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This study examines the development of a STEM-based (Science, Technology, Engineering, and Mathematics) and parent-involved engineering design curriculum for early childhood education and investigates its design principles and contributions to children’s STEM-related learning. This research utilized a design-based research methodology broken into three main phases (preliminary research, prototyping, and assessment). The data were gathered over a 2-year span, and the curriculum was designed and revised during the prototyping phase’s three iterative cycles. This paper presents the findings of a field test that involved two early childhood education teachers, five preschool children, and five parents. Findings validated eight key design principles of the curriculum and revealed that the curriculum contributes to the knowledge, skills, feelings, and dispositions children have towards STEM. The findings suggest that the developed curriculum can be used as a novel way of integrating STEM into early childhood education, with a particular focus on engineering, while encouraging parental involvement.

Melanocortin 4 receptor gene plays an important role in food intake, energy balance, and weight control. The autosomal dominantly inherited MC4R variants cause obesity by causing hyperphagia and decreased sense of satiety. Homozygous variants are rarely reported, and they cause earlier/severe obesity. Our objective is to determine the MC4R gene variant frequency in children and adolescents with familial early-onset obesity. One hundred thirty-nine children and adolescents (57 girls/82 boys) whose weight increase started before the age of 5 years and who had early-onset obesity in at least one of their first-degree relatives were included in the study. Obesity is defined as body mass index (BMI) of ≥ 95th percentile, and as extreme obesity is defined if the BMI ≥ 120% of the 95th percentile or ≥ 35 kg/m2. Children having genetic syndromes associated with obesity and mental retardation or taking drugs that promote changes in eating behavior or weight were excluded from the study. Coding region of the MC4R gene was sequenced by using the Illumina MiSeq Next Generation Sequencing System. The mean age of the patients was 7.3 ± 3.7 years, and the mean BMI SDS was 3.7 ± 0.7. While 118 patients (85%) were prepubertal, 21 patients (15%) were pubertal. Seven different variants were identified in 12 patients by giving a variant detection rate of 8.6%, of these five were previously identified missense variants p.N274S, p.S136F, p.V166I, p.R165W, and p.I291SfsX10. One homozygous variant p.I291SfsX10 (c.870delG) was detected in a severely obese 2-year-old boy, and other variants were heterozygous. Two novel variants were found: p.M200del and p.S188L. By using the in silico analysis software, these novel variants were predicted to be disease causing.Conclusion: MC4R gene variants are quite common in childhood obesity in Turkish population. Screening the variants in MC4R gene is necessary in patients with severe childhood-onset obesity. In such patients, comorbidities of obesity can be seen from early years.What is known• The frequency of MC4R mutations in obese patients was approximately 0–6.3%.What is new• In obese Turkish pediatric population, unlike other European countries, MC4R gene variants are quite common as we found a variant rate of 8.6%• We believe it is necessary to screen the variants in MC4R gene in patients with severe childhood-onset obesity and who had early-onset obesity in at least one of their first-degree relatives in Turkish population.

Carboxypeptidase E (CPE) plays a critical role in the biosynthesis of peptide hormones and neuropeptides in the endocrine system and central nervous system. knockout mice models exhibit disorders such as diabetes, hyperproinsulinaemia, low bone mineral density and neurodevelopmental disorders. Only one patient is described with morbid obesity, intellectual disability, abnormal glucose homeostasis and hypogonadotropic hypogonadism, which was associated with a homozygous frameshift deletion in . Herein are described three siblings with obesity, intellectual disability and hypogonadotropic hypogonadism. Whole exome sequencing (WES) was performed in the index case. Candidate variants were prioritised and segregation of the variant, consistent with the phenotype of the index case, was assessed by Sanger sequencing in affected siblings and parents. WES analysis revealed a homozygous nonsense c.405C>A (p.Y135*) mutation in . Validation and segregation analysis confirmed the homozygous mutation in the index case and his affected siblings. The parents were phenotypically normal heterozygous mutation carriers. This study provides additional evidence of the association between a homozygous nonsense mutation in and a clinical phenotype consisting of obesity, intellectual disability and hypogonadotropic hypogonadism, which may be considered as a new monogenic obesity syndrome.

