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This study aimed to investigate whether supplemental soluble fiber, oat β-glucan, has any effect on glycemic control and variability of adolescents with type 1 diabetes mellitus.This study was conducted in 30 adolescents with type 1 diabetes mellitus and it consisted of three phases lasting one week. At Phase I, all subjects followed a standard diet program. For Phase II and Phase III, the subjects continued their standard diet program and added natural oat flakes containing 3 g/day and 6 g/day β-glucan, respectively. Glucose levels were monitored by continuous glucose monitoring (CGM). The maximal, minimal, mean and daytime and night blood glucose levels, percentages of glucose values in a target range and below and above a target values were calculated for each of the phases. Premeal, postmeal, peak blood glucose values and peak times of meals were evaluated for each of the phases. Glycemic variability was measured via SD, CV, MAGE, IQR, MODD, LBGI, HBGI, and CONGA parameters.The maximal, mean and daytime and night blood glucose levels were the lowest at Phase III (p 0.05). Minimal blood glucose levels were the highest at Phase III (p 0.05). Phase I, II, and III showed similar durations elapsed for Level 2 and Level 1 hypoglycemia, euglycemia, Level 1 and Level 2 hyperglycemia (p 0.05). Premeal and postmeal blood glucose levels were lowest at Phase III for breakfast, lunch, and overall (p 0.05). The lowest peak blood glucose levels were detected at Phase III for breakfast, lunch, dinner and overall (p 0.05). Phase III also showed delayed peaks for all time-points (p 0.05 for each) compared to other phases. Phase III had significantly lower levels of SD, CV, LBGI, and CONGA levels than those in either Phase I or Phase II (p 0.05 for each).6 g/day oat β-glucan have favorable outcomes in glycemic control and variability in adolescents with type 1 diabetes mellitus.

Kistik fibrozis ile ilişkili diyabet (KFİD), kistik fibrozisin (KF) yaygın bir komplikasyonudur. Kistik fibrozis ile ilişkili diyabetin temel nedeni hastalarda yıllar içerisinde pankreas hasarının artması ile beraber pankreas adacık hücrelerinin kaybı ve insülin eksikliğinin gelişmesidir. Tedavideki amaç çocuk ve adolesanlarda normal büyüme ve gelişmenin ve kan glukoz regülasyonunun sağlanmasıdır. Tedavinin temelini insülin tedavisi ve tıbbi beslenme tedavisi oluşturur. İnsülin tedavisi, KFİD hastalarında akciğer fonksiyonunu stabilize eder ve beslenme durumunu iyileştirir. Kistik fibrozis ile ilişkili diyabetli hastalara yüksek enerji, yüksek yağ ve yüksek tuz içeren bir beslenme planı önerilmektedir. Karbonhidrat sayımı, insülin tedavisi alan hastalarda öğün öncesi insülin dozunun belirlenmesinde yararlıdır. Bu yazıda kliniğimizde izlenen KFİD’li bir çocuk olgunun medikal ve tıbbi beslenme tedavisi literatür derlemesi eşliğinde sunulmuştur.

Despite the significant medical and technological advances, the management of Type 1 diabetes remains unsatisfactory. The most significant challenge herein is the difficulty in controlling post-prandial glycemia. The type and amount of carbohydrates consumed have a greater influence on the post-prandial hyperglycemia and glycemic variability than other dietary factors, which consequently generates interest in carbohydrate-modified diets for the management of Type 1 diabetes. Individuals with Type 1 and Type 2 diabetes prefer low-carbohydrate diet regimens in order to maintain glycemic control. Few studies have examined the effects of a low-carbohydrate diets on the course of glycemic control in individuals with Type 1 diabetes. Low-carbohydrate diets may reduce glycemic fluctuation, hemoglobin A1c levels, and insulin requirement in adults with Type 1 diabetes. The long-term effects of a low-carbohydrate diets are not well documented, although it is believed to alter the lipid profile and cause nutrient deficiencies and cardiac complications in the long term. In children and adolescents with Type 1 diabetes, it may improve glucose levels in the short term, although it may lead to growth retardation, deficiency in the intake of vitamin, minerals, and fiber, increase in blood lipids, fatigue, anxiety, and social isolation. This review discusses the effects of low-carbohydrate diets on Type 1 diabetes.

Objective: Soluble endoglin (S-endoglin) has been implicated as a potential marker of endothelial dysfunction (ED) and was reported to be elevated in diabetic adults, correlating with the severity of diabetic vasculopathy. However, circulating S-endoglin and its association with other markers of ED have not been formerly analyzed in the first decade of diabetes onset in adolescents with type 1 diabetes mellitus (T1DM). Methods: Fifty-eight adolescents with moderately/poorly controlled T1DM were included in this study and twenty-nine healthy adolescents served as controls. The diabetic group was divided into two groups based on the presence of microalbuminuria, as the microalbuminuria group (n=15) and the normoalbuminuria group (n=43). Functional vascular alterations were evaluated by measuring serum S-endoglin and plasma nitric oxide (NO) concentrations, the flow-mediated dilatation (FMD) of the brachial artery. Carotid intima media thickness (CIMT) was measured for evaluation of structural vascular alterations. Results: The S-endoglin and NO levels of both microalbuminuria and normoalbuminuria groups were higher than those of the control group (for S-endoglin, p=0.047 and p

Individuals with 45,X/46,XY karyotype are at increased risk for germ cell tumor development. We report a case with a diagnosis of 45, X/46, XY gonadal dysgenesis who presented with short stature, physical stigmata of Turner syndrome. Her pubertal development was at Tanner stage 3. At follow-up, bilateral prophylactic gonadectomy was performed when considering the risk factors. Pathological assessment was consistent with gonadoblastoma in the left gonad, and dysgerminoma and gonadoblastoma in neighboring areas in the right gonad. The karyotype analysis of the right and left gonadal tissues reveled 45,X[97,3]/46,XY[2,7] and 45,X[92,7]/46,XY[4,5]/47,XYY [2,8] mosaic, respectively. The clinical management of such patient should be individualized according to the present risk factors. Additionally, signs of estrogenization like advanced breast development always suggest the possible presence of germ cell tumor.

