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Ucakturk A.,Ankara Childrens Hematology and Oncology Training Hospital | Avci B.,Ondokuz Mayis University | Genc G.,Ondokuz Mayis University | Ozkaya O.,Ondokuz Mayis University | Aydin M.,Ondokuz Mayis University
Journal of Pediatric Endocrinology and Metabolism | Year: 2016

Background: We aimed at evaluating the urinary levels of kidney injury molecule-1 ( KIM-1) and neutrophil gelatinase associated lipocalin (NGAL), and the relationship between these markers and clinical and laboratory variables in normoalbuminuric children with type 1 diabetes (T1D). Methods: The study group consisted of 60 (F/M: 28/32) children with T1D with a median age of 13 (min: 7.1-max: 17.9) years and a mean HbA1c of 8.6%. The average period of treatment was 6.8±2.2 years. The control group consisted of 60 healthy children [(F/M: 32/28); median age: 13.6 (min: 6.9-max: 17.9) years]. Results: Urinary KIM-1 and NGAL levels were significantly elevated in the diabetic group (KIM-1: 0.50±0.34 ng/mg-cr; NGAL: 33±31 ng/mg-cr) compared with the nondiabetic control subjects (KIM-1: 0.26±0.25 ng/mg-cr, NGAL 13.3±14.5 ng/mg-cr) (p<0.001). No significant associations were observed between NGAL or KIM-1 and the duration of diabetes and HbA1c levels. NGAL was found to be weakly correlated with KIM-1 (p<0.005, r=0.289). Conclusions: NGAL and KIM are high in normoalbuminuric diabetic children before reduction in glomerular filtration rate. High NGAL and KIM-1 levels may indicate early diabetic kidney injury; however, we did not observe any relationship between these markers and diabetic indices. For clinical usefulness of these markers, long-term studies are required. © 2016 by De Gruyter. Source


Komsu-Ornek Z.,Zonguldak Karaelmas University | Demirel F.,Ankara Childrens Hematology and Oncology Training Hospital | Dursun A.,Zonguldak Karaelmas University | Ermis B.,Sakarya University | And 2 more authors.
Turkish Journal of Pediatrics | Year: 2012

The aim of the study was to investigate the relationship between leptin receptor gene (LEPR) Gln223Arg polymorphism and obesity in Turkish children. Ninety-two obese and 99 lean children (between 5-15 years) were included in the study. Twenty-three of the obese children were diagnosed with metabolic syndrome. Blood samples were collected for morning fasting blood glucose, insulin, leptin, and lipid level measurements. LEPR Gln223Arg polymorphism was analyzed by restriction fragment length polymorphism. Significant differences were observed in anthropometric measurements, fasting blood glucose, insulin, leptin, and lipid levels between obese and lean children. Serum leptin levels were markedly higher in obese children. No significant association was noted between Gln223Arg polymorphism and serum leptin, insulin and lipid levels. There were no differences in the genotype frequencies or allele distribution for Gln223Arg polymorphism among obese, obese with metabolic syndrome and lean children. Our findings suggest that there is no association between Gln223Arg polymorphism and obesity in Turkish children. Source


Akman A.O.,Ankara Childrens Hematology and Oncology Training Hospital | Basaran O.,Ankara Childrens Hematology and Oncology Training Hospital | Ozyoruk D.,Ankara Childrens Hematology and Oncology Training Hospital | Han U.,Ankara Dskap Yldrm Beyazt Training and Research Hospital | And 2 more authors.
European Journal of Pediatrics | Year: 2016

Castleman disease (CD) is a rare poorly understood lymphoproliferative disorder. Pediatric onset CD has been reported before. However, most of them have benign unicentric pattern. Multicentric CD (MCD) is quite rare in children. Herein, we report a 13-year-old adolescent boy with MCD of the hyaline vascular variant presenting with pleural and pericardial effusion, which is an uncommon presentation. Conclusion: MCD should be considered in the differential diagnosis of pleural and/or pericardial effusion with unexplained lymph nodes in children.What is Known•Pediatric Castleman disease (CD) most commonly occurs in the unicentric form, which typically is asymptomatic and cured by lymph node excision.•The diagnosis of MCD can be difficult owing to the heterogeneity of presentation and potential for nonspecific multisystem involvement.What is New•A 13-year-old adolescent boy was diagnosed with MCD of the hyaline vascular variant presenting with pleural and pericardial effusion, which is an uncommon presentation.•In a pediatric patient with fever, pleural-pericardial effusion and multiple lymph nodes, MCD should be considered in differantial diagnosis. © 2015, Springer-Verlag Berlin Heidelberg. Source