IntroductionThis study aimed to determine the absorbed radiation doses in radiosensitive female pelvic organs during abdominal computed tomography (CT) imaging using thermoluminescent dosimeters (TLD-100) and an anthropomorphic phantom.MethodsA Computerized Imaging Reference Systems, Inc. female pelvic anthropomorphic phantom was used to simulate realistic human anatomy. TLD-100 dosimeters were positioned within organ-equivalent cavities corresponding to the ovaries, uterus, and urinary bladder. CT scans were performed using a Toshiba Aquilion 64-slice scanner with standard abdominal parameters (120 kVp, 300 mA, 5 mm slice thickness, pitch 1.0). After exposure, TLDs were read using a Harshaw 3500 reader, and organ doses were calculated based on calibration coefficients.ResultsThe urinary bladder exhibited the highest mean absorbed dose (27.89 mGy), followed by the fundus uteri (26.34 mGy), left ovary (23.22 mGy), cervix uteri (21.88 mGy), and right ovary (20.91 mGy). Lower doses were measured in deeper or more peripheral regions, such as the recessus rectouterina (19.37 mGy) and the medulla spinalis (16.73 mGy).ConclusionThe results indicate that even under standard clinical protocols, radiosensitive organs of the female reproductive system receive measurable radiation doses during abdominal CT. These findings emphasize the need for protocol optimization and shielding strategies to minimize exposure, especially in reproductive-age women.

The database http://cedd.saglik-network.org (CEDD-NET) has been operating since 2013 in Turkey. All pediatric endocrinologists can propose projects to this network. The aim of our study was to determine the impact of CEDD-NET on the transformation of multicenter studies into scientific publications and assess the academic characteristics of the studies that have been transcribed into publication. All the studies that were opened to patient admission on the website between August 26, 2013 and March 1, 2021 were reviewed. A total of 30 studies were accepted and opened for data entry. The median data collection period was 12 (1.5-24) months, while the median number of researchers participated was 23 (3-180), the median number of cases was 120 (26-192). The average cost was $2113 (1370-3118). Out of 30 studies, data entry was completed for 27. Sixteen publications were produced from 14 studies, 13 ot them have not published yet. The median time from the end of data entry to publication of the study was 686 (168-1608) days. While the median impact factor of the journals in which the studies were published was 1.803 (1.278-5.399), the median number of citations was 6.5 (0-49), and cited by 99 times in Web of Science indexed journals in total. CEDD-NET appears to be productive and effective as all the publications are of high quality that have been published in the Q1-Q2 categories. This study demonstrated the benefits and necessity of establishing nationwide databases, even covering more than one country, in specialized branches, such as pediatric endocrinology where rare diseases are of concern.

Aim:Gonadotropin-releasing hormone analogs (GnRHa) are standard medical treatments for precocious puberty. Studies on their side effects in adults have shown that these drugs can cause changes in electrocardiography (ECG), along with some cardiovascular effects; however, the number of studies on children is limited. This study investigated the effects of these drugs on ECG parameters in children diagnosed with central precocious puberty (CPP).Materials and Methods:This prospective study included 44 girls who were initiated GnRHa treatment and diagnosed with CPP. ECG was performed before treatment and repeated after 6 months of treatment.Results:The mean age of the children was 9.13±1.55 years. Leuprolide acetate (3.75 mg IM) was administered to all of the patients following the standard protocol. A comparison of the pre-treatment and 6-month ECG parameters revealed a prolonged QT interval after treatment, with a statistically significant difference (p<0.001). There were no significant differences in the pre- and post-treatment values of PR, QRS, QT interval, QTc interval, QT dispersion, or QTc dispersion (p>0.05).Conclusion:Despite a significant increase in QT interval on ECG with GnRHa compared to pre-treatment ECGs, this increase was attributed to a variability in heart rate. Even if regular ECG monitoring is considered after initiation of GnRHa treatment, they are believed to be safe drugs in children.

The potential of children’s picture books to provide knowledge to support STEM education is high. Engineering is a field about which children may have little awareness or understanding. Children’s picture books can be an important tool in introducing engineering to children. In this study, we investigate children’s picture books written in or translated into Turkish for 3–6-years-old children to address two major points: (a) how engineers are portrayed and (b) how the engineering design process is included. We examined the content of 691 picture books and identified 23 books, in accordance with specific criteria (e.g., identification through predefined keywords), relevant to engineers and engineering. Findings indicated that characters who think and work like engineers were often portrayed as male characters rather than females. While 21 of the 23 books sampled discussed the engineering design process, none of these books identified all the steps in such a process. Children’s picture books are important tool to introduce engineering to children. We recommend the publication of more engineering-centered picture books to provide children with information about engineers and the engineering design process, in support of STEM education. However, it is important that such books do not allow misunderstandings to emerge about the field of engineering in the creation of characters or the messages conveyed.

