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Öğe Canakinumab: New treatment choice for systemic juvenile idiopathic arthritis(Future Medicine Ltd., 2015) Sozeri B.; Sevgi S.; Kasapcopur O.Juvenile idiopathic arthritis (JIA) is the most common chronic rheumatic disease in children and systemic juvenile idiopathic arthritis (sJIA) accounts for 6-15% of the patients. It was found that there was spontaneous expression of IL-1ß in patients with sJIA. Canakinumab (ACZ885, Canakinumab) is a human anti-IL-1ß monoclonal antibody. Its mode of action is based on the neutralization of IL-1ß signaling which may result in the suppression of inflammation process in patients with disorders of autoinflammatory origin including sJIA. In addition to its use in the treatment of CAPS (cryopyrin-associated periodic syndromes) and acute gouty arthritis flares in many countries, the drug provides significant advantages over existing competitive therapies, including monthly SC administration and favorable safety profile. © 2015 Future Medicine Ltd.Öğe Childhood vasculitides in Turkey: A nationwide survey(2007) Ozen S.; Bakkaloglu A.; Dusunsel R.; Soylemezoglu O.; Ozaltin F.; Poyrazoglu H.; Kasapcopur O.; Ozkaya O.; Yalcinkaya F.; Balat A.; Kural N.; Donmez O.; Alpay H.; Anarat A.; Mir S.; Gur-Guven A.; Sonmez F.; Gok F.Aim: The aims of this study were to evaluate the characteristics of childhood vasculitides and to establish the first registry in Turkey, an eastern Mediterranean country with a white population. Patients and methods: A questionnaire was distributed to the main referral centers asking for the registration of the Henoch-Schönlein purpura (HSP) patients in the last calendar year only and 5 years for other vasculitides. Demographic, clinical, and laboratory data were assessed. Results: Vasculitic diseases were registered from 15 pediatric centers. These centers had a fair representation throughout the country. In the last calendar year, incidences were as follows: HSP 81.6%, Kawasaki disease (KD) 9.0%, childhood polyarteritis nodosa (C-PAN) 5.6%, Takayasu arteritis (TA) 1.5%, Wegener's granulomatosis 0.4%, and Behçet disease 1.9%. There was no clear gender dominance. The mean age was 11.05±4.89 years. Acute phase reactants were elevated in almost all, highest figures being in C-PAN. Renal involvement was present in 28.6% of HSP and 53% of the C-PAN patients. Abdominal aorta was involved in all TA patients. Among the C-PAN patients, 25% had microscopic PAN with necrotizing glomerulonephritis; antineutrophil cytoplasmic antibody was positive in those who were studied. Among the patients, 12.5% and 15% had classic PAN and cutaneous PAN, respectively. The remaining majority were classified as systemic C-PAN diagnosed with biopsies and/or angiograms demonstrating small to midsize artery involvement. The overall prognosis was better than reported in adult series. Conclusion: This is the largest multicenter study defining the demographic data for childhood vasculitides. The distribution of childhood vasculitides was different in our population where KD is much less frequent, whereas HSP constitutes an overwhelming majority. C-PAN was more frequent as well. © Clinical Rheumatology 2006.Öğe Development of a medication adherence scale for familial Mediterranean fever (MASIF) in a cohort of Turkish children(Clinical and Experimental Rheumatology S.A.S., 2015) Yesilkaya S.; Acikel C.; Fidanci B.E.; Polat A.; Sozeri B.; Ayaz N.A.; Makay B.B.; Simsek D.; Akinci N.; özçelik G.; Kavukçu S.; Emre S.; Donmez O.; Delibas A.; Yüksel S.; Berdeli A.; Poyrazoglu H.; Saldir M.; Fidanci K.; çakar N.; Peru H.; Bakkaloglu S.; Tabel Y.; Sari O.; Aydogan U.; Ozenc S.; Basbozkurt G.; Unsal E.; Kasapcopur O.; Gok F.; Ozen S.; Demirkaya E.Objective. To develop and assess the validity and reliability of an adherence scale concerning medical treatment in paediatric FMF patients. Methods. The Medication Adherence Scale in FMF Patients (MASIF) is a 18-item questionnaire that evaluates adherence to medication in four domains. Validation of the instrument was accomplished in paediatric FMF patients (aged 2-18 years) under medication at least for 6 months. The first step was to build up the scale through qualitative approach (with interviews using semi-structured questions). Validation analyses included assessment of feasibility, face and content validity; construct validity, internal consistency and test-retest reliability. Results. One hundred and fifty patients with FMF were enrolled in the study. The mean age of the patients was 11.11±4.02 years and 48.7% of them were male. The MASIF was found to be feasible and valid for both face and content. It correlated with the Morisky Medication Adherence Scale as a gold standard thereby demonstrating good construct validity (r=0.515, p < 0.001). Assessment of content validity identified four subscales. The internal consistency, Cronbach's alpha was 0.728. There was a positive and significant correlation between test and retest scores (r=0.843; p < 0.001). Also, a significant correlation between parents' and children's reports (r=0.781, p < 0.001). Conclusion. Based on these results, the use of this scale to assess and follow up the adherence to treatment in paediatric FMF patients under medical treatment is recommended. © Clinical and Experimental Rheumatology 2015.