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Öğe 46,XX maleness and 46,XX 21-hydroxylase deficiency in dizygotic twins: Association or coincidence?(FREUND PUBLISHING HOUSE LTD, 2007) Atabek, Mehmet Emre; Acar, Hasan; Cora, Tulin; Pirgon, Ozgur46,XX maleness is a rare abnormality of gonadal differentiation. We present dizygotic twins, one having ambiguous genitalia due to 21-hydroxylase deficiency, and the other having normal male genitalia with 46,XX maleness. One of the twins was referred to the endocrinology unit at 2 days old because of ambiguous genitalia. The other twin with bilateral undescended testes located in the inguinal canal was diagnosed with 46,XX maleness. The karyotype was 46,XX. Molecular analysis revealed the presence of SRY in the latter twin without Mullerian structures. Association of congenital adrenal hyperplasia (46,XX 21-hydroxylase deficiency) and 46,XX maleness in twins has not been previously reported.Öğe Achondroplasia associated with metabolic syndrome: Patient report(BLACKWELL PUBLISHING, 2008) Pirgon, Ozgur; Atabek, Mehmet Emre; Sert, Ahmet[Abstract not Available]Öğe Assessment of insulin sensitivity from measurements in fasting state and during an oral glucose tolerance test in obese children(WALTER DE GRUYTER GMBH, 2007) Atabek, Mehmet Emre; Pirgon, OzgurBackground. Few previous studies have examined the validity of the fasting glucose-to-insulin ratio (FGIR), homeostasis model assessment of insulin resistance (HONIA-IR) and quantitative insulin-sensitivity check index (QUICKI) in pediatric populations. Objective: To compare simple indices of insulin resistance calculated from fasting glucose and insulin levels with insulin sensitivity indices (area under the response curve [AUC(insulin)], insulin sensitivity index [ISI-composite]) determined by oral glucose tolerance testing (OGTT) in obese children. Methods: One hundred and forty-eight obese children and adolescents (86 girls and 62 boys, mean age: 10.86 +/- 3.08 years, mean body mass index (BMI): 27.7 +/- 4.2) participated in the study. OGTT was performed in all participants. After glucose and insulin measurements from OGTT, the children were divided into two groups according to the presence or absence of insulin resistance.. Insulin sensitivity indices obtained from the OGTT were compared between the groups. The total plasma glucose response and insulin secretion were evaluated from the AUC estimated by the trapezoid rule. Cut-off points, and sensitivity and specificity calculations were based on insulin resistance with receiver operating characteristic curve (ROC) analysis. Results: The prevalence of insulin resistance, glucose intolerance and dyslipidemia was 37.1%, 24.3% and 54% in obese children, respectively. The groups consisted of 93 children without insulin resistance (54 girls and 39 boys; mean age: 10.5 +/- 3.3 years; mean BMI: 27.0 +/- 4.2) and 55 children with insulin resistance (32 girls and 23 boys; mean age: 11.4 +/- 2.5 years; mean BMI: 27.9 +/- 3.9). There were significant differences in mean FGIR (10.0 +/- 7.2 vs 5.6 +/- 2.8, p < 0.001), HONIA-IR (3.2 +/- 2.3 vs 4.9 +/- 2.3, p < 0.001) and QUICKI (0.33 +/- 0.03 vs 0.30 +/- 0.02, p < 0.001) between the groups. The cut-off points for diagnosis of insulin resistance were < 5.6 for FGIR (sensitivity 61.8, specificity 76.3), > 2.7 for HONIA-IR (sensitivity 80, specificity 59.1), and < 0.328 for QUICKI (sensitivity 80, specificity 60.2). Conclusions: Indices derived from fasting samples for diagnosis of insulin sensitivity are reliable criteria in obese children and adolescents. HONIA-IR and QUICKI appeared to have similar sensitivity and specificity and to have higher sensitivity than FGIR.Öğe Bilateral sacroiliitis following lsotretinoin treatment in an adolescent with Klinefelter syndrome(LIPPINCOTT WILLIAMS & WILKINS, 2007) Atabek, Mehmet Emre; Pirgon, Ozgur; Elmas, SefikaKlinefelter syndrome has been noted to have an association with several autoimmune and rheumatic diseases, including systemic lupus erythematosus, rheumatoid arthritis, and ankylosing spondylitis. We report a 14-year-old boy with Klinefelter syndrome who developed severe acne fulminans and bilateral sacroiliitis after testosterone and isotretinoin administration.