Demirbilek H.,Great Ormond Street Hospital for Children NHS Trust | Demirbilek H.,University College London | Arya V.B.,Paediatric Endocrinology | Arya V.B.,University College London | And 14 more authors.
European Journal of Endocrinology | Year: 2014

Objective: Congenital hyperinsulinism (CHI) is the commonest cause of hyperinsulinaemic hypoglycaemia in the neonatal, infancy and childhood periods. Its clinical presentation, histology and underlying molecular biology are extremely heterogeneous. The aim of this study was to describe the clinical characteristics, analyse the genotype-phenotype correlations and describe the treatment outcome of Turkish CHI patients. Design and methods: A total of 35 patients with CHI were retrospectively recruited from four large paediatric endocrine centres in Turkey. Detailed clinical, biochemical and genotype information was collected. Results: Diazoxide unresponsiveness was observed in nearly half of the patients (n=17; 48.5%). Among diazoxide-unresponsive patients, mutations in ABCC8/KCNJ11 were identified in 16 (94%) patients. Among diazoxide-responsive patients (n=18), mutations were identified in two patients (11%). Genotype-phenotype correlation revealed that mutations in ABCC8/KCNJ11 were associated with an increased birth weight and early age of presentation. Five patients had p.L1171fs (c.3512del) ABCC8 mutations, suggestive of a founder effect. The rate of detection of a pathogenic mutation was higher in consanguineous families compared with non-consanguineous families (87.5 vs 21%; P<0.0001). Among the diazoxide-unresponsive group, ten patients were medically managed with octreotide therapy and carbohydrate-rich feeds and six patients underwent subtotal pancreatectomy. There was a high incidence of developmental delay and cerebral palsy among diazoxide-unresponsive patients. Conclusions: This is the largest study to report genotype-phenotype correlations among Turkish patients with CHI. Mutations in ABCC8 and KCNJ11 are the commonest causes of CHI in Turkish patients (48.6%). There is a higher likelihood of genetic diagnosis in patients with early age of presentation, higher birth weight and from consanguineous pedigrees. © 2014 European Society of Endocrinology. Source


Oden Akman A.,Ankara Childrens Hematology and Oncology Training Hospital | Tayfun M.,Ankara Childrens Hematology and Oncology Training Hospital | Demirel F.,Yildirim Beyazit University | Ucakturk S.A.,Ankara Childrens Hematology and Oncology Training Hospital | Gungor A.,Ankara Childrens Hematology and Oncology Training Hospital
Journal of Pediatric and Adolescent Gynecology | Year: 2015

Background: Van Wyk Grumbach syndrome (VWGS) and Kocher-Debre Semelaigne syndrome (KDSS) are rare forms of pseudo-precocious puberty and myopathy in patients with longstanding untreated hypothyroidism. We present the case of an adolescent girl who developed pseudo-precocious puberty and myopathy caused by long-term untreated hypothyroidism. Case: A 17-year-old female patient was referred to our outpatient clinic due to menstrual irregularities. She had muscle pain and multiple cystic ovaries in addition to clinical signs of severe congenital hypothyroidism. High levels of creatinine and creatinine kinase and accompanying muscle hypertrophy were present. After the initiation of l-thyroxine therapy, the symptoms were alleviated in a short time. Conclusion: Congenital and acquired hypothyroidism should be considered in the differential diagnosis of pseudo-precocious puberty and myopathy that presents with muscle pain, muscle hypertrophy, and elevated creatinine kinase levels. © 2015 North American Society for Pediatric and Adolescent Gynecology. Source

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