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Öğe Adult height in Turkish patients with Turner syndrome without growth hormone treatment(2008) Bereket A.; Turan S.; Elçioglu N.; Hacihanefioglu S.; Memioglu N.; Baş F.; Bundak R.; Darendeliler F.; Günöz H.; Saka N.; Ercan O.; Arslanoglu I.; Işgüven P.; Yildiz M.; Can Ş.; Özerkan E.; Çoker M.; Darcan Ş.; Özkan B.; Orbak Z.; Öztaş S.; Palandüz Ş.; Sezgin I.; Atabek E.; Erkul I.; Erdogan G.Spontaneous adult height (AH) in Turner syndrome (TS) varies among populations. Population-specific AH data is essential to assess the efficacy of growth-promoting therapies in TS. A multicenter study was performed to establish AH of non-growth hormone (GH)-treated Turkish patients with TS. One hundred ten patients with TS (diagnosed by karyotype) who reached AH (no growth in the previous year, or bone age >15 years) without receiving GH treatment were included in the study. The average AH was found to be 141.6±7.0 cm at the age of 22.9±6.2 years, which is 18.4 cm below the population average and 16.4 cm below the patients' mid-parental heights. Bone age at start of estrogen replacement was 12.3±1.3 year. Karyotype distribution of the patients was 45X (43%), 45X/46XX (16%), 45X/46Xi (12%), 45XiXq (10%) and others (19%). When the patients were evaluated according to their karyotype as 45X and non-45X, no significant difference in AH was observed (142.4±6.9 cm vs 140.9±7.1 cm, respectively). Adult height of non-GH-treated Turkish TS patients obtained in this study was comparable to that of other Mediterranean populations, but shorter than that of Northern European patients. Karyotype does not seem to affect AH in TS.Öğe The effect of growth hormone treatment on bone mineral density in prepubertal girls with Turner syndrome: A multicentre prospective clinical trial(2008) Aycan Z.; Cetinkaya E.; Darendeliler F.; Vidinlisan S.; Bas F.; Bideci A.; Demirel F.; Darcan S.; Buyukgebiz A.; Yildiz M.; Berberoglu M.; Bundak R.Background: Patients with Turner syndrome (TS) are treated with GH to increase adult height. Although it is well established that GH promotes longitudinal bone growth, the effects of GH treatment on bone density are less clear. Objective: To determine how GH treatment affects trabecular bone mineral density (BMD) in girls with TS at prepubertal ages in a prospective multicentre study. Patients and method: Twenty-two patients with TS in the prepubertal period with a mean age of 9.8 ± 2.5 (range 3.6-12.8) years were included in the study. All girls with TS underwent measurement of areal BMD using dual-energy X-ray absorptiometry (DXA) to obtain pretreatment anteroposterior (AP) lumbar spine values at L1-L4. Patients received GH (Genotropin) subcutaneously for 1 year at a dose of 0.05 mg/kg/day. Height and weight were measured at 3-monthly intervals. The AP lumbar spine areal BMD was remeasured using the same technique after 1 year of treatment. Lumbar spine BMD Z-scores and volumetric BMD (vBMD) Z-scores were calculated using national standards. Results: The height SDS of our cases showed a significant increase with GH therapy. The pretreatment lumbar spine (L1-L4) BMD Z-score was -1.2 ± 1.2 SD and the vBMD Z-score was -0.8 ± 1.6 SD. There were no significant changes in these values after 1 year of GH treatment. Prepubertal TS girls more than 11 years of age had lower vBMD Z-scores (-1.7 ± 1.7 SD) than the girls aged less than 11 (-0.1 ± 1.0 SD) (P < 0.05) at the onset of therapy. No significant changes were observed in these values after 1 year of GH therapy. Conclusions: Osteopaenia becomes apparent in prepubertal TS patients as they reach pubertal age. BMD evaluation may be necessary in these prepubertal TS girls at diagnosis. Short-term GH therapy in these TS patients does not have a significant effect on bone density when measured at a site with a predominance of trabecular bone. © 2007 The Authors.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 Response to growth hormone with respect to pubertal status on increased dose in idiopathic growth hormone deficiency: An analysis of Turkish children in the KIGS database (Pfizer International Growth Study)(Freund Publishing House Ltd, 2005) Darendeliler F.; Berberoglu M.; Öcal G.; Adiyaman P.; Bundak R.; Günöz H.; Baş F.; Darcan Ş.; Gökşen D.; Arslanoglu I.; Yildiz M.; Ercan O.; Ercan G.; Özerkan E.; Can Ş.; Böber E.; Adal E.; Sarikaya S.; Dallar Y.; Şiklar Z.; Bircan I.; Bideci A.; Yüksel B.; Büyükgebiz A.Aim: To compare the growth response to growth hormone (GH) treatment in patients with idiopathic GH deficiency (IGHD) who were prepubertal with the response of those who were pubertal at the onset of GH therapy on an increased GH dose. Patients and methods: Among the Turkish patients enrolled in the Pfizer