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Öğe Clinical and Laboratory Characteristics of Hyperprolactinemia in Children and Adolescents: National Survey(Galenos Yayincilik, 2019) Eren, Erdal; Ergur, Ayca Torel; Isguven, Sukriye Pinar; Bitkin, Eda Celebi; Berberoglu, Merih; Siklar, Zeynep; Bas, FirdevsObjective: We aimed to report the characteristics at admission, diagnosis, treatment, and follow-up of cases of pediatric hyperprolactinemia in a large multicenter study. Methods: We reviewed the records of 233 hyperprolactinemic patients, under 18 years of age, who were followed by different centers. The patients were divided as having microadenomas, macroadenomas, drug-induced hyperprolactinemia and idiopathic hyperprolactinemia. Complaints of the patients, their mode of treatment (medication and/or surgery) and outcomes were evaluated in detail. Results: The mean age of the patients with hyperprolactinemia was 14.5 years, and 88.4% were females. In terms of etiology, microadenomas were observed in 32.6 %, macroadenomas in 27 %, idiopathic hyperprolactinemia in 22.7% and drug-induced hyperprolactinemia in 6.4 %. Other causes of hyperprolactinemia were defined in 11.3%. Common complaints in females (n = 206) were sorted into menstrual irregularities, headaches, galactorrhea, primary or secondary amenorrhea and weight gain, whereas headache, gynecomastia, short stature and blurred vision were common in males (n = 27). Median prolactin levels were 93.15 ng/mL, 241.8 ng/ml, 74.5 ng/mL, 93.2 ng/mL, and 69 ng/mL for microadenomas, macroadenomas, idiopathic hyperprolactinemia, drug-induced hyperprolactinemia, and other causes of hyperprolactinemia, respectively. Of 172 patients with hyperprolactinemia, 77.3 % were treated with cabergoline and 13.4 % with bromocriptine. 20.1 % of the patients with pituitary adenomas underwent pituitary surgery. Conclusion: We present the largest cohort of children and adolescents with hyperprolactinemia in the literature to date. Hyperprolactinemia is more common in females and cabergoline is highly effective and practical to use in adolescents, due to its biweekly dosing. Indications for surgery in pediatric cases need to be revised.Öğe CLINICAL AND LABORATORY CHARACTERISTICS OF HYPERPROLACTINEMIC CHILDREN AND ADOLESCENTS: NATIONAL SURVEY(Karger, 2017) Eren, Erdal; Ergur, Ayca Torel; Isguven, Sukriye Pinar; Bitkin, Eda Celebi; Berberoglu, Merih; Siklar, Zeynep; Genens, Mikayir[Abstract Not Available]Öğe Clinical features, diagnosis and treatment outcomes of Cushing's disease in children: A multicenter study(John Wiley and Sons Inc, 2023) Tarçın, Gürkan; Çatlı, Gönül; Çetinkaya, Semra; Eren, Erdal; Kardelen, Aslı Derya; Akıncı, Ayşehan; Yıldırım, RukenObjective: Since Cushing's disease (CD) is less common in the paediatric age group than in adults, data on this subject are relatively limited in children. Herein, we aim to share the clinical, diagnostic and therapeutic features of paediatric CD cases. Design: National, multicenter and retrospective study. Patients: All centres were asked to complete a form including questions regarding initial complaints, physical examination findings, diagnostic tests, treatment modalities and follow-up data of the children with CD between December 2015 and March 2017. Measurements: Diagnostic tests of CD and tumour size. Results: Thirty-four patients (M:F = 16:18) from 15 tertiary centres were enroled. The most frequent complaint and physical examination finding were rapid weight gain, and round face with plethora, respectively. Late-night serum cortisol level was the most sensitive test for the diagnosis of hypercortisolism and morning adrenocorticotropic hormone (ACTH) level to demonstrate the pituitary origin (100% and 96.8%, respectively). Adenoma was detected on magnetic resonance imaging (MRI) in 70.5% of the patients. Transsphenoidal adenomectomy (TSA) was the most preferred treatment (78.1%). At follow-up, 6 (24%) of the patients who underwent TSA were reoperated due to recurrence or surgical failure. Conclusions: Herein, national data of the clinical experience on paediatric CD have been presented. Our findings highlight that presenting complaints may be subtle in children, the sensitivities of the diagnostic tests are very variable and require a careful interpretation, and MRI fails to detect adenoma in approximately one-third of cases. Finally, clinicians should be aware of the recurrence of the disease during the follow-up after surgery.Öğe Molecular diagnosis of monogenic diabetes and clinical/laboratory features in Turkish children(Galenos Yayıncılık, 2021) Gökşen, Damla; Yeşilkaya, Ediz; Özen, Samim; Kor, Yılmaz; Eren, Erdal; Korkmaz, Özlem; Ünal, EdipObjective: Monogenic diabetes is a heterogeneous disease that causes functional problems in pancreatic beta cells and hyperglycemia. The aim of this study was to determine the clinical and laboratory features, the admission characteristics and distribution of monogenic form of diabetes in childhood in Turkey. Methods: Patients aged 0-18 years, who were molecularly diagnosed with monogenic diabetes, and consented to participate, were included in the study. Results: Seventy-seven (45.6%) female and 92 male cases with a mean age of 8.18 +/- 5.05 years at diagnosis were included. 