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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 < 0.001). The mean birth length was 1.3 cm shorter and mean birth weight was 0.36 kg lower than that of the normal population. The mean age at diagnosis was 10.1 ± 4.4 years. Mean height, weight and body mass index standard deviation scores at presentation were -3.1 ± 1.7, -1.4 ± 1.5, and 0.4 ± 1.7, respectively. Patients with isochromosome Xq were significantly heavier than those with other karyotype groups (P = 0.007). Age at presentation was negatively correlated and mid-parental height was positively correlated with height at presentation. Mid-parental height and age at presentation were the only parameters that were associated with height of children with TS. The frequency of SGA birth was found higher in preterm than term neonates but the mechanism could not be clarified. We found no effect of karyotype on height of girls with TS, whereas weight was greater in 46,X,i(Xq) and 45,X/46,X,i(Xq) karyotype groups.

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.

NORMALITIES; PREVALENCE; GIRLS 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. ALI, SAYGIN/0000-0001-6552-2801; Turan, Serap/0000-0002-5172-5402; ozkan, Behzat/0000-0002-9153-8409; Eren, Erdal/0000-0002-1684-1053; binay, cigdem/0000-0002-7749-8818; yuksel, bilgin/0000-0003-4378-3255; gurbuz, fatih/0000-0003-2160-9838

Lactococcus lactis is an important lactic acid bacteria (LAB) species that is used for the manufacture of dairy products, such as cheese, buttermilk, and other fermented products. The predominant function of this bacterium in dairy fermentation is the production of lactic acid, as its major fermentation end-product that contributes to preservation and microbial safety of the product. Moreover, L. lactis is frequently encountered in natural ecosystems such as in (rotting) plant material. Due to restricted energy source availability, natural microbial communities commonly live in a situation that can be characterized as ‘hunger’, which is different from strict nutrient-starvation. As a consequence, environmental microbes commonly grow at very low-growth rates as compared to laboratory cultures. Analogously, microorganisms can experience such nutrient-poor conditions in diverse industrial fermentation applications. For example, LAB encounter extreme low or no energy source availability during the extended ripening process of cheeses or dry sausages, which can take months. Despite these harsh environmental conditions, many LAB are able to remain viable in these processes for months and sustain a low-level metabolic activity, which plays an important role in their contribution to flavor and aroma formation in the product matrix. In this thesis, the quantitative physiology of L. lactis at near-zero specific growth rates was studies, employing both metabolic and genome-wide transcriptome studies in an experimental set-up of carbon-limited retentostat cultivation. Chapter 2 describes how retentostat cultivation enables uncoupling of growth and non-growth related processes in L. lactis, allowing the quantitative analysis of the physiological adaptations of this bacterium to near-zero growth rates. In chapter 3, transcriptome and metabolome analyses were integrated to understand the molecular adaptation of L. lactis to near-zero specific growth rate, and expand the studies in chapter 2 towards gene regulations patterns that play a profound role in zero-growth adaptation. Chapter 4 describes the enhanced robustness to several stress conditions of L. lactis after its adaptation to extremely low-specific growth rate by carbon-limited retentostat cultivation. In this chapter correlations were modelled that quantitatively and accurately describe the relationships between growth-rate, stress-robustness, and stress-gene expression levels, revealing correlation coefficients for each of the varieties involved. Chapter 5 evaluates the distinction between the transcriptome responses to extended carbon-limited growth and severe starvation conditions, where the latter condition was elicited by switching off the medium supply of the retentostat cultures described in chapter 1. Chapter 6 highlights the comparison of the physiological and molecular adaptations of industrially important microorganisms towards carbon-limited retentostat conditions. In conclusion, this thesis describes the quantitative physiological, metabolic, and genome-wide transcriptional adaptations of L. lactis at near-zero specific growth rates induced by carbon source limited retentostat cultivation, and compares these molecular adaptations to those elicited by strict carbon-starvation conditions.

This study aimed to adapt the 'Beliefs about Climate Change' (BACC) scale developed by Liarakou, Athanasiadis and Gavrilakis (2011) to Turkish students by administering it to a group of 6th, 7th and 8th graders. The scale was sent to expert academicians after translation to ensure equivalency across languages and language equivalency was evaluated according to the Kendall coefficient of concordance. Results of exploratory versus confirmatory factor analysis undertaken to determine the factor structure of the BACC showed a single dimensional structure. A relationship above 0.34 was found for all items of the scale as a result of item-total correlation for the BACC. Item factor loads of BACC for each item were between 0.34 and 0.61. The Cronbach alpha internal consistency coefficient and test-retest correlation for the scale were found to be 0.85 and 0.78, respectively. Correlation analysis implemented for criterion-related validity of the BACC also showed a meaningful relationship.

