consanguineous parents and com plained typical symptoms since childhood, The boy suffered from one fe brile attack every week; he presented three episodes of acute scrotum at age 8 and 9, The elder sister showed a spontaneous partial relief d uring adolescence, Juvenile rheumatoid arthritis was suspected and Asp irin was used for many years without any clinical improvement, Treatme nt with colchicine 1 mg/day was established at age 13 and 17 respectiv ely, and a sudden reduction of frequency of attacks was obtained. A gi ngival biopsy did not show amyloid, The three elder brothers are, at p resent, in good health, Our experience point out the diagnostic diffic ulties of FMF expecially in a country were the disease is uncommon.