Purpose: It was aimed to compare circulating levels of ghrelin, leptin, peptide YY (PYY), and neuropeptide (NPY) between girls with idiopathic central precocious puberty (ICPP) and prepubertal girls, as well as to evaluate alterations in these hormone levels and body composition during leuprolide acetate treatment in girls with ICPP. Methods: This prospective study was conducted on girls with isolated premature thelarche (IPT), girls with ICPP, and age-matched prepubertal controls. Anthropometric measurements, body composition analysis and appetite-regulating hormone level measurements were performed in each group and also at the 6th and 12th months of the leuprolide acetate treatment for the girls with ICPP. Results: Seventy-three girls participated in the study (24 girls with ICPP, 28 with IPT, and 21 prepubertal controls). No significant differences were observed in ghrelin, leptin, PYY, and NPY levels among the three groups. Leuprolide acetate treatment resulted in increased leptin, decreased PYY and NPY levels, and no significant changes in ghrelin. Despite no significant change in body mass index standard deviation score (BMI SDS), body fat percentage increased during treatment. Conclusion: While appetite-regulating hormones do not seem to directly contribute to precocious puberty pathogenesis, puberty blockade was shown to lead to altered levels of these hormones along with changes in body composition.

This study aims to explore the long-term endocrine and gonadal effects of chemotherapy and radiotherapy in female acute lymphoblastic leukemia (ALL) patients. A cohort study included girls diagnosed with ALL and treated between 2000 and 2020. Patients with at least 2 years elapsed since treatment completion were included. Endocrinological evaluations included anthropometric measures and pubertal status, as well as fasting insulin, glucose, lipid levels, and hormone assessments for adrenal, and thyroid functions. Reproductive functions were evaluated based on gonadotropin, estradiol, and anti-Müllerian hormone (AMH) levels. A total of 51 female patients were included. At the time of study participation, the mean age was 14.7 years, and the mean time since treatment completion was 9.4 years. At least one endocrine disorder was present in 39.2% of participants, with dyslipidemia, insulin resistance, and obesity being the most common. Low AMH levels (< 1.1 ng/dL) were found in 41.6%, particularly in those who underwent bone marrow transplantation. A significant positive correlation was found between the time elapsed since treatment and AMH levels ( p < 0.001, r = 0.612), while age at diagnosis, risk group (standard, intermediate or high risk), and cranial radiotherapy showed no significant associations. A substantial proportion of ALL survivors developed endocrine complications, with ovarian reserve compromised in over 40% of cases. Notably, this is the first cohort study to demonstrate a significant positive correlation between AMH levels and the time elapsed since treatment, suggesting a potential for gonadal recovery except in those exposed to intensive chemotherapy or transplantation.

Aim: Growth differentiation factor-15 is a novel biomarker of increasing importance in cardiovascular diseases. This study aimed to evaluate the relationship between ventricular measurements assessed by cardiac magnetic resonance imaging (MRI) and serum growth differentiation factor-15 levels in children with surgically corrected tetralogy of Fallot.Materials and method: Serum growth differentiation factor-15 levels were measured in 40 patients (mean age: 15.2 ± 2.9 years; 52.5% male; 87.5% NYHA I). End-diastolic volume index, end-systolic volume index, and ejection fractions of both ventricles and pulmonary regurgitation fraction were measured on cardiac MRI. The correlation between growth differentiation factor-15 levels and cardiac MRI parameters of the patients was investigated. Also, growth differentiation factor-15 levels of the patients were compared with healthy controls since reference values have not been determined in children.Results: The mean growth differentiation factor-15 level was 254.9 ± 6.3 pg/ml in the patient group. There was no correlation between growth differentiation factor-15 levels and cardiac MRI parameters in patients. Also, there was no significant difference in growth differentiation factor-15 levels between the patients and control groups.Conclusion: The serum levels of growth differentiation factor-15 were uncorrelated with ventricular size, function, and pulmonary regurgitation fraction assessed by cardiac MRI in children with operated tetralogy of Fallot. Moreover, growth differentiation factor-15 levels were not different in these patients from healthy children.

Objectives: Silver–Russell syndrome (SRS) is a rare imprinting disorder characterized by intrauterine and postnatal growth retardation. Its genetic etiology shows a heterogeneous distribution. This study aimed to evaluate the clinical characteristics of children diagnosed with SRS, their response to growth hormone therapy, and compare the data of genetically confirmed and clinically diagnosed SRS cases. Methods: A total of 69 patients were included in the study. Genetically confirmed cases were considered Group 1, and cases with a clinical diagnosis according to the Netchine–Harbison scoring system were considered Group 2. The anthropometric data of the patients at birth, at the time of diagnosis, before and during the first year of growth hormone (GH) treatment, final height-SDS values of patients who reached final height, and accompanying comorbidities were recorded. Results: In Group 1, 75.8 % had hypomethylation in the ICR1 region, 13.7 % had maternal uniparental disomy 7, 6.8 % had an IGF-2 mutation, and 3 % had a duplication in the 11p15 region. Central precocious puberty, gastroenterological, and neurologic comorbidities were found to be more frequent than those from other systems. Final height-SDS was −2.32 ± 1.57 (n=5) in Group 1 and −2.41 ± 0.86 (n=5) in Group 2. Conclusions: 11p15 LOM was the most common genetic disorder in children with SRS in our case series. Gastroenterological problems and neurologic complications were observed frequently in these cases. Central precocious puberty was more commonly observed compared to the general population. The duration of treatment was the most critical factor in the success of GH therapy.