BACKGROUND Vitamin D, an important factor in calcium-phosphate homeostasis, has recently been suggested to play an important role in the pathogenesis of numerous chronic conditions such as hyperandrogenism. The aim of this study was to investigate the relationship between vitamin D status and premature adrenarche (PA). METHODS A total of 71 girls with PA and 52 healthy girls, as the control group, were recruited. Axillary and/or pubic hair development before the age of 8 years was defined as PA. Bone age and anthropometric measures including height, weight, and body mass index (BMI) were obtained. 25-Hydroxyvitamin D (25(OH)D), fasting plasma glucose and insulin were measured. Vitamin D insufficiency was defined as

Objective To evaluate neural and vascular retinal morphology of children with isolated growth hormone deficiency (GHD) and to determine any retinal changes due to GH treatment. Methods Twenty-eight children with isolated GHD and 53 age-, gender- and body mass index-matched healthy volunteers were enrolled in this prospective study. The retinal nerve fibre layer (RNFL) and macular thickness (MT) were measured, as well as intraocular pressure (IOP). The number of retinal vascular branching points were calculated. Effect of GH treatment on the retina and IOP was evaluated after one year of treatment. Measurements were also made in the control group at baseline and following the initial examination. Pre- and post-treatment changes were compared. The findings were also compared with those of the controls. The correlation between ocular dimensions and insulin-like growth factor-I (IGF-1) levels were also analysed. Results The number of branching points was significantly lower in GHD patients as compared with control subjects (15.11±2.67 and 19.70±3.37, respectively, p=0.05 for all comparisons). No statistically significant differences were found in mean RNFL, MT and IOP values between GHD patients and control subjects. GH treatment did not create any significant changes in the retinal vascularization or other retinal neural parameters and IOP either within the patient group or when compared with the control group. No correlations were observed between ocular dimensions and IGF-1 levels. Conclusion Our findings suggest that isolated GHD may lead to decreased retinal vascularization. However, retinal neural growth and differentiation were not affected by GHD. These findings may be related to the fetal development process of pituitary somatotropic cells and the retina. Additionally, GH treatment did not cause any changes in retinal neural and vascular tissues.

Background/aim: The aim of this study was to investigate the prevalence of the microdeletions and mutations of the SHOX gene in children with idiopathic short stature (ISS) by the usage of fluorescence in situ hybridization (FISH) and direct sequencing technique. Materials and methods: Thirty-seven children referred to our clinic because of short stature were classified as having ISS after clinical examination. Chromosome analyses, FISH analysis of the SHOX gene, and direct sequencing of the coding exons of SHOX , through the second to the sixth exon, in 24 of the 37 patients were also performed. Results: All children had normal karyotypes and the SHOX gene region was found to be intact in all. No mutation was detected in the exonic sequences and exon/intron boundaries of the SHOX gene in 24 children analyzed. Conclusion: No mutation was detected in the exonic sequences and exon/intron boundaries of the SHOX gene and this indicated that the prevalence of the SHOX mutations can differ according to the selection criteria, used methods, sample size, and population.

Background/aim: Various studies have shown that adult patients with polycystic ovary syndrome (PCOS) have higher levels of anxiety and depression compared to their normal counterparts. However, it is still unclear whether these mood disorders already exist in adolescents affected by PCOS. The aim of the present study is to assess differences in anxiety and depression levels between adolescents with PCOS and age- and body mass index (BMI)-matched controls and to determine the possible factor(s) impacting these psychological parameters in adolescents with PCOS. Materials and methods: The study included 80 adolescents with PCOS and 50 age- and BMI-matched controls. All participants completed standardized questionnaires assessing anxiety and depression. A multiple linear regression model was used to analyze the impact of potential variables on anxiety and depression scores of the adolescents with PCOS. Results: Significantly higher levels of anxiety, specifically generalized and social anxieties, as well as depression were found in adolescents with PCOS compared to controls. Higher BMI was found to be associated with higher levels of depression and generalized anxiety, and higher modified Ferriman-Gallwey score with higher level of panic disorder in adolescents affected by PCOS. Conclusion: Adolescents with PCOS experience significantly more emotional distress compared to adolescents without PCOS. This emotional distress may be related, at least in part, to certain clinical features of PCOS including obesity and hirsutism. PCOS in adolescents should be assessed not only for the gynecological and metabolic aspects but also for the emotional aspects of the disease.

Aim To report, for the first time, the incidence and prevalence of childhood Type 1 diabetes in Turkey using a nationwide registry. Methods Information on birth date, city of birth, diagnosis date and gender of all patients with Type 1 diabetes aged < 18 years were obtained from the Turkish Social Security Institute for the period from January 2011 to December 2013. Results There were 17 175 prevalent cases of Type 1 diabetes over the 3-year period. The prevalence of Type 1 diabetes was 0.75/1 000 (95% CI 0.74–0.76) and was higher in girls than in boys (0.79 vs 0.72 /1 000; P < 0.01). There were 2465 incident cases in 2013. The incidence was slightly higher among girls (50.6%) than boys (49.4%); the girl:boy case ratio was 1.02. The incidence was 10.4/100 000 for boys and 11.3/100 000 for girls. The age-standardized incidence rate was 10.8 per 100 000 (95% CI 10.1–11.5) according to the WHO standard population, estimated using the direct method. The mean patient age at diagnosis was 10.6 ± 4.6 years. The highest proportion of cases (40.6%) was diagnosed in children aged 10–14 years. Conclusions This is the first study to report the incidence and prevalence of Type 1 diabetes in children in Turkey. The incidence of Type 1 diabetes reflects the geographical location of Turkey, bridging Asia and Europe, with the incidence being higher than in Asia but lower than in Europe.