Öğe Congenital Anodontia in Ectodermal Dysplasia(FREUND PUBLISHING HOUSE LTD, 2008) Pirgon, Ozgur; Atabek, Mehmet Emre; Tanu, Ilhan Asya[Abstract not Available]Öğe Diabetic Ketoacidosis, Thyroiditis and Alopecia Areata in a Child with Down Syndrome(SPRINGER INDIA, 2009) Pirgon, Ozgur; Atabek, Mehmet Emre; Sert, AhmetA 10-year-old girl with Down syndrome and alopecia areata was admitted with severe ketoacidosis. She had high blood glucose level: 615 mg/dL and her HbAlc level was 13.3 %. After the control of ketoacidosis and reduction of the plasma glucose level, we found low free T4 level and high TSH levels. Thyroid peroxidase antibodies titer was 1383 IU/mL and both gliadin and endomysial antibodies were negative. This was the first report of a child with Down syndrome showing type 1 diabetes combined with alopecia areata and autoimmune thyroid disease. [Indian J Pediatr 2009; 76 (12) : 1263-1264] E-mail: ozpirgon@hotmail.comÖğe Evidence for association between insulin resistance and premature carotid atherosclerosis in childhood obesity(INT PEDIATRIC RESEARCH FOUNDATION, INC, 2007) Atabek, Mehmet Emre; Pirgon, Ozgur; Kivrak, Ali SamiThe present study was undertaken to determine the presence and predictors of the subclinical atherosclerosis in obese children. Fifty obese children [mean age: 11.7 +/- 2.5 y, mean body mass index (BMI): 28.2 +/- 4.0 kg/m(2)] and 50 age- and sex-matched healthy nonobese controls (mean age: 11.4 +/- 3.73 y, mean BMI: 17.6 +/- 3.0 kg/m(2)) were enrolled in the present study. Oral glucose tolerance test was performed to all obese subjects. Common carotid artery intima-media thickness (IMT) was measured by high-resolution B-mode ultrasonography. Carotid artery IMT was significantly increased (0.0476 +/- 0.007 versus 0.033 +/- 0.011 cm; p < 0.001) in the obese group. There were significant relations between carotid artery IMT and insulin sensitivity indexes derived from fasting samples (fasting glucose to insulin ratio (FGIR; p = 0.004, r = -0.404), quantitative insulin-sensitivity check index (QUICK-1; p = 0.002, r = -0.401) and homeostasis model assessment of insulin resistance (HOMA-IR; p = 0.034, r = 0.300) in the obese group. In a multivariate regression model, QUICK-I emerged as independent correlates for mean IMT in obese children with the total variance explained being 20.7% (beta = -0.58, p < 0.001). We concluded that insulin resistance is an independent risk factor for increased carotid artery IMT in obese children.Öğe Extensive brown tumors caused by parathyroid adenoma in an adolescent patient(SPRINGER, 2008) Atabek, Mehmet Emre; Pirgon, Ozgur; Sert, Ahmet; Esen, H. HasanPrimary hyperparathyroidism is a rare endocrine disease in children and young adults. The early detection and treatment of primary hyperparathyroidism led to a marked decrease in classical bone and renal manifestations of the disease. Osteitis fibrosa cystica and brown tumors have become extremely rare clinical entities. Moreover, the skeletal involvement in primary hyperparathyroidism secondary to parathyroid adenoma is extremely rare. We report on an adolescent girl with multiple brown tumors and a history of recurrent fractures as the manifestation of primary hyperparathyroidism associated with a parathyroid adenoma. The patient's clinical presentation mimicked parathyroid carcinoma. She had a large tumor associated with marked elevation in the parathyroid hormone and serum calcium levels. Skeletal manifestations were also atypical for benign primary hyperparathyroidism, with widespread brown tumors in the patient.Öğe Familial Mediterranean fever associated with type 1 diabetes - Association or coincidence?