Cystic fibrosis (CF) is a chronic genetic disorder characterized by pancreatic insufficiency and lung disease. Advancements in highly effective modulator therapies (HEMTs) have improved life expectancy, shifting the focus to endocrine comorbidities, such as CF-related diabetes (CFRD) and bone disease (CFRBD). Therefore, current guidelines recommend routine screening for diabetes and osteoporosis in people with cystic fibrosis (PwCF) starting from age of 10 years. Increased risk of osteoporosis has been shown in type 1 and type 2 diabetes; however, there are limited studies evaluating the impact of glucose metabolism disorders on osteoporosis in children with cystic fibrosis. Therefore, this study investigates the impact of glucose metabolism disorders on PwCF. This cross-sectional retrospective study included 81 PwCF, aged between 10 and 21 years, who were screened routinely for diabetes and bone metabolism between 2019 and 2024. Data on demographics, CFTR variants, glucose metabolism, and biochemical bone parameters, including calcium, phosphorus, ALP, PTH, vitamin D levels with bone mineral density (BMD) of L1-L4 lumber spine were analyzed. Cases were categorized as normal, indeterminate, impaired glucose tolerance (IGT), or CFRD based on OGTT. Statistical analyses were conducted to determine factors affecting BMD, including pairwise comparison and multivariate regression analysis. Of the 81 cases, 55 (67.9%) had normal glucose tolerance, 9 (11.1%) had indeterminate (INDET), 9 (11.1%) had impaired glucose tolerance (IGT), and 8 (9.9%) had CFRD. IGT and CFRD cases demonstrated significantly lower body mass index (BMI), lung function, and BMD z-score than the normal group ( p < 0.05). HbA1c had the negative association with BMD z-score ( β = -0.36 per %1 increase in HbA1c, p < 0.001), while elevated BMI levels had positive relation ( β = 0.28 per 1 kg/m ² increase in BMI, p = 0.009 ) . HEMT showed no significant impact on glucose or bone metabolism, likely due to short treatment durations. Conclusions : Impaired glucose metabolism has a significant association with BMD in PwCF. Integrated monitoring of glucose and bone metabolism, along with a multidisciplinary approach is essential to optimize outcomes and reduce complications. What is Known : • Cystic fibrosis-related diabetes (CFRD) is the most common non-pulmonary comorbidity in CF .• Impaired glucose metabolism has been associated with reduced bone mineral density and increased fracture risk. What is New : • This study demonstrates that even early glucose metabolism disorders are associated with reduced bone mineral density in CF patients .• Higher HbA1c levels were found to be associated with lower bone mineral density, highlighting the relationship between hyperglycemia and bone health in CF .

Background: Achieving optimal glycemic control in children with type 1 diabetes(T1D) is challenging. Insulin glargine U300 offers a flatter pharmacodynamic profile and longer duration of action than glargine U100, but pediatric real-world data are limited. This study evaluated the effects of switching from U100 to U300 on glycemic control, body composition, pain, and quality of life. Methods: This prospective, single-center observational study included children aged 6–20 years with T1D who switched from U100 to U300. Assessments were performed at baseline, week12, and week24. The primary endpoint was change in HbA1c; secondary endpoints included insulin dose(U/kg), hypoglycemia frequency, 3:00 a.m. glucose, lipid parameters, body composition, Numeric Rating Scale (NRS) for pain, and Pediatric Quality of Life Inventory (PedsQL) scores. Participants were stratified by baseline HbA1c (< 9% vs. ≥ 9%). Results: Sixty-three participants (mean age 14.98 ± 3.53 years) were analyzed. Basal insulin dose increased significantly from 0.48 ± 0.12 U/kg/day on Gla-100 to 0.51 ± 0.14 and 0.51 ± 0.15 U/kg/day at weeks 12 and 24, respectively ( p < 0.001). HbA1c levels demonstrated a small but statistically significant change during follow-up ( p = 0.048). (8.20 (5.9–14.8) %→ 8.00 (6.0–12.2) %→ 8.25 (5.7–12.6) %). Participants with HbA1c ≥ 9% showed a significant reduction(ΔHbA1c − 0.50 (− 3.80–1.00) %; p = 0.014), while those < 9% had a mild increase(ΔHbA1c 0.10 (− 0.90–5.30) %). Night-time (03:00 AM) blood glucose levels decreased significantly across the three time points ( p = 0.007), more prominently in the higher HbA1c group. NRS improved markedly ( p < 0.001), though PedsQL scores were unchanged. Conclusions: Switching from U100 to U300 maintained glycemic control with modest insulin dose increases. Greater benefits were observed in patients with higher baseline HbA1c, alongside reduced nocturnal glucose and injection pain.U300 appears to enhance comfort and nighttime stability in pediatric T1D. Clinical trial number: Not applicable.