Cardio-facio-cutaneous (CFC) syndrome is a rare disorder characterized by craniofacial dysmorphia, ectodermal abnormalities, cardiac malformations, as well as growth and developmental delay. Although some endocrine abnormalities have been reported in this syndrome, very little is known about CFC syndrome-related endocrine disorders. A 7.5-year-old boy was admitted to our endocrinology clinic with the complaint of short stature. He had a height of 103 cm [-4 standard deviation (SD)], a weight of 16 kg (3(th) percentile, -1.7 SD), a facial appearance typical for the CFC syndrome, optic nerve hypoplasia and pulmonary stenosis. Genetic investigation revealed a heterozygous mutation in exon 3 of the MEK1 gene, c.389AG (p. Y130C). During his long-term follow-up, the patient developed a variety of endocrine disorders including precocious puberty, growth hormone deficiency and hyperprolactinemia.

Objective: Although polymorphisms in suppressor of cytokine signaling 3 (SOCS3) was reported to be related to obesity, Metabolic syndrome (MS), and type 2 diabetes mellitus in various adult studies, there is a lack of data in children. In this study, we examined eight reported polymorphisms of SOCS3 in obese Turkish children and adolescent with and without MS and compared the results with that of controls. Methods: One hundred and forty eight obese and 63 age- and sex-matched control subjects were enrolled in the study. Obesity classification was carried out according to body mass index. World Health Organization and National Cholesterol Education Program criteria were used for the diagnosis of MS. Genotyping procedure was carried out by polymerase chain reaction and Sanger sequencing protocol. Results: The frequency of rs2280148 polymorphism was significantly higher in obese subjects with MS than in the control group, whereas the frequency of rs8064821 polymorphism was significantly higher in obese subjects with MS than in obese children without MS. Conclusion: The significant associations of certain SOCS3 polymorphisms with obesity parameters in both MS and MS -related insulin resistance, hypertension, and fatty liver suggest that polymorphisms in this gene may play a role in the pathogenesis of MS and also that they can be potentially used as a marker for attenuated or aggressive disease.

Background:The aim of this study was to investigate the early signs of atherosclerosis and to evaluate serum endoglin and obestatin levels as predictors of subclinical atherosclerosis in obese children.Methods:A total of 95 children (60 obese and 35 controls) aged 10–18 years were included in the study. Their endoglin and obestatin levels and biochemical parameters were measured. The carotid intima media thickness (cIMT) and brachial artery flow-mediated dilatation (FMD) responses were evaluated.Results:The cIMT values were higher (p < 0.001) and FMD responses were lower (p = 0.003) in the obese group than in the control group. A logistic regression multivariate analysis revealed that cIMT was independently associated with the body mass index (BMI) Z-score (β = 0.323, p = 0.003) and low density lipoprotein (LDL) (β = 0.29, p = 0.008), while FMD % was independently associated with waist circumference (β = −0.36, p = 0.002). The obese and control groups were similar in endoglin (p = 0.67) and obestatin levels (p = 0.70). The endoglin level was inversely correlated with the cholesterol and LDL levels (r = −0.23, p = 0.032; rho = −0.25, p = 0.019).Conclusions:The cIMT and brachial artery FMD response in obese children are significantly different compared to healthy controls. Circulating endoglin and obestatin levels are not predictive markers for subclinical atherosclerosis in obese children aged 10–18 years old.

ABALI, SAYGIN/0000-0001-6552-2801; Gurbuz, Fatih/0000-0003-2160-9838; Hatun, Sukru/0000-0003-1633-9570; Abaci, Ayhan/0000-0002-1812-0321; Turan, Serap/0000-0002-5172-5402; Buyukinan, Muammer/0000-0002-2937-823X; yuksel, bilgin/0000-0003-4378-3255; Ozgen, Ilker Tolga/0000-0001-6592-9652; binay, cigdem/0000-0002-7749-8818; ozkan, Behzat/0000-0002-9153-8409; Demir, Korcan/0000-0002-8334-2422; Eren, Erdal/0000-0002-1684-1053 WOS: 000373099300016 PubMed: 26788866 To evaluate the anthropometric features of girls with Turner syndrome (TS) at birth and presentation and the effect of karyotype on these parameters. Data were collected from 842 patients with TS from 35 different centers, who were followed-up between 1984 and 2014 and whose diagnosis age ranged from birth to 18 years. Of the 842 patients, 122 girls who received growth hormone, estrogen or oxandrolone were excluded, and 720 girls were included in the study. In this cohort, the frequency of small for gestational age (SGA) birth was 33%. The frequency of SGA birth was 4.2% (2/48) in preterm and 36% (174/483) in term neonates (P

Aim. This study aimed to evaluate the effects of monotherapy with valproate or oxcarbazepine on the linear growth of children with idiopathic epilepsy. Methods. Antiepileptic treatment with valproate or oxcarbazepine was initiated in 76 patients. These were evaluated at baseline and at 6 and 18 months after commencement of therapy to determine height standard deviations (height z-scores). Serum ghrelin, insulin-like growth factor-1, and insulin-like growth factor-binding protein-3 levels were measured. Results. In prepubertal patients receiving oxcarbazepine, height z-scores were elevated after 6 and 18 months of therapy (p = 0.008 and p = 0.001, respectively); in pubertal patients, a significant increase was noted at the 18th month of therapy (p = 0.004). In prepubertal patients receiving oxcarbazepine, serum standardized insulin-like growth factor-1 and insulin-like growth factor-binding protein-3 levels were significantly higher at the 18th month of therapy compared with baseline (p = 0.005 and p = 0.004, respectively). In pubertal patients receiving valproate, serum ghrelin levels were significantly decreased at the 18th month of therapy compared with baseline (p = 0.006). Conclusion. Exposure to oxcarbazepine stimulated linear growth in epileptic patients through mechanisms involving the release of insulin-like growth factor-1 and insulin-like growth factor-binding protein-3. In contrast, exposure to valproate did not affect linear growth, but did lead to a decrease in serum ghrelin