(LIPPINCOTT WILLIAMS & WILKINS, 2006) Atabek, Mehmet Emre; Pirgon, Ozgur; Sert, Ahmet; Arslan, UgurFamilial Mediterranean fever, also known as a periodic disease or recurrent polyserositis, is an autosomal-recessive disorder characterized by recurrent attacks of fever, synovitis, peritonitis, or pleuritis. In patients presenting with typical clinical features and with an appropriate ethnic origin, the diagnosis can be made without genetic confirmation. The discovery of the MEFV gene has led to a molecular approach to diagnosis, aiming at improving the global diagnosis of the disease. Some diseases, mainly vasculitides, seem to be more common in familial Mediterranean fever. The "decreased antiinflammatory response" hypothesis and other putative mechanisms (cytokines) in familial Mediterranean fever can also take a predisposing and facilitating role in type I diabetes autoimmune pathogenicity. We describe a previously unreported association between familial Mediterranean fever and type I diabetes in a 9-year-old girl.Öğe Hypokalemic rhabdomyolysis in a child with 11-hydroxylase deficiency(FREUND PUBLISHING HOUSE LTD, 2008) Atabek, Mehmet Emre; Pirgon, Ozgur; Sert, AhmetMyopathy is a well-recognized complication of persistent hypokalemia. Although hypokalemic myopathy may be due to divers diseases or to drug administration, hypokalemic rhabdomyolysis as a complication of under-treated 11-hydroxylase deficiency has not previously been described in the literature. We describe a 10 year-old boy with under-treated 11-hydroxylase deficiency who developed rhabdomyolysis following severe hypokalemia. Patients with under-treated 11-hydroxylase deficiency may present with hypokalemia in association with muscle weakness; if serum potassium is markedly low, rhabdomyolysis may occur. Hypokalemia-induced rhabdomyolysis should be carefully followed.Öğe Identical twins synchronously presented with diabetic ketoacidosis in infancy period(WILEY, 2006) Atabek, Mehmet Emre; Pirgon, Ozgur; Sert, Ahmet[Abstract not Available]Öğe Infantile systemic hyalinosis with early thyroid dysfunction(WALTER DE GRUYTER GMBH, 2007) Pirgon, Ozgur; Atabek, Mehmet Emre; Esen, H. Hasan; Cangul, H.Infantile systemic hyalinosis is an autosomal recessive disorder characterized by diffuse hyaline deposits in the skin, gastrointestinal tract, muscles and glands. The molecular basis of infantile systemic hyalinosis is unknown. The main pathological feature is widespread hyalinosis of many tissues and organs. We present an 18 month-old girl with infantile systemic hyalinosis and hypothyroidism. Newly diagnosed children with infantile systemic hyalinosis should have thyroid studies as a routine part of diagnostic work-up.Öğe Insulin detemir improves glycemic control and reduces hypoglycemia in children with type 1 diabetes: findings from the Turkish cohort of the PREDICTIVE (TM) observational study(WILEY, 2009) Kurtoglu, Selim; Atabek, Mehmet Emre; Dizdarer, Ceyhun; Pirgon, Ozgur; Isguven, Pinar; Emek, SevilBackground: Insulin detemir is a basal insulin analog designed to produce a superior pharmacokinetic profile to basal formulations of human insulin. It has shown consistently improved tolerability ill comparison to neutral protamine Hagedorn (NPH) insulin in adult cohorts, but there are relatively few publications involving pediatric cohorts. Methods: The efficacy and safety of insulin detemir in children with type I diabetes was assessed using data from the Turkish cohort of PREDICTIVE(TM) (a large, multinational, observational) study. The children investigated were using basal-bolus therapy involving NPH insulin or insulin glargine at baseline but were switched to insulin detemir as part of routine clinical care by their physicians. Results: Twelve weeks of treatment with insulin detemir significantly reduced mean hemoglobin A1c (9.7-8.9%, p < 0.001) and mean fasting glucose [185-162 mg/dL (10.3-9 mmol/L), p < 0.01]. Fasting glucose variability was also lower after treatment with insulin detemir than previously (oil either NPH or glargine, p < 0.05). The frequencies of total, major and nocturnal hypoglycemic events were significantly reduced with insulin detemir relative to baseline, with all estimated mean of 6.89 fewer events/patient/yr overall (p < 0.001) and 2.6 fewer nocturnal events/patient/yr (p < 0.01). Weight and insulin dose remained relatively unchanged. Conclusions: Twelve weeks of treatment with insulin detemir improved glycemic control and reduced hypoglycemia ill children with type I diabetes. This improved tolerability might allow further dose titration and therefore additional improvements in glucose control.