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Panminerva Medica 1998 June;40(2):157-60

lingua: Inglese

Familial Med­i­ter­ra­ne­an ­fever in two Ital­ian broth­ers

Breda L., Magrì M., Morgese G.*, Chiarelli F.

From the Department of Pediatrics University of Chieti and * Siena, Italy


Familial Mediterranean fever (FMF) is an auto­so­mal reces­sive dis­ease of ­unknown eti­ol­o­gy, char­ac­ter­ized by recur­rent ­self lim­it­ed epi­sodes of ­fever and poly­ser­o­sitis. Some ­patients devel­op gen­er­al­ized amy­loi­do­sis, ­which can be ­fatal. Colchicine ther­a­py mod­i­fies the nat­u­ral his­to­ry of the dis­ease by decreas­ing the ­attack fre­quen­cy and pre­vent­ing amy­loid dep­o­si­tion. The dis­ease is com­mon ­among Sephardic Jews, Arabs, Armenians and has ­also ­been spo­rad­i­cal­ly ­found in oth­er eth­nic ­groups of Mediterranean ori­gin. We ­report two cas­es of FMF in broth­ers liv­ing in Abruzzo, Italy. They ­were ­born ­from con­san­guin­e­ous par­ents and com­plain­ed typ­i­cal symp­toms ­since child­hood. The boy suf­fered ­from one feb­rile ­attack eve­ry ­week; he pre­sent­ed ­three epi­sodes of ­acute scro­tum at age 8 and 9. The eld­er sis­ter ­showed a spon­ta­ne­ous par­tial ­relief dur­ing ado­les­cence. Juvenile rheumatoid arthritis was sus­pect­ed and Aspirin was ­used for ­many ­years with­out any clin­i­cal improve­ment. Treatment ­with col­chi­cine 1 mg/day was estab­lished at age 13 and 17 respec­tive­ly, and a sud­den reduc­tion of fre­quen­cy of ­attacks was ­obtained. A gin­gi­val biop­sy did not ­show amy­loid. The ­three eld­er broth­ers are, at ­present, in ­good ­health. Our expe­ri­ence ­point out the diag­nos­tic dif­fi­cul­ties of FMF expe­cial­ly in a coun­try ­were the dis­ease is uncom­mon.

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