Damla Gökşen

The optimal intravenous fluid (IVF) administration rate in the treatment of pediatric diabetic ketoacidosis (DKA) is controversial. Our aim is to investigate the efficacy of a modified fixed IVF calculation in pediatric DKA. All patients received rehydration fluid at a standard rate of 3000 mL/m²/24 h. The infusion rate remained constant and was not changed for dehydration severity, age, baseline blood glucose level, daily fluid deficit, or maintenance fluid requirement calculation. Data from 252 patients diagnosed with DKA were recorded. Of them, 126 were treated with the modified IVF procedure and 126 with the International Society for Paediatric and Adolescent Diabetes IVF regimen. Acidosis recovery time was 9 (2-46) and 9 (2-34) hours, respectively ( P = .48). Treatment groups showed no statistically significant differences in treatment-related problems. The modified IVF protocol can be safely and efficiently utilized in the pediatric population, excluding those requiring initial intensive care unit management, without the need to calculate daily fluid deficit or maintenance fluid need.

Objective: The study aims to evaluate the nutrition literacy and healthy lifestyle beliefs among adolescents with Type 1 Diabetes Mellitus (T1D) and identify their relationship to metabolic control. Methods: This cross-sectional study included 153 adolescents aged 10-19 years who were being followed in the outpatient clinic of a tertiary hospital with a diagnosis of T1D for more than one year and had no comorbidities requiring dietary treatment. All participants completed the Adolescent Nutrition Literacy Scale (ANLS) and Healthy Lifestyle Belief Scale for Adolescents (HLBS) during a regularly scheduled medical visit. Hospital records yielded the last three months’ HbA1c levels to assess metabolic control. Targeted metabolic control was defined as ≤7% HbA1c. Results: The median age of the participants was 14.66 (4.42) years (45.8% were female), 58.2% had a normal BMI z-score, and 26.8% were achieving target HbA1c (≤7%). There was a positive correlation between the HLBS total score and the ANLS total score (r=0.39, p=0.001). The ‘interactive’ subfactor of ANLS, which evaluates the capacity to manage the process of nutrition in cooperation with nutrition and health professionals, was correlated with healthy lifestyle beliefs (r= 0.28, p= 0.001). Another ANLS subfactor, the ‘critical’ subfactor, which assesses the capacity to make critical judgments about nutrition-related information and to take actions to raise awareness in this area, was also correlated with healthy lifestyle beliefs (r = 0.37, p = 0.001). There was no difference between HLBS and ANLS total and subfactor scores between those who achieved (≤7%) and failed to achieve (>7%) the HbA1c target (p>0.05). Conclusions: The findings of this study suggest that nutrition literacy is a notable determinant of the adoption of healthy lifestyle beliefs among adolescents with T1D. Further research with a larger sample is needed to investigate indirect effects on diabetes self-management and long-term metabolic outcomes.

Purpose: Acquired hypothalamic obesity is a complex condition resulting from hypothalamic damage, often due to tumors. However, not all children with hypothalamic masses develop obesity; while hypothalamic involvement is a recognised factor, the impact of specific surgical approaches and concurrent endocrine deficits on diverse tumour cohorts requires further elucidation. To evaluate clinical, radiological, surgical, and endocrine factors associated with obesity in children with hypothalamic tumors. Methods: This retrospective single-center study included 34 pediatric patients with hypothalamic tumors (median age at diagnosis: 6.0 years; IQR: 6) followed between 1999 and 2020. Patients with conditions or medications causing obesity were excluded. Data on tumor characteristics, treatment modalities, surgical approach, and endocrine abnormalities were collected. Obesity was defined as BMI > +2 SDS, and patients were grouped as obese and non-obese for comparative analysis. Results: The most frequent tumor types were optic glioma, pilocytic astrocytoma, and craniopharyngioma. Obesity developed in 15 patients (44.1%). The median time to obesity onset was 3.00 months (IQR: 12.50) after diagnosis, with no significant difference in total follow-up duration between obese and non-obese groups (3.00 vs 3.50 years, p=0.471). At diagnosis, the groups differed significantly regarding surgical history, particularly pterional craniotomy (p=0.048), and the presence of adrenal insufficiency (p=0.020) or hypogonadism (p=0.004). However, univariable logistic regression showed that surgical method was not significantly associated with obesity. Obesity was also significantly associated with insulin resistance (p=0.010) and dyslipidemia (p=0.019). Conclusion: In conclusion, hypothalamic obesity develops rapidly in children with hypothalamic tumors, often within the first few months following diagnosis. While surgical intervention—particularly via pterional craniotomy—showed a significant association with obesity in bivariate analysis, this association was not statistically significant in univariable logistic regression. The presence of obesity is closely linked to metabolic disturbances such as insulin resistance and dyslipidemia, as well as adrenal insufficiency and hypogonadism. These findings emphasize the necessity of early metabolic screening and personalized follow-up strategies immediately after diagnosis for this high-risk population.