Background/Aim: The exon 3-deleted/full-length (d3/fl) growth hormone (GH) receptor (GHR) polymorphism has been associated with responsiveness to GH therapy in some diagnostic groups. However, there are still controversies on this issue. To evaluate the effect of the GHR exon 3 polymorphism on growth after 1 and 2 years of GH therapy in Turkish patients with GH deficiency (GHD) and Turner’s syndrome (TS) and the distribution of GHR exon 3 isoforms. Materials and Methods: 218 patients with GHD (125 males/93 females) and 43 patients with TS were included in the study. The control group included 477 healthy adults aged from 18 to 57 years (54 females/423 males). Anthropometric parameters and insulin-like growth factor (IGF)-1 and IGF binding protein (IGFBP)-3 were evaluated annually. GHR isoforms were studied using simple multiplex PCR. Height and body mass index were expressed as standard deviation score (SDS). Results: There were no differences among TS, GHD and healthy adults regarding the distribution of GHR exon 3 isoforms (fl/fl, fl/d3 and d3/d3). There was a significant increase in height SDS in both diagnostic groups on GH therapy; however, there were neither differences in height SDS and Δheight velocity between fl/fl, fl/d3 and d3/d3 groups nor a correlation between the distribution of GHR exon 3 isoforms and change in IGF-1 SDS and IGFBP-3 SDS levels on GH therapy in either of the diagnostic groups. There was also no gender difference in GHR isoforms in healthy adults. Conclusion: The results suggest that responsiveness to GH therapy does not depend on the exon 3 GHR genotypes in GHD and TS patients.

Aim: The age-related values of penile length must be known to determine abnormal penis sizes and to follow the treatment of underlying diseases. The aim of this study is to evaluate abnormal penile length in Turkish children by establishing novel reference values for Turkish population and to compare the mean penile length and other parameters with alternates from different ethnic populations and geography. Methods: This cross-sectional study was conducted on a voluntary basis between November 2008 and November 2009 from four centres in Turkey and comprised of 1278 healthy volunteered prepubertal children. Complete stretched penile length and penis circumference measurements were used for penile length and penis circumference evaluations, respectively. All measurements were taken twice by only one investigator, and mean values were recorded. Results: Penile length and penis circumference for every age group were obtained, percentile curves were established and these findings were compared with the results of previous studies. Significant differences were found between penile length of Turkish children and recently used reference values. Conclusion: With this study, novel reference values for penile length in prepubertal children were presented to the literature.

Arachnoid cysts (ACs), particularly suprasellar cysts, cause a wide spectrum of endocrine disorders. Herein, we report two patients diagnosed with an extensive AC in the middle cranial fossa while being investigated for etiologies of precocious puberty and short stature. One of them required surgery due to his pubertal disorders associated with compression effects of cyst. After surgery, his puberty progression was regressed within one year. On the other hand, surgery was not planned for the second patient considering of his cranial imaging findings and the extremely low incidence of growth hormone (GH) deficiency caused by middle fossa AC (MFAC). We started treatment with recombinant human GH and no complication was found during treatment follow-up. Endocrine disorders associated with MFACs are extremely rare. By presenting with these two cases, we aimed to remain our fellow physcians that ACs can be possibly cause of endocrine disorders. Clinicians should be careful evaluating endocrine disorders because real cause may not be cyst itself but masked by it.

Background A number of clinical studies conducted in adults have demonstrated the prognostic significance of angiogenic factors in malignancies, however, only a limited number of studies have been conducted in children. The aim of this study was to determine serum vascular endothelial growth factor (VEGF), endostatin, and leptin levels in children with lymphoma and to investigate whether these factors provide prognostic information. Procedure Serum samples from 36 children with lymphoma (non-Hodgkin lymphoma (NHL) N = 21, Hodgkin lymphoma (HL) N = 15) were collected at diagnosis and during remission. Serum samples were also collected from 18 healthy children as the control group. Serum VEGF and endostatin levels were quantified by using enzyme-linked immunosorbent assay (ELISA) and serum leptin by immunoradiometric assay. Results The serum VEGF levels were found elevated in patients compared to controls (P = 0.033), while endostatin and leptin levels were lower in patients than in controls (endostatin, 43.9 ± 5.8 ng/ml vs. 123.6 ± 13.5 ng/ml, P

INTRODUCTION High levels of anticardiolipin (aCL) antibodies may predict vascular complications that could develop in type 1 diabetes and autoimmune thyroiditis (AIT). However, the clinical relevance of these antibodies in subjects with type 1 diabetes and AIT is unclear. OBJECTIVES The aim of the study was to determine the prevalence and significance of aCL antibodies in patients with type 1 diabetes and AIT. PATIENTS AND METHODS The study involved 74 patients with type 1 diabetes (mean age 12.9 +/-4.2 years), 64 patients with AIT (mean age 14.1 +/-3.7 years), and 35 healthy control subjects (mean age 12.8 +/-3.3 years). The levels of aCL immunoglobulin (Ig) G and aCL IgM antibodies were measured by enzyme-linked immunosorbent assays. Low-positive and medium/high-positive cut-off values were selected for aCL antibody positivity. RESULTS The prevalence of aCL antibodies was higher in AIT patients compared with diabetic and healthy subjects with low positive levels (P