Öğe An interesting coexistence of 46, XX maleness and 46, XX 21-hydroxylase deficiency in dizygotic twins(KARGER, 2006) Atabek, Mehmet Emre; Acar, Hasan; Pirgon, Ozgur; Piskin, Mehmet Mesut[Abstract not Available]Öğe Laparoscopic hysterectomy and bilateral salpingo-oophorectomy incongenital adrenal hyperplasia(MARY ANN LIEBERT INC, 2006) Klinic, Mehmet; Atabek, Emre; Guven, Selcuk; Pirgon, Ozgur[Abstract not Available]Öğe Mayer-Rokitansky-Kuster-Hauser syndrome presenting as premature thelarche in a young child(BLACKWELL PUBLISHING, 2007) Atabek, Mehmet Emre; Pirgon, Ozgur; Sert, Ahmet[Abstract not Available]Öğe Metabolic syndrome manifestations in an adolescent with acrodysostosis(FREUND PUBLISHING HOUSE LTD, 2007) Atabek, Mehmet Emre; Pirgon, Ozgur; Sert, AhmetAcrodysostosis is an extremely rare disorder characterized by short stature and peripheral dysostosis. The co-existence of metabolic syndrome and acrodysostosis in adolescence has not been previously reported in the pediatric endocrinology literature. We report a 17 year-old boy with acrodysostosis who developed metabolic syndrome, with insulin resistance and impaired glucose tolerance exhibited by an oral glucose tolerance test as well as other features of metabolic syndrome including hyperlipidemia and hypertension.Öğe Metabolic syndrome manifestations in Cohen syndrome: Description of two new patients(SAGE PUBLICATIONS INC, 2006) Pirgon, Ozgur; Atabek, Mehmet Emre; Sert, AhmetWe report on sporadic cases that have the proposed diagnostic criteria for Cohen syndrome associated with metabolic syndrome. The patients, aged 14 and 16 years, had truncal obesity, mild mental retardation, hypotonia, narrow hands and feet, a high-arched palate, prominent upper central incisors, a high nasal bridge, and ocular abnormalities. Both had impaired glucose tolerance, with marked hyperinsulinemia exhibited by an oral glucose tolerance test. Moreover, they had the other metabolic syndrome features of hyperlipidemia and hypertension. We suggest that it is necessary to consider the possibility of metabolic syndrome in a case of Cohen syndrome.Öğe Pituitary-adrenal axis suppression due to topical steroid administration in an infant(WILEY, 2007) Atabek, Mehmet Emre; Pirgon, Ozgur; Unal, Ekrem[Abstract not Available]Öğe Possible effect of leptin on renal magnesium excretion in adolescent patients with type 1 diabetes(WILEY, 2006) Atabek, Mehmet Emre; Kurtoglu, Selim; Pirgon, OzgurBackground : Hypomagnesaemia and hyperleptinemia are common in patients with diabetes. Moreover, it has been demonstrated that leptin stimulates diuresis and natriuresis. The aim of this study was to evaluate the relationship between serum leptin, serum magnesium (Mg) and urinary Mg/urinary creatinine levels in patients with type 1 diabetes. Methods : Serum leptin and Mg and urinary Mg/urinary creatinine levels were measured in 67 patients with diabetes (33 girls and 34 boys). The age, diabetes duration, anthropometric and metabolic parameters of the subjects were matched between girls and boys. The relation of serum leptin levels to serum and urinary Mg/urinary creatinine levels was assessed. Results : Serum leptin levels of girls with diabetes were higher than those of the boys (14 +/- 5.3 mu g/L vs 5.8 +/- 1.5 mu g/L, P < 0.001, respectively). The differences for serum Mg and for urinary Mg/urinary creatinine levels were not significant between girls and boys with diabetes. Leptin levels were correlated with urinary Mg/urinary creatinine levels in both girls and boys (r = 0.39, P = 0.02 and r = 0.37, P = 0.03, respectively). In a multivariate regression model, leptin emerged as independent correlates for mean urinary Mg/urinary creatinine in both girls and boys with the total variance explained being 14%, and 15%, respectively. Conclusion : The data suggest that serum leptin might be related to increased urinary Mg loss in patients with type I diabetes.