52.7% of the cases were diagnosed with monogenic diabetes by random blood glucose measurement. The reason for genetic analysis in 95 (56.2%) of cases was having a family member diagnosed with diabetes under the age of 25. At the time of diagnosis, ketone was detected in urine in 16.6% of the cases. Mean hemoglobin A1c on admission, fasting blood glucose, fasting insulin, and c-peptide values were 7.3 +/- 2.1%, 184.9 +/- 128.9 mg/dL, 9.4 +/- 22.9 IU/L, 1.36 +/- 1.1 and ng/L respectively. GCK-MODY was found in 100 (59.2%), HNF1A-MODY in 31 (18.3%), and variants in ABCC8 in 6 (3.6%), KCNJ11 in 5 (3%), HNF4A in 2 (1.2%), and HNF1B in 2 (1.2%). Conclusion: Recent studies have indicated HNF1A-MODY is the most frequent of all the MODY-monogenic diabetes cases in the literature (50%), while GCK-MODY is the second most frequent (32%). In contrast to these reports, in our study, the most common form was GCK-MODY while less than 20% of cases were diagnosed with HNF1A-MODY.Öğe Nationwide Turkish cohort study of hypophosphatemic rickets(Galenos Yayincilik, 2020) Şıklar, Zeynep; Turan, Serap; Bereket, Abdullah; Baş, Firdevs; Güran, Tülay; Akberzade, Azad; Abacı, Ayhan; Demir, Korcan; Böber, Ece; Özbek, Mehmet Nuri; Kara, Cengiz; Poyrazǒglu, Şükran; Aydın, Murat; Kardelen, Aslı Derya; Tarım, Ömer Faruk; Eren, Erdal; Hatipoǧlu, Nihal; Büyükinan, Muammer; Akyürek, Nesibe; Çetinkaya, Semra Çaǧlar; Bayramoǧlu, Elvan; Eklioǧlu, Beray Selver; Uçaktürk, Ahmet; Abali, Saygin; Gökşen, Damla; Kör, Yilmaz; Ünal, Edip; Esen, İhsan; Yıldırım, Ruken; Akın, Önur; Çayır, Atilla; Dilek, Emine; Kırel, Birgül; Anık, Ahmet; Çatlι, Gönül; Berberoǧlu, MerihObjective: Hypophosphatemic rickets (HR) is a rare renal phosphate-wasting disorder, which is usually X-linked and is commonly caused by PHEX mutations. The treatment and follow-up of HR is challenging due to imperfect treatment options. Methods: Here we present nationwide initial and follow-up data on HR. Results: From 24 centers, 166 patients were included in the study. Genetic analysis (n=75) showed PHEX mutation in 80% of patients. The mean follow-up period was 6.7±2.4 years. During the first 3-years of treatment (n=91), mild increase in phosphate, decrease in alkaline phosphatase and elevation in parathyroid hormone (PTH) levels were detected. The height standard deviation scores were-2.38,-2.77,-2.72,-2.47 at initial, 1st, 2nd and 3rd year of treatment, respectively (p>0.05). On follow-up 36% of the patients showed complete or significant improvement in leg deformities and these patients had similar phosphate levels at presentation with better levels in 1st and 2nd years of treatment; even the treatment doses of phosphate were similar. Furthermore, 27 patients developed nephrocalcinosis (NC), the patients showed no difference in biochemical differences at presentation and follow-up, but 3rd year PTH was higher. However, higher treatment doses of phosphate and calcitriol were found in the NC group. Conclusion: HR treatment and follow-up is challenging and our results showed higher treatment doses were associated with NC without any change in serum phosphate levels, suggesting that giving higher doses led to increased phosphaturia, probably through stimulation of fibroblast growth factor 23. However, higher calcitriol doses could improve bone deformities. Safer and more efficacious therapies are needed.Öğe Türkiye'de bilgi edinme hakkı ve uygulaması(2015) Eren, ErdalBilgi edinme hakkı düşünce hürriyetinin bir parçasıdır. Bilgi edinme hakkı olmadan düşünce hürriyeti düşünülemez. Bu kavram İçinde bulunduğumuz çağın ortaya çıkardığı yönetim anlayışının ve Anayasal gereksinimlerin sonucu olan yeni bir kavramdır.Bilgi edinme hakkı insan yaşamının olmazsa olmazı olup, onun yaşamını düzenleyen ve geleceğini planlamasını sağlayan bir haktır. Bu hak, bazı ülkelerde doğrudan anayasal güvence altına alınırken, bazılarında ise sadece yasal düzenleme yapılmakla yetinilmiştir. Ayrıca birçok uluslararası düzenlemeye konu olmuştur. Son yüzyılda bilgi edinme hakkının önemi birçok ulusça anlaşılmış durumdadır. İnsanın sadece insan olması dolayısıyla sahip olduğu kişiliğine bağlı, dokunulmaz, devredilmez, vazgeçilmez, zamanaşımına uğramaz hak ve hürriyetler olarak nitelendirilen insan hakkı kavramı herkes için tanınmış olmasına rağmen, bütün insanların bu haklardan istifadesi hemen olamamıştır. Söz konusu hakkın elde edilebilmesi için büyük mücadeleler yapılmıştır. Bilgi edinme hakkı, kamu yönetiminin bir ideali haline gelen saydam, şeffaf, katılımcı yönetimin en önemli araçlarından biridir. Ülkemizde vatandaşın bilgi edinme hakkını ve idarenin açıklığını düzenlemeye yönelik dilekçe hakkından önce pek de kayda değer düzenlemeler bulunmamaktadır. Nihayetinde bilgi edinme hakkına ilişkin düzenleme ülkemizde de yapılarak yürürlüğe girmiştir. Söz konusu düzenleme bilgi edinme hakkına ilişkin en önemli adımdır. Bilgi edinme hakkının iyi bir yönetiminin anahtarı olduğu unutulmamalıdır. Sosyal devlet anlayışının, herkesin insanca yaşaması için olmazsa olmazı bilgi edinme hakkıdır. Anahtar Sözcükler 1. Bilgi Edinme 2. Bilgi Edinme Hakkı 3. İnsan Hakları