Background/Aims: Plant growth regulators are considered to leave minimal amounts of remnants and therefore cause no significant side effects in humans. In this study, we aimed to investigate the hormonal and histopathological effects of 4-chlorophenoxy acetic acid (4-CPA), a commonly used plant growth regulator, on the gonadal functions of rats. Methods: The study was implemented on 64 Wistar albino rats (20 days old). Forty-eight rats received 4-CPA every day until 50 days of age. The rats were randomized into 4 groups (a control group and three 4-CPA groups with doses of 25, 50 and 100 mg/kg/day); each group was further divided into males and females, making a total of 8 groups. The levels of FSH, LH, testosterone, estradiol, leptin, inhibin-B and neuropeptide-Y were measured. Histopathological examination of the testes and ductus deferens in male rats, and ovaries and uterus of female rats (caspase-3 and -9 immunoreactivity) was performed. Results:Although hormone levels were similar between the groups, rats that received 4-CPA showed significantly higher degrees of apoptosis compared to the control group (p < 0.001) and increased doses of 4-CPA were directly correlated with the amount of apoptosis (p < 0.001). Conclusion: 4-CPA induced apoptosis in the gonads of rats without concurrent changes in plasma hormone levels.

Two common forms of autoimmune thyroid diseases are Graves' disease and Hashimoto's thyroiditis. Cytotoxic T lymphocyte antigen 4 (CTLA4) encoded by the CTLA4 gene on chromosome 2q33 plays a role in susceptibility to Graves' disease and is probably important also for Hashimoto's thyroiditis as well as for the other endocrine autoimmune disorders. The CTLA4 locus is the only nonhuman leukocyte antigen locus that has been found in association with Graves' disease repeatedly. Particularly, association of three polymorphic markers of CTLA4 gene, namely, C(-318) T, A49G, and (AT)n dinucleotide repeat, with Graves' disease was demonstrated in most of the population-based investigations. On the other hand, there are few studies to reveal the association of these markers with Hashimoto's thyroiditis. A49G polymorphism was proposed to be associated with Hashimoto's thyroiditis, and C(-318) T was suggested to be not associated. The patient groups consisted of 88 patients (10 males and 78 females; mean age: 14.5 +/- 3.2 years [4.6-21.0 years]) with a previous diagnosis of Hashimoto's thyroiditis and 112 euthyroid volunteers (51 males and 61 females; mean age: 14.1 +/- 2.9 years [5.2-18 years]). The frequency of A/G (A49G) genotype was high and statistically significant in patients with Hashimoto's thyroiditis in comparison with the control group. Although the frequency of C/T [C(-318) T] genotype is not significantly high in children with Hashimoto's thyroiditis according to the control group, the risk of Hashimoto's thyroiditis in A/G genotype group was 4.66 times greater than the group with A/A genotype. In this study, we documented that the A49G polymorphism might increase the susceptibility for Hashimoto's thyroiditis.

The aim of the present study was to investigate the effects of carbamazepine (CBZ), oxcarbazepine (OXC), and valproic acid (VPA) on thyroid function and volume in epileptic children.Fifty-three epileptic children (age, 3-17 years) treated with OXC (n = 10), CBZ (n = 12), or VPA (n = 31) at least for 1 year were evaluated in terms of thyroid hormones, thyroid-stimulating hormone (TSH) levels, response to thyrotropin-releasing hormone (TRH) stimulation test, and thyroid volumes.The patients in the OXC and CBZ groups had similar total thyroxin (TT4) and free T4 (fT4) median levels that were significantly lower than those of the VPA group (P0.016). Total tri-iodothyrosin median levels were lower in the CBZ group compared to the VPA group (P0.016). Basal TSH levels and thyroid volumes were similar in all groups (P0.016). One child from the OXC group (10%), one from the CBZ group (%8.3), and six from the VPA group (19.3%) had hypothyroidic status according to the TRH stimulation test. No statistically significant correlations were found between thyroid gland volume and thyroid function variables and between anti-epileptic drug receiving time and thyroid function or thyroid volume, respectively, in any of the groups (P0.05).Thyroid function should be evaluated periodically in children using CBZ, OXC or VPA. The children taking VPA seems to be at greater risk compared to children onr CBZ or OXC therapy. Except for the basal TSH values in the VPA group, the parameters predictive for the subclinical hypothyroid status remain to be evaluated in further studies.

WOS: 000247339300019, PubMed: 17465999, Objective The aim of this study was to investigate serum leptin, oxidized low density lipoprotein (ox-LDL) and asymmetric dimethylarginine (ADMA) levels and their interaction with dyslipidaemia in adolescents with polycystic ovary syndrome (PCOS). Patients and design The study group consisted of 23 obese (obPCOS) and 21 nonobese girls with PCOS (nPCOS), and 31 lean healthy controls. PCOS was defined by the National Institutes of Health (NIH) criteria as the presence of chronic oligoanovulation and hyperandrogenism. Fasting leptin, ox-LDL, ADMA and detailed lipid-lipoprotein profile were determined. Atherogenic index (AI) was calculated as [Total cholesterol - HDL cholesterol/HDL cholesterol]. Logarithmic transformations were made for ox-LDL. Results Total cholesterol, triglycerides, LDL cholesterol, very low density lipoprotein (VLDL) cholesterol, apolipoprotein B, lipoprotein A levels and AI were higher and apolipoprotein AI was lower in obPCOS compared to those in controls (P < 0.05). LDL cholesterol, apolipoprotein B and lipoprotein A levels were higher in nPCOS compared to controls (P < 0.05). ADMA and ox-LDL levels did not differ in the three groups. Leptin was significantly higher in obPCOS compared with that in the other two groups (P < 0.001) and it was correlated with triglycerides (r = 0.62), VLDL cholesterol (r = 0.45), lipoprotein A (r = 0.38) and AI (r = 0.43) in the PCOS group (P < 0.05). Conclusion Our data demonstrate that ADMA and ox-LDL levels in adolescent PCOS subjects were not different than those in controls. Abnormal lipid profile was shown in obese and nonobese girls with PCOS and leptin was related with these lipid abnormalities in the PCOS subjects.

