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OBJECTIVE To characterize the clinical and genetic features of familial Mediterranean fever (FMF). STUDY DESIGN Clinical presentation and MEditerranean FeVer mutation type of all patients with FMF, who first manifested the disease at < or =2 years of age were analyzed and compared with patients who first presented with FMF between 2 and 16 years. (More)
OBJECTIVE Familial Mediterranean fever (FMF) is an inherited disease characterized by attacks of febrile polyserositis. In children, attacks of fever alone, or with headache and malaise, may precede other forms of attacks. Our objective was clinical and genetic characterization of FMF and its development in pediatric patients who first presented with(More)
Cardiac amyloid deposition in FMF may cause increased QT dispersion (QTd), a marker for cardiac arrhythmias. The aim of this study was to further evaluate repolarization dispersion in familial Mediterranean fever (FMF) with amyloidosis. Findings on 12-lead electrocardiography were compared between 18 patients with FMF-amyloidosis and 18 age- and sex-matched(More)
The aim of the study was to further evaluate repolarization dispersion in familial Mediterranean fever (FMF). Findings on 12-lead electrocardiography were compared with 32 patients with uncomplicated FMF and age- and sex-matched control subjects. All procedures followed stringent standards. Repolarization and dispersion parameters were computed with(More)
BACKGROUND To demonstrate and clinically, genetically and demographically characterize familial Mediterranean fever (FMF) patients, maintaining remission despite colchicine abstinence. METHODS FMF patients were screened for an endurance of prolonged remission (≥ 3 years), despite refraining from colchicine. Clinical, demographic and genetic parameters(More)
Familial Mediterranean fever (FMF) is a hereditary disease, characterized by recurrent episodes of fever and polyserositis. Heart rate variability (HRV) is a powerful, simple and reliable technique to evaluate autonomic nervous system function. Previous studies of physiologic parameters during tilt-test have suggested that patients with FMF have abnormal(More)
BACKGROUND 'Erysipelas-like' erythema (ELE) is a well recognized, although uncommon, manifestation of familial Mediterranean fever (FMF), which is frequently mistaken for infectious erysipelas, especially when forming the initial disease presentation. AIM To clinically and genetically characterize ELE as the first manifestation of FMF. METHODS FMF(More)
OBJECTIVE Familial Mediterranean fever (FMF) is refractory to colchicine prophylaxis in 10-20% of patients. In a number of patient series, treatment with anakinra, an interleukin-1-blocking agent, prevented FMF attacks in those with colchicine-resistant FMF. This study was undertaken to evaluate the efficacy and safety of anakinra in the treatment of(More)
We present a case of Rickettsia typhi infection (the causative agent of endemic typhus) associated with an isolated splenic infarction. Large vessel infarction is a rare complication of murine typhus, unlike infections caused by Rickettsia rickettsii and Rickettsia conorii (the spotted fever group rickettsias) which are known to cause hemostatic changes(More)
BACKGROUND A scarcity of data exists relating to the effect of amyloidosis of Familial Mediterranean fever (FMF) on the autonomic nervous system. Our aim was to further investigate the presence of dysautonomia in FMF-AA amyloidosis, using a comparative case series design. MATERIAL AND METHODS The study group consisted of 40 patients with FMF: 20 without(More)