International Growth Study (KIGS) database with the diagnosis of IGHD, the growth data over 2 years of GH therapy were analyzed longitudinally of 113 (79 M) prepubertal (Group 1) and 44 (33 M) pubertal (Group 2) patients. Pubertal signs were reported to be present initially or to have appeared within 6 months of GH therapy in Group 2. Mean ± SD age at onset of therapy was 8.7 ± 3.5 and 13.5 ± 1.8 years; height SDS -4.2 ± 1.4 and -3.2 ± 1.1 (p <0.05) in Groups 1 and 2, respectively. Mid-parental height (MPH) SDS did not show a significant difference between the two groups (4.5 ± 1.1 vs -1.7 ± 1.1). Results: Delta height SDS over 2 years of therapy was significantly higher in Group 1 (1.1 ± 1.0) than in Group 2 (0.7 ± 0.6) (p <0.05) in spite of a significantly lower dose of GH (14.6 ± 3.3 in Group 1 vs 17.0 ± 3.1 IU/m2/week in Group 2, p <0.05). Ht - MPH SDS showed an increase from -2.4 ± 1.7 to -1.4 ± 1.5 in Group 1 and from -1.5 ± 1.5 to -0.8 ± 1.3 in Group 2. Overall delta height SDS showed negative correlations with age (r = -0.32), height SDS (r = -0.41) and height - MPH SDS (r = -0.40) at onset of therapy (p <0.001). Conclusions: These data show that in IGHD the slight increase (15-20%) in the dose of GH during puberty was not adequate to maintain height velocity at the same magnitude as in prepuberty, and thus was not cost effective. © Freund Publishing House Ltd., London.Öğe Serum IGF-1 and IGFBP-3 levels in healthy children between 0 and 6 years of age(2011) Yüksel B.; Özbek M.N.; Mungan N.O.; Darendeliler F.; Budan B.; Bideci A.; Çetinkaya E.; Berberoglu M.; Evliyaoglu O.; Yeflilkaya E.; Arslanoglu L.; Darcan F.; Bundak R.; Ercan O.Objective: Along with growth hormone (GH) levels, measurements of serum insulin-like growth factor-1 (IGF-1) and IGF-binding protein-3 (IGFBP-3) are used in the diagnosis of GH deficiency and in monitoring the efficacy and safety of long-term GH treatment. The purpose of the present study was to establish reference values for serum IGF-1 and IGFBP-3 in healthy Turkish children less than 6 years of age. Methods: This study was designed as a multicenter project. Five hundred sixty-seven healthy children younger than 6 years of age from different geographical regions of Turkey, with weight and height values between the 10 th and 90 th percentiles according to the national standards were included in the study. In addition to anthropometric parameters, serum IGF-1 and IGFBP-3 levels were measured in all subjects. Results: Although not statistically significant, the serum IGF-1 levels in infants at age 6 months were lower than those in infants at age 3 months. The IGF-1 levels showed a slow increase with age. Serum IGF-1 levels were lower in girls as compared to boys only at age 6 months. No correlation was found between either serum IGFBP-3 levels and body mass index (BMI) or serum IGFBP-3 and weight and height standard deviation scores (SDS). A weak correlation was observed between serum IGF-1 and IGFBP-3 concentrations. Conclusions: The age- and gender-specific reference values for serum IGF-1 and IGFBP-3 reported in this study will aid in the diagnosis of GH deficiency and in the monitoring of children receiving GH treatment. © Journal of Clinical Research in Pediatric Endocrinology, Published by Galenos Publishing.Öğe Target height estimation in children with idiopathic short stature who are referred to the growth clinic(2009) Poyrazoglu S.; Darendeliler F.; Bas F.; Bundak R.; Saka N.; Darcan S.; Wit J.M.; Gunoz H.Aim: It was the aim of this study to evaluate adult height (AH) and different methods used for estimation of target height (TH) in children with idiopathic short stature (ISS). Methods: Eighty-five ISS children (36 female, 49 male) were followed until AH was evaluated retrospectively. TH was calculated according to the following 4 methods: (1) as ±6.5 cm to the mean parental heights for boys or girls, respectively, (2) as the mean standard deviation score (SDS) of the parents' heights, (3) as the sum of the SDS of the parents' heights divided by 1.61, and (4) as the mean SDS of the parents' heights multiplied by 0.72. ISS was classified as familial short stature (FSS) if the height was within the TH range and as nonfamilial short stature (NFSS) if it was below the TH range. Results: The number of FSS and NFSS children differed by the method chosen. The mean AH SDS was lower than the TH SDS in FSS in all methods, except in method 3. NFSS children did not attain their TH in either of the methods. Conclusions: Classification of ISS depends on the method of the TH range chosen. ISS children reach a mean AH SDS lower than the mean TH SDS. Only FSS children classified by method 3 reached a mean AH SDS close to the mean TH SDS. Copyright © 2009 S. Karger AG, Basel.