Öğe Familial Mediterranean Fever (FMF) in Turkey: Results of a nationwide multicenter study(Lippincott Williams and Wilkins, 2005) Tunca M.; Ozdogan H.; Kasapcopur O.; Yalcinkaya F.; Tutar E.; Topaloglu R.; Yilmaz E.; Arici M.; Bakkaloglu A.; Besbas N.; Akpolat T.; Dinc A.; Erken E.; Tirpan K.; Ozer H.T.E.; Soyturk M.; Senturk T.; Balci B.; Ozguc M.; Dundar M.; Akar E.; Ozel D.; Dundar M.; Gunesacar R.; Booth D.R.; Hawkins P.N.; Touitou I.; Aksentijevich I.; Matzner Y.; Arslan S.; Balaban Y.; Batman F.; Bayraktar Y.; Apras S.; Calguneri M.; Duzova A.; Kav T.; Ozaltin F.; Simsek H.; Sivri B.; Tatar G.; Akkoc N.; Kavukcu S.; Soylu A.; Turkmen M.; Unsal E.; Arisoy N.; Caliskan S.; Gogus F.; Masatlioglu S.; Sever L.; Akkok N.; Cakar N.; Kara N.; Kocak H.; Ozalp S.; Bilge I.; Sevinc E.; Gul A.; Kamali S.; Sadikoglu B.; Selcukbiricik F.; Sirin A.; Sucu A.; Bek K.; Bulbul M.; Delibas A.; Demircin G.; Erdogan O.; Oner A.; Mesiha M.; Ozkaya N.; Tekin M.; Demirkaya E.; Erdem H.; Gok F.; Pay S.; Islek I.; Kabasakal Y.; Keser G.; Ozmen M.; Akoglu E.; Atagunduz P.; Direskeneli H.; Temel M.; Tuglular S.; Buyan N.; Bakkaloglu S.; Derici U.; Goker B.; Kalman S.; Ozkaya O.; Dusunsel R.; Gunduz Z.; Poyrazoglu M.H.; Korkmaz C.; Baskin E.; Koseoglu H.K.; Saatci U.; Yucel E.; Coban E.; Yakupoglu G.; Oktem F.; Tunc E.; Cobankara V.Familial Mediterranean fever (FMF) is an autosomal recessive disease that is prevalent among eastern Mediterranean populations, mainly non-Ashkenazi Jews, Armenians, Turks, and Arabs. Since a large proportion of all the FMF patients in the world live in Turkey, the Turkish FMF Study Group (FMF-TR) was founded to develop a patient registry database and analyze demographic, clinical, and genetic features. The cohort was composed of 2838 patients (mean age, 23.0 ± 13.33 yr; range, 2-87 yr), with a male:female ratio of 1.2:1. There was a mean period of 6.9 ± 7.65 years from disease onset to diagnosis; the period was about 2 years shorter for each decade since 1981. Ninety-four percent of patients were living in the central-western parts of the country; however, their familial origins (70% from the central-eastern and Black Sea regions) reflected not only the ongoing east to west migration, but also the historical roots of FMF in Turkey. Patients' clinical features included peritonitis (93.7%), fever (92.5%), arthritis (47.4%), pleuritis (31.2%), myalgia (39.6%), and erysipelas-like erythema (20.9%). Arthritis, arthralgia, myalgia, and erysipelas-like erythema were significantly more frequent (p < 0.001) among patients with disease onset before the age of 18 years. Genetic analysis of 1090 patients revealed that M694V was the most frequent mutation (51.4%), followed by M680I (14.4%) and V726A (8.6%). Patients with the M694V/M694V genotype were found to have an earlier age of onset and higher frequencies of arthritis and arthralgia compared with the other groups (both p < 0.001). In contrast to other reported studies, there was no correlation between amyloidosis and M694V homozygosity in this cohort. However, amyloidosis was still remarkably frequent in our patients (12.9%), and it was prevalent (27.8%) even among the 18 patients with a disease onset after age 40 years. Twenty-two patients (0.8%) had nonamyloid glomerular diseases. The high prevalence of vasculitides (0.9% for polyarteritis nodosa and 2.7% for Henoch-Schönlein purpura) and high frequency of pericarditis (1.4%) were striking findings in the cohort. Phenotype II cases (those patients with amyloidosis as the presenting or only manifestation of disease) were rare (0.3% or less). There was a high rate of a past diagnosis of acute rheumatic fever, which suggested a possible misdiagnosis in children with FMF presenting with recurrent arthritis. To our knowledge, this is the largest series of patients with FMF reported from 1 country. We describe the features of the disease in the Turkish population and show that amyloidosis is still a substantial problem.
