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Familial Mediterranean Fever – First Experiences in Slovakia


Authors: Tomáš Dallos 1;  Lucia Lukáčiková Gálová 2;  Eva Macejková 3;  Jozef Sedlačko 4;  Nataša Toplak 5;  Maruša Debeljak 6;  Hasmik Sargsyan 7;  Denisa Ilenčíková 1;  László Kovács 1
Authors place of work: II. detská klinika Lekárskej fakulty UK a DFNsP, Bratislava, Slovenská republika, prednosta prof. MUDr. László Kovács, DrSc., MPH 1;  Kardiologická klinika FN Nitra, Slovenská republika, prednosta MUDr. Pavol Poliačik, PhD. 2;  Reumatologická ambulancia Bella s. r. o., Banská Bystrica, Slovenská republika, vedúci pracoviska MUDr. Eva Macejková 3;  III. interná klinika Lekárskej fakulty UK a UN Bratislava, Slovenská republika, prednosta prof. MUDr. Viliam Bada, CSc. 4;  Oddelenie alergológie, reumatológie a klinickej imunológie Detskej univerzitnej nemocnice Ljubljana, Slovinsko, prednosta prof. Tadej Avčin, MD, PhD. 5;  Centrum lekárskej genetiky Detskej univerzitnej nemocnice Ljubljana, Slovinsko, vedúci pracoviska Mirjana Župančič, mag. med. bioch. 6;  Detské centrum pre FMF Štátnej lekárskej univerzity Jerevan, Arménsko, vedúci pracoviska Arman A. Babloyan, MD 7
Published in the journal: Vnitř Lék 2014; 60(1): 80-85
Category: Case Report

Summary

Familial Mediterranean fever (FMF) is the most prevalent genetically determined autoinflammatory disease. FMF significantly decreases the quality of life and limits life expectancy due to the development of amyloidosis in affected individuals. Prevalence of FMF is highest in the south-eastern Mediterraneans. In other parts of the world, its occurance is often restricted to high-risk ethnic groups. In Central Europe, experience with FMF is scarse to none, as in the case of Slovakia, where no cases have been reported, so far. Herein we report the first five patients (3 adults and 2 children, 4 native Slovaks) in whom the diagnosis of FMF could be confirmed in Slovakia. Our experience demonstrates that FMF does occur in low-risk populations in Central Europe. Due to low prevalence and lack of experience, FMF diagnosis may be significantly delayed (4.5–30 years) and undiagnosed cases are to be expected in our population.

Key words:
familial mediterranean fever – amyloidosis – colchicin – Slovakia


Zdroje

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Štítky
Diabetology Endocrinology Internal medicine
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