WOS: 000360842500004, PubMed: 26831551, 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., Turkish Pediatric Endocrinology and Diabetes Society [012013], This work was supported by the Turkish Pediatric Endocrinology and Diabetes Society (Grand number: 012013).

WOS: 000351307200005, PubMed ID: 25800473, Objective: Turner syndrome (TS) is a chromosomal disorder caused by complete or partial X chromosome monosomy that manifests various clinical features depending on the karyotype and on the genetic background of affected girls. This study aimed to systematically investigate the key clinical features of TS in relationship to karyotype in a large pediatric Turkish patient population. Methods: Our retrospective study included 842 karyotype-proven TS patients aged 0-18 years who were evaluated in 35 different centers in Turkey in the years 2013-2014. Results: The most common karyotype was 45, X (50.7%), followed by 45, X/46, XX (10.8%), 46, X, i(Xq) (10.1%) and 45, X/46, X, i(Xq) (9.5%). Mean age at diagnosis was 10.2 +/- 4.4 years. The most common presenting complaints were short stature and delayed puberty. Among patients diagnosed before age one year, the ratio of karyotype 45, X was significantly higher than that of other karyotype groups. Cardiac defects (bicuspid aortic valve, coarctation of the aorta and aortic stenosis) were the most common congenital anomalies, occurring in 25% of the TS cases. This was followed by urinary system anomalies (horseshoe kidney, double collector duct system and renal rotation) detected in 16.3%. Hashimoto's thyroiditis was found in 11.1% of patients, gastrointestinal abnormalities in 8.9%, ear nose and throat problems in 22.6%, dermatologic problems in 21.8% and osteoporosis in 15.3%. Learning difficulties and/or psychosocial problems were encountered in 39.1%. Insulin resistance and impaired fasting glucose were detected in 3.4% and 2.2%, respectively. Dyslipidemia prevalence was 11.4%. Conclusion: This comprehensive study systematically evaluated the largest group of karyotype-proven TS girls to date. The karyotype distribution, congenital anomaly and comorbidity profile closely parallel that from other countries and support the need for close medical surveillance of these complex patients throughout their lifespan.

Summary Hyperthyrotropinaemia, in which normal levels of T4 occur in association with raised thyroid stimulating hormone (TSH), is usually picked up on neonatal screening. High TSH level can continue for a long time in some of the cases. There is no consensus concerning the follow-up or treatment plan for hyperthyrotropinaemia. In this study, results of a 4-year follow-up of 36 cases who had been medically treated are discussed. Low-dose (5 μg/kg/day) L-thyroxin treatment was carried out in 36 cases that had 5 mU/l or higher TSH and showed exaggerated response to TRH test. Dose was decreased to 2–3 μg/kg/day in 24 of these patients during 6 months follow-up. The drug was stopped in three cases because of the development of biochemical hyperthyroidism. Denver developmental assessment test was applied to all cases at the end of the third year. All patients showed a normal development in relation to their age. According to our results, cases with hyperthyrotropinaemia need to be followed regularly for a long time and a need for low-dose L-thyroxin treatment may exist at ages varying from patient to patient.

This study was planned to investigate goiter prevalence and serum selenium and urine iodine status among school-age children in the Ankara region of Turkey. Nine hundred five (905) children were investigated; 847 of them were included in the study. Thyroid ultrasound was performed on children who were suspected of being goitrous at physical examination. Serum TSH, thyroxine, triiodotyronine, thyroid antibody, and urine iodine concentrations (UIC) are also measured. Ultrasound measurements revealed a goiter in 107 (12.6%) of the 847 children. Goiter prevalence was significantly lower among iodized-salt users compared to the noniodized salt using group. UIC and serum selenium levels in the goitrous group were significantly lower compared to the nongoitrous group. Despite legally enforced table salt iodization, the region shows the characteristics of mild iodine deficiency. In addition to lower UIC, goitrous children have lower serum selenium levels compared to the nongoitrous ones. Thus, selenium deficiency plays an important role in goiter endemics in Turkey. It can be postulated that table salt iodization might not be enough for the preventive measures of goiter, but informing people about the correct ways of iodized salt consumption, enforcing the iodization of industrial salts, and, as important as these measures, taking selenium deficiency into consideration are essential for preventing goiters in endemic areas.

Serum insulin-like growth factor-I (IGF-I) and insulin-like growth factor binding protein-3 (IGFBP-3) levels were investigated in 31 children living in an endemic goiter area and 33 healthy subjects living in an nonendemic area. Serum lGF-I and IGFBP-3 levels of iodine- and selenium deficient children were found to be lower than those of control subjects (p

To investigate whether there is a change in bone turnover-related biochemical markers and bone mineral density of children with constitutional delay of growth and puberty (CDGP) in the prepubertal period.We measured serum calcium, phosphorus, alkaline phosphatase, parathormone, 25-OH vitamin D, osteocalcin, osteoprotogerin and urinary deoxypyridinoline levels (D-pyd), and bone mineral density (BMD) in 31 prepubertal boys with CDGP. These children were compared with 22 prepubertal boys with familial short stature (FSS) and 27 normal prepubertal boys.Urinary D-pyd was significantly high in CDGP group as compared to control group (p=0.010). Volumetric BMD did not significantly differ between CDGP, FSS, and control groups (p=0.450). Volumetric BMD and urinary D-pyd levels of FSS and control groups were similar. Mean or median levels of calcium, phosphorus, alkaline phosphatase, parathormone, and osteoprotegerin did not significantly differ between CDGP, FSS, and control groups.Our data suggest that prepubertal boys with CDPG have normal bone turnover. However, their significantly higher urinary D-pyd levels relative to those of FSS and control groups might be an indicator of later development of osteoporosis. Therefore, long-term follow-up studies monitoring bone mineral status of prepubertal boys with CDPG from prepuberty to adulthood are needed to better understand bone metabolism of these patients.