Introduction It has been shown that cardiac functions begin to deteriorate in growth hormone (GH) deficiency even in childhood. However, little is known about how GH deficiency affects arrhythmogenesis. The aim of this study was to evaluate the parameters of P wave dispersion (Pd), QT dispersion (QTd), corrected QT (QTc) dispersion (QTcd), T wave peak-to-end (Tp-e) interval, Tp-e/QT ratio, and Tp-e/QTc ratio in children with GH deficiency. This study also aimed to evaluate the relationship of these parameters with insulin-like growth factor 1 (IGF-1) and insulin-like growth factor binding protein 3 (IGFBP-3). Method In the study, records of children diagnosed with GH deficiency in Adana City Training and Research Hospital Pediatric Endocrine Outpatient Clinic between September 2021 and December 2022 were retrospectively reviewed. The control group consisted of children in the same age group who applied to the Emergency Outpatient Clinic with a complaint of chest pain and no pathological finding was detected. The electrocardiograms (ECGs) of all patients were retrospectively evaluated. Results There were a total of 82 children in the study, 41 of whom were diagnosed with GH deficiency and 41 in the healthy control group. The age and male/female ratio of children with GH deficiency were similar to those in the control group (p>0.05). There were 27 (66%) children with complete GH deficiency and 14 (34%) children with partial GH deficiency. P wave dispersion was similar in both GH-deficient children and control group children. It was also similar in children with complete and partial GH deficiency (p>0.05). QT and QTc dispersions were found to be increased in children with GH deficiency, although not statistically significant, compared to the control group (p>0.05). Tp-e interval, Tp-e/QTmax (longest QT interval), and Tp-e/QTcmax (longest QTc interval) ratios were increased in children with GH deficiency compared to the control group (p=0.001, p=0.003, and p=0.001, respectively). QT and QTc dispersion, Tp-e interval, Tp-e/QTmax, and Tp-e/QTcmax ratios were found to be increased in children with complete GH deficiency compared to children with partial GH deficiency, but the difference was not significant (p>0.05). No correlation was found between these ECG parameters and IGF-1, IGFBP-3, and peak GH levels after stimulation tests (p>0.05). Conclusion We found in our study that the Tp-e interval was longer and Tp-e/QT and Tp-e/QTc ratios were increased in children with GH deficiency. These results suggest that the risk of ventricular arrhythmias in children with GH deficiency may start to increase from childhood. However, further prospective studies are needed to confirm our results.

Introduction. Hyperandrogenism is a clinical condition in girls, resulting from excessive androgen production originating from the adrenal glands or ovaries. The measurement of androgen hormones plays an essential role in supporting the clinical diagnosis. These hormone levels can be assessed using immunoassay methods or liquid chromatography – tandem mass spectrometry (LC-MS/MS). Our study aimed to assess the efficacy of hormone measurement with both methods in girls clinically diagnosed with hyperandrogenism. Methods. Girls presenting with hyperandrogenism were included in this cross-sectional retrospective study. The exclusion criteria included a diagnosis of precocious puberty, classical congenital adrenal hyperplasia (CAH), adrenocortical tumors, and the use of medications known to affect androgen levels. Hormones measured simultaneously by both methods were compared. Regression analysis was performed to adjust hormone levels for age and pubertal stage. Receiver operating characteristic (ROC) analysis was performed based on diagnosis, and androgen hormones with the highest specificity and sensitivity for diagnosis were identified. Results. A total of 96 girls with hyperandrogenism were included in the study. 60 (62.5%) were diagnosed with premature adrenarche (PA), 31 (32.3%) with polycystic ovary syndrome (PCOS), and 5 (5.2%) with non-classical congenital adrenal hyperplasia (NCCAH). Dehydroepiandrosterone sulfate (DHEAS) measured by LC-MS/MS was significantly lower (p<0.001) but less concordant with clinical diagnosis than electrochemiluminescence immunoassay in PA cases. The androgen hormone with the highest area under the curve (AUC) value was androstenedione for PCOS (AUC: 0.792), and 17-hydroxyprogesterone (AUC: 0.994) using LC-MS/MS for NCCAH. Conclusions. The measurement of DHEAS levels by both methods has low specificity. Androstenedione and total testosterone measured by LC-MS/MS had the highest sensitivity and specificity in PCOS.