The Relationship Between HbA1c and GMI and Glucose Metrics in Children and Adolescents with Type 1 Diabetes Using AID - Journal of Clinical Research in Pediatric Endocrinology

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.

Background: Cystic Fibrosis Bone Disease (CFBD) is a known complication in children with CF and may cause serious problems in adulthood or transplantation processes. This study aimed to identify potential predictable risk factors for the development of low BMD by evaluating pediatric patients screened with DXA as a “Heart‐Lung Transplantation Center” and created new strategic plans to improve our CFBD screening program by evaluating our results in literature and guidelines recommendations. Methods: This retrospective cohort study includes 86 children ages 6–18 years with CF who underwent at least one DXA scan between August 2016 and October 2024. Participants were compared according to BMD z scores and the relationship between BMD and disease‐related parameters was evaluated. Results: The rate of DXA screening in our center was 81.1% over 8 years of age and 72.8% over 6 years of age. 41.8% of our population had abnormal BMD ( z scores < −1), and the rate of very low BMD ( z scores < −2) was 17.4%. The frequency of abnormal BMD was higher in children with BMI< 50th percentile, SKS ≤ 70, low FEV ₁ z score, respiratory microorganism colonization, ≥ 2 annual pulmonary exacerbations, required respiratory support, low albumin, and high CRP levels. Systemic inflammation marker CRP increase was the most predictable parameter for low BMD. Conclusion: This study informs clinical practice by highlighting the need for multidisciplinary interventions, such as earlier evaluation of DXA scans due to the risk factors and poor clinical conditions, a consistent follow‐up protocol, individualized nutrition programs with the dietitian, and enhanced physical therapy.

Purpose: Alström syndrome is a rare autosomal recessive monogenic ciliopathy, which is caused by a mutation of the Alström syndrome 1 gene. It is a multisystemic disorder characterized by insulin resistance, childhood obesity, cardiomyopathy, progressive hepatic and renal failure, sensorineural hearing loss, and retinal degeneration. In this study, we aimed to report a novel variant in Alström syndrome 1 gene causing Alström syndrome in a patient presenting with visual impairment. Methods: This was a case report. Results: A 10-year-old male patient presented with photophobia and visual impairment in both eyes. Anterior and posterior segment examinations were unremarkable bilaterally. Optical coherence tomography showed attenuated ellipsoid zone. Electroretinography revealed diminished cone and rod responses consistent with cone–rod dystrophy. Genetic testing demonstrated a novel homozygous variant in Alström syndrome 1 (NM_015120.4) gene. The patient also was found to have early stage dilated cardiomyopathy through systemic evaluation after the diagnosis of Alström syndrome. Conclusion: Cone–rod dystrophy in pediatric population is relatively rare condition that can be associated with syndromic ciliopathies. The authors presented a case of Alström syndrome with a novel variant in Alström syndrome 1 gene based on ophthalmic findings. Ophthalmologists play an important role in the diagnosis of this syndrome and early detection of systemic manifestations.