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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 Endocrine complications in patients with ß-thalassemia major(2002) Aydinok Y.; Darcan S.; Polat A.; Kavakli K.; Nişli G.; Çoker M.; Kantar M.; Çetingul N.Thirty-seven patients with thalassemia major (TM) were studied to determine the extent and rate of endocrine complications. Mean haemoglobin and ferritin concentrations were 8.8 ± 0.6 and 3597 ± 1931, respectively. Provocation tests for growth hormone secretion were applied in patients with standing heights below the third centile and/or growth velocities below the 10th centile. Sexual maturation was assessed by using the criteria of Tanner. Glucose metabolism was assessed by fasting plasma glucose and glucose tolerance test. Basal thyroid function was measured and thyrotropin-releasing hormone tolerance test was carried out. Growth retardation was found in 40 per cent of patients and growth hormone deficiency was a prominent cause of growth retardation. Gonadal dysfunction was detected in 47 per cent of patients. Hypothyroidism was observed in 16 per cent and impaired glucose metabolism in 10.8 per cent patients. The high rate of endocrine disturbances indicates the importance of regular follow-up of thalassemia major patients with regard to endocrine complications of the disease. © 2002 Oxford University Press.Öğe Growth curves for Turkish girls with turner syndrome: Results of the Turkish turner syndrome study group(Galenos Yayincilik,, 2015) Darendeliler F.; Yeşilkaya E.; Bereket A.; Baş F.; Bundak R.; Sarı E.; Aydın B.K.; Darcan Ş.; Dündar B.; Büyükinan M.; Kara C.; Mazıcıoğlu M.M.; Adal E.; Akıncı A.; Atabek M.E.; Demirel F.; Çelik N.; Özkan B.; Özhan B.; Orbak Z.; Ersoy B.; Doğan M.; Ataş A.; Turan S.; Gökşen D.; Tarım Ö.; Yüksel B.; Ercan O.; Hatun Ş.; Şimşek E.; Ökten A.; Abacı A.; Döneray H.; Özbek M.N.; Keskin M.; Önal H.; Akyürek N.; Bulan K.; Tepe D.; Emeksiz H.C.; Demir K.; Kızılay D.; Topaloğlu A.K.; Eren E.; Özen S.; Demirbilek H.; Abalı S.; Akın L.; Eklioğlu B.S.; Kaba S.; Anık A.; Baş S.; Ünüvar T.; Sağlam H.; Bolu S.; Özgen T.; Doğan D.; Çakır E.D.; Şen Y.; Andıran N.; Çizmecioğlu F.; Evliyaoğlu O.; Karagüzel G.; Pirgon Ö.; Çatlı G.; Can H.D.; Gürbüz F.; Binay Ç.; Baş V.N.; Sağlam C.; Gül D.; Polat A.; Açıke C.; Cinaz P.Objective: Children with Turner syndrome (TS) have a specific growth pattern that is quite different from that of healthy children. Many countries have population-specific growth charts for TS. Considering national and ethnic differences, we undertook this multicenter collaborative study to construct growth charts and reference values for height, weight and body mass index (BMI) from 3 years of age to adulthood for spontaneous growth of Turkish girls with TS. Methods: Cross-sectional height and weight data of 842 patients with TS, younger than 18 years of age and before starting any therapy, were evaluated. Results: The data were processed to calculate the 3rd, 10th, 25th, 50th, 75th, 90th and 97th percentile values for defined ages and to construct growth curves for height-for-age, weight-for-age and BMI-for-age of girls with TS. The growth pattern of TS girls in this series resembled the growth pattern of TS girls in other reports, but there were differences in height between our series and the others. Conclusion: This study provides disease-specific growth charts for Turkish girls with TS. These disease-specific national growth charts will serve to improve the evaluation of growth and its management with growth-promoting therapeutic agents in TS patients. © Journal of Clinical Research in Pediatric Endocrinology, Published by Galenos Publishing.Öğe Hepatitis C virus and the treatment of hemophilia in Turkey [1](Blackwell Publishing Inc., 1998) Kavakli K.; Nisli G.; Polat A.; Aydinok Y.; Ozacar T.[No abstract available]Öğe Long-term Prophylaxis and immune parameters in haemophilia [5](Oxford University Press, 1999) Kavakli K.; Nisli G.; Kutukculer N.; Polat A.; Aydinok Y.; Oztop S.; Centingul N.[No abstract available]Öğe A new chromosomal rearrangement in acute myeloid leukemia [2](1998) Cetingul N.; Oniz H.; Cogulu O.; Özkınay F.; Kavakli K.; Kantar M.; Aydinok Y.; Polat A.; Nisli G.; Oztop S.[No abstract available]Öğe Plasma platelet-activating factor (PAF) levels and desmopressin response in children with hemophilia A and von Willebrand's disease [8](1999) Kavakli K.; Polat A.; Huseyinof A.; Nisli G.; Aydinok Y.[No abstract available]Öğe Urinary zinc excretion and zinc status of patients with ß-thalassemia major(Humana Press, 1999) Aydinok Y.; Coker C.; Kavakli K.; Polat A.; Nisli G.; Cetiner N.; Kantar M.; Çetingül N.In this study, zinc status and urinary zinc excretion with and without desferrioxamine (DFO) infusion and the relationship between urinary zinc excretion and renal tubular dysfunction in thalassemia major (TM) patients were investigated. Forty TM patients were given four DFO infusions on alternate days over a 1-wk period prior to the transfusion. On each day that DFO was given, a 24-h urine collection initiated. DFO was omitted for 1-wk before the following transfusion and during the period four 24-h urine collections were performed. Twenty healthy children provided 24-h urine collection as controls. Blood samples were taken on each of two consecutive transfusion days of the patients and from the controls. Urinary zinc excretion was measured and plasma and red blood cell (RBC) zinc analysis were performed by inductively coupled plasma-atomic emission spectrophotometry. Urinary N-acetyl-ß-D-glucosaminidase (NAG) activity and creatinine were determined in morning urine specimens. The mean plasma zinc concentration was significantly lower in the patients not given DFO compared to the values of the patients given DFO and the control group. The mean RBC zinc concentration (µmol/g Hb) in the patients (with and without DFO) and the control group were similar. Urinary zinc excretion was significantly higher in the patients receiving DFO compared to the control group, whereas urinary zinc excretion in the patients not given DFO was not different from the controls. Urinary NAG indices (U/g Cr) were significantly higher in the patients compared to controls. Urinary zinc excretion was correlated with the urinary NAG indices.