We describe a newly diagnosed Turkish adolescent female with Dyskeratosis congenita along with the novel ocular finding of corneal limbal insufficiency. Corneal limbal insufficiency was suggested to be a premature aging sign resulting from a deficiency in corneal stein cell activity, a biological process caused by underlying telomeric defect in this disease. Pediatr Blood Cancer 2009;53:95-97. (C) 2()09 Wiley-Liss, Inc.

A 5-year-old child with precocious puberty and complete masculinization of the genitalia was diagnosed to have 21-hydroxylase deficiency. The patient was also found to have a prostate gland and increased prostate-specific antigen. The presence of a prostate and its relation to prostate-specific antigen and prostate adenocarcinoma are discussed in the light of the relevant literature.

Aim: To investigate the relationship of adiponectin, leptin, resistin and RBP4 levels in obese and metabolic syndrome children with nonalcoholic fatty liver disease (NAFLD). Patients & methods: Group I consisted of 63 obese children with liver steatosis, group II consisted of 12 obese children with elevated serum ALT activity from group I, and group III included 85 obese children without liver steatosis. Results: Leptin levels were higher in the NAFLD children than in the control group. Serum RBP4 levels in obese children with NAFLD were higher than those in obese children without NAFLD and controls. Adiponectin and resistin levels were negatively correlated and RBP4 levels positively correlated with ALT activity and ultrasonographic grading. Conclusion: These data suggest that adiponectin, resistin and RBP4 may have a role in the pathogenesis of NAFLD in obese children. Adiponectin, leptin, resistin and RBP4 may be suitable markers for predicting metabolic syndrome and NAFLD.

Aim: To investigate the relationship between serum adiponectin, resistin and RBP4 levels and the components of metabolic syndrome. Patients & methods: Serum adiponectin, resistin and RBP4 levels were detected and analyzed in 148 8–18-year-old Turkish obese pubertal children with/without metabolic syndrome. Results: Adiponectin and resistin concentrations were significantly inversely correlated with BMI standard deviation score, homeostatic model assessment insulin resistance, waist circumference, triglyceride levels and diastolic and systolic blood pressure, and were directly correlated with high-density lipoprotein cholesterol. RBP4 concentrations were directly correlated with homeostatic model assessment insulin resistance, waist circumference, triglyceride levels and diastolic and systolic blood pressure, and inversely correlated with high-density lipoprotein cholesterol. Conclusion: Adiponectin, RBP4 and, in particular, resistin levels may be used as suitable predictive biomarkers of metabolic syndrome.

Background/Aims: Congenital hyperinsulinism (CHI) denotes an inappropriate secretion of insulin from pancreatic β-cells in the presence of a low blood glucose level due to various genetic causes. Diazoxide is the first-line medical treatment for CHI. In case of failure, a somatostatin analogue called octreotide is used. A prolonged QT interval is an unusual side effect of octreotide which can be lethal if unrecognized. Case Presentation: We report on a 35-day-old infant who was diagnosed with CHI on the 3rd day of his life and underwent pancreatectomy due to failure of medical treatment at 8 months. His genetic analysis revealed a compound heterozygosity for a novel missense mutation (p.Met115Val) and a nonsense mutation (p.Trp1339X) in the ABCC8 gene. Furthermore, at the 6th month of follow-up, a long QT (0.49 s) was determined by ECG examination, which was normalized following discontinuation of octreotide treatment after pancreatectomy. Thus, the long QT was considered to be secondary to octreotide medication. Conclusion: We recommend ECG monitoring before and during octreotide treatment in order to recognize a prolonged QT interval and to prevent related complications in cases with congenital hyperinsulinemia.

Serum levels of carboxyterminal propeptide of type 1 procollagen (PICP) and aminoterminal propeptide of type III procollagen (PIIINP) can be used as markers of bone formation and the evaluation of children with growth disorders. The authors measured the serum levels of these collagens by radioimmunoassay (RIA) insulin-like growth factor I (IGF-I) levels (by immunoradiometric assay) and insulin-like growth factor binding protein-3 (IGFBP-3) levels (by RIA) in 24 children aged 4-14 years with chronic renal failure (CRF) (n = 12 dialysis; n = 12 non-dialysis) and 12 age-matched healthy controls to find out whether these parameters have a prognostic or therapeutic value on monitoring of growth retardation in CRF. Mean serum IGFBP-3 and PIIINP levels in the dialysis patients were higher than in the control group; the difference between the groups was statistically significant (P < 0.05). It seemed that the pubertal stage of the patients did not affect the levels of PICP PIIINP and IGFBP-3 while serum IGF-I levels in pubertal patients were significantly higher than those in prepubertal patients (P < 0.001). There was no significant correlation between PICP and PIIINP in any patients. Neither PICP nor PIIINP correlated with height z-score bone age IGF-I or IGFBP-3. It was concluded that the increased serum IGFBP-3 and PIIINP levels in chronic renal disease are poor prognostic indicators leading to progressive renal failure and end-stage renal disease (ESRD). Further investigations into the effects of these collagens on growth failure associated with CRF are needed. (authors)

OBJECTIVE To investigate vitamin D status in children with Hashimoto thyroiditis. SUBJECTS AND METHODS The study group consisted of 78 children recently diagnosed as Hashimoto thyroiditis and 74 subjects as the control group. Parameters of calcium metabolism, thyroid function tests, and 25-hydroxyvitamin D [25(OH)D] levels were measured. RESULTS Vitamin D deficiency rate was significantly higher in the Hashimoto group compared with the control subjects (73.1% vs. 17.6%, p < 0.0001). In the Hashimoto group, mean 25(OH)D levels were significantly lower compared with the control group (31.2 +/- 11.5 versus 57.9 +/- 19.7 nmol/L, p < 0.001) and was inversely correlated with the anti-thyroid peroxidase (anti-TPO) levels (r = -0.30, p = 0.007). CONCLUSION The higher vitamin D deficiency rates besides lower vitamin D levels in the Hashimoto group together with the inverse correlation between vitamin D and anti-TPO suggest that vitamin D deficiency may have a role in the autoimmune process in Hashimoto thyroiditis in children.