This paper describes the metabolic adaptation of Lactococcus lactis during the transition from a growing to a non-growing state using retentostat cultivation. Under retentostat cultivation, the specific growth rate decreased from 0.025 h-1 to 0.0001 h-1 in 42 days, while doubling time increased to more than 260 days. Viability of the overall culture was maintained above 90% but included approximately 20% damaged cells, which had lost their colony forming capacity on solid media. Although culture biomass and viability had reached a steady-state after 14 days of retentostat cultivation, the morphology of the cells changed from coccus-to-rod shape at later stages of retentostat cultivation, by which the cell’s surface to volume ratio was estimated to increase 2.4-fold. Furthermore, the metabolic patterns switched between homolactic and mixed-acid fermentation during the retentostat cultivation. Retentostat cultivation enabled the calculation of accurate substrate- and energy-related maintenance coefficients and biomass yields under nongrowing conditions, which were in good agreement with those calculated by extrapolation from chemostat cultivations at high dilution rates. In this study, we illustrate how retentostat cultivation allows decoupling of growth and non-growth associated processes in L. lactis, enabling the analysis of quantitative physiological responses of this bacterium to near zero-specific growth rates.

This study aimed to adapt the 'Beliefs about Climate Change' (BACC) scale developed by Liarakou, Athanasiadis and Gavrilakis (2011) to Turkish students by administering it to a group of 6th, 7th and 8th graders. The scale was sent to expert academicians after translation to ensure equivalency across languages and language equivalency was evaluated according to the Kendall coefficient of concordance. Results of exploratory versus confirmatory factor analysis undertaken to determine the factor structure of the BACC showed a single dimensional structure. A relationship above 0.34 was found for all items of the scale as a result of item-total correlation for the BACC. Item factor loads of BACC for each item were between 0.34 and 0.61. The Cronbach alpha internal consistency coefficient and test-retest correlation for the scale were found to be 0.85 and 0.78, respectively. Correlation analysis implemented for criterion-related validity of the BACC also showed a meaningful relationship.

PubMed ID: 21750637, 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.

Background: We aimed to determine the status of and factors associated with adolescent health care delivery and training in Europe on behalf of the European Paediatric Association-UNEPSA. Materials and methods: A questionnaire was mailed to the presidents of 48 national paediatric societies in Europe. For statistical analyses, non-parametric tests were used as appropriate. Results: Six of the countries had a paediatric (PSPCA), 14 had a combined and nine had a general practitioner/family doctor system for the primary care of adolescents (GP/FDSA). Paediatricians served children 17 years of age or older in 15 and 17, up to 16 years of age in three and six, and up to 14 years of age in six and six countries in outpatient and inpatient settings, respectively. Fifteen and 18 of the countries had some kind of special inpatient wards and outpatient clinics for adolescents, respectively. Twenty-eight of the countries had some kind of national/governmental screening or/and preventive health programmes for adolescents. In countries with a PSPCA, the gross national income (GNI) per capita was significantly lower than in those with a GP/FDSA, and the mean upper age limit of adolescents was significantly higher than in those with the other systems. In the eastern part of Europe, the mortality rate of 10-14 year olds was significantly higher than that in the western part (p=0.008). Training in adolescent medicine was offered in pre-graduate education in 14 countries in the paediatric curriculum and in the context of paediatric residency and GP/family physician residency programmes in 18 and nine countries, respectively. Adolescent medicine was reported as a recognised subspecialty in 15 countries and as a certified subspecialty of paediatrics in one country. In countries with a PSPCA, paediatric residents were more likely to be educated in adolescent medicine than paediatric residents in countries with a GP/FDSA. Conclusion: The results of the present study show that there is a need for the reconstruction and standardisation of adolescent health care delivery and training in European countries. The European Paediatric Association-UNEPSA could play a key role in the implementation of the proposals suggested in this paper. © 2008 The Author(s).

Udgivelsesdato: 2009-Sep OBJECTIVES: To examine overweight prevalence and its association with demographic and lifestyle factors in 11-15 year olds in the HBSC 2005-2006 survey. METHODS: Self-reports of height, weight, eating patterns, physical activity and sedentary behaviours were obtained from nationally representative samples in 41 countries (n=204,534). RESULTS: Overweight prevalence was highest in USA (28.8 %) and lowest in Latvia (7.6 %). In most countries, overweight was more prevalent in boys than girls. Overweight was consistently negatively associated with breakfast consumption and moderate to vigorous physical activity; OR range: 0.48-0.79 and 0.50-0.78, respectively. CONCLUSION: Overweight prevalence in youth remained high across the countries examined. The primary factors linked to overweight were breakfast consumption and physical activity. These data should contribute to formulating preventive programs and policies.

Objective: To investigate associations of daily breakfast consumption (DBC) with demographic and lifestyle factors in 41 countries. Methods: Design: Survey including nationally representative samples of 11-15 year olds (n = 204,534) (HBSC 2005-2006). Statistics: Multilevel logistic regression analyses Results: DBC varied from 33% (Greek girls) to 75% (Portuguese boys). In most countries, lower DBC was noticed in girls, older adolescents, those with lower family affluence and those living in single-parent families. DBC was positively associated with healthy lifestyle behaviours; and negatively with unhealthy lifestyle behaviours. Conclusion: Breakfast skipping deserves attention in preventive programs. It is common among adolescents, especially girls, older adolescents and those from disadvantaged families. The results indicate that DBC can serve as an indicator to identify children at risk for unhealthy lifestyle behaviours.