4-Chlorophenoxy acetic acid (4-CPA) is one of the plant growth regulators that are widely used in greenhouses to increase yield and the quality of products. Plant growth regulators do not produce significant effects on organisms; however, their remnants on plants due to either high dose or inappropriate use may lead to certain adverse effects on humans. High doses of 4-CPA have been reported to cause hepatocellular necrosis. In our study, we investigated the oxidant and anti-oxidant parameters in the liver tissues of male and female rats exposed to 4-CPA before sexual maturity. The study was implemented on 20 day-old, 80 Wistar albino rats. Forty rats received 4-CPA/day until 50 days of age. The rats were randomized into five groups (a control group, a saline group and three 4-CPA groups that received 25 to 50 to 100 mg/kg/day, respectively); and each group were further divided according to gender, making a total of 10 groups. Comparison of each gender in control and saline groups showed no difference for MDA, GSH and NOx levels. However, exposure to 4-CPA caused a dose-dependent increase in MDA and a decrease in NOx levels. These findings may be the result of 4-CPA’s effects on lipid peroxidation and inflammatory reactions in liver tissue. Key words: Liver, plant growth regulator, endocrine disrupters, oxidative stress, 4-Chlorophenoxy acetic acid (4-CPA).

The age-related values of penile length must be known to determine abnormal penis sizes and to follow the treatment of underlying diseases. The aim of this study is to evaluate abnormal penile length in Turkish children by establishing novel reference values for Turkish population and to compare the mean penile length and other parameters with alternates from different ethnic populations and geography.This cross-sectional study was conducted on a voluntary basis between November 2008 and November 2009 from four centres in Turkey and comprised of 1278 healthy volunteered prepubertal children. Complete stretched penile length and penis circumference measurements were used for penile length and penis circumference evaluations, respectively. All measurements were taken twice by only one investigator, and mean values were recorded.Penile length and penis circumference for every age group were obtained, percentile curves were established and these findings were compared with the results of previous studies. Significant differences were found between penile length of Turkish children and recently used reference values.With this study, novel reference values for penile length in prepubertal children were presented to the literature.

Introduction: It has currently been understood that monitoring diabetic patients only with metabolic variables is insufficient, and thus, quality of life assessments have been initiated. The aim of the present study was to compare the quality of life scored between diabetic and healthy children and to investigate the factors affecting the quality of life in diabetic patients. Material-Method: Sixty-one patients aged between 7-16 years who were followed-up with diagnosis of type 1 Diabetes Mellitus in the Department of Pediatric Endocrinology of Gazi University School of Medicine and 57 age- matched healthy volunteers were enrolled. Patient and control groups were evaluated in terms of quality of life, depressive mood, and accompanying behavioral problems. The assessment of children was performed using Conners’ parent and teacher rating scales, Children Depression Inventory, State-Trait Anxiety Inventory for Children, and Short Form-36 for quality of life, Kid–KINDL questionnaire, Kiddo–KINDL questionnaire, and KINDL questionnaire. Clinical and laboratory characteristics of the diabetic patients were retrieved from their patient files. Results: Short Form-36 general health, vitality, and mental health subdimension scores of type 1 diabetic children were lower than those in the controls (p

Aims: The objective of this study was to investigate the cause of valproate (VPA)-associated weight gain in children. Methods: Eighteen children (10.94 ± 3.78 years) with epilepsy were assigned to VPA therapy. Serum levels of glucose, insulin, cortisol, leptin, neuropeptide Y (NPY), galanin and ghrelin were assessed before (month 0) and after 18 months of therapy. Eighteen age- and gender-matched patients (10.78 ± 3.95 years) were enrolled as the control group. Results: Excess per capita weight of 2.3 kg was determined in the children receiving VPA over 18 months compared to the control group. In these patients, a statistically significant increase in standardized weight score, Homeostasis Model Assessment index, serum leptin, NPY and galanin values was determined at the 18th month compared to those before VPA treatment and in the control group, and there was also a significant decrease in ghrelin values. Conclusion: Increased serum levels of leptin, NPY and galanin play an important role in VPA-associated weight gain in children. While ghrelin is not directly associated with weight gain, its serum levels decline as a response to weight gain.

Purpose The aim of this study was to investigate whether oxcarbazepine (OXC) monotherapy causes weight gain in epileptic children. Methods A total of 22 children with epilepsy (age 3.0–16.4 years) were assigned to OXC therapy. Serum levels of glucose, insulin, cortisol, leptin, neuropeptide Y (NPY), galanin and ghrelin were assessed before OXC therapy (month 0) and after the 6th and 18th months. Results There was no statistically significant difference in weight–standard deviation score (SDS), Height–SDS, BMI–SDS, serum glucose, insulin, cortisol, leptin, NPY, galanin and ghrelin levels between initial values (month 0) and those in the 6th and 18th months after OXC therapy (p > 0.05). Conclusions Our results indicate that OXC therapy causes neither weight change nor alterations in serum glucose, insulin, cortisol, leptin, NPY, galanin and ghrelin levels in children with epilepsy.