Pediatrics International最新文献

Background: Walking is a common intervention for treating obesity in adults, but data on the effectiveness of walking for childhood obesity are limited. We therefore investigated the effectiveness of walking in the treatment of childhood obesity and the factors that make its effect stronger.

Methods: Participants who visited our clinic for obesity were instructed to walk at least 10,000 steps on holidays and given lifestyle guidance at the first visit. CV risk factors and blood chemistry were examined at every visit. The number of steps walked on holidays between each visit was also assessed. We defined successful treatment as a final decrease in relative body weight (RBW) of ≥8.6% in this study. The predictors of final RBW reduction and factors of dropout were examined with a focus on the number of holiday steps.

Results: The final number of participants was 131 (74 boys and 57 girls; mean age 10.1 ± 2.4 years). The mean reduction in RBW was 14.7 ± 12.8% (p < 0.001). Predictors of final RBW reduction were the level of RBW reduction from the first to the second visits (p = 0.01) and the mean number of steps on holiday between the second and the third visits (p = 0.04). Fewer steps on holiday between the first and the second visits were a predictor of dropout (p = 0.03).

Conclusions: This study confirmed the effectiveness of lifestyle modification, particularly walking. Furthermore, the establishment of walking habits and reduction in RBW early in the treatment were found to be important.

Background: Non-obese survivors of childhood hematopoietic stem cell transplantation (HSCT) often exhibit severe insulin resistance and mild hepatic dysfunction. Although the exact pathophysiology remains unclear, one possible explanation is the increasingly recognized condition known as HSCT-associated partial lipodystrophy. In lipodystrophy, hepatic dysfunction is characterized by fibrosis and severe steatosis, resembling nonalcoholic steatohepatitis. Accordingly, detailed hepatic assessment would improve our overall understanding of the condition. This study aimed to evaluate hepatic dysfunction using a non-invasive method (FibroScan) in patients who developed glucose intolerance post-HSCT.

Methods: Fourteen non-obese childhood HSCT survivors from two institutions underwent FibroScan. They were classified according to the presence or absence of diabetes mellitus (DM) with insulin resistance (HOMA-IR >2.5 or insulin therapy >1.5 U/kg/day). FibroScan provided controlled attenuation parameter (CAP) for hepatic steatosis and liver stiffness measurements (LSM) for fibrosis. Serum hepatic fibrosis markers (collagen type IV and hyaluronic acid) were also measured.

Results: Seven patients developed insulin-resistant DM while seven had normal glucose/lipid metabolism. All DM patients showed "Dunnigan"-type subcutaneous fat distribution. CAP values were higher in the DM group [307 (261.0-343.5) vs. 237 (216.5-271.0), p = 0.041], as were LSM scores [6.7 (5.9-9.9) vs. 4.1 (3.5-4.7), p = 0.007], indicating more severe steatosis and increased liver stiffness. However, serum hepatic fibrosis markers did not differ significantly.

Conslusions: Non-obese HSCT survivors with insulin-resistant DM exhibited severe hepatic fat accumulation and progression of liver fibrosis attributable to lipodystrophy. FibroScan may have potential utility for monitoring liver health in this population.

Background: Due to the amazing developments in modulatory treatments, genetic analysis of cystic fibrosis (CF) patients has become even more important. More than 2000 disease-causing variants of the cystic fibrosis transmembrane conductance regulator (CFTR) gene have been found, and their ethnic and geographical distributions vary. We aimed to present the first genetic data from the Southeastern Anatolia region of Turkey and evaluate patients' clinical and genetic characteristics and identify modulatory drugs covering a wider range of mutations by detecting and reporting new mutations.

Methods: Our study included 337 CF patients from three CF reference centers in the Southeastern Anatolia region.

Results: Ninety-one distinct mutations and four significant deletions were identified by analyzing CFTR mutations. The most prevalent mutation in our research was F508del (8.92%); the second most prevalent mutation was 2183AA->G, and the third most prevalent mutation was R347P. Additionally, a novel mutation (V1160X) was identified in two siblings. Only 33.5% of our patients qualified for CFTR modulator medication therapy.

Conclusion: This study elucidates the diverse nature of CFTR mutations in the Turkish population. The heterogenous genetic pool of the Southeastern Anatolia region is more similar to Mesopotamia than to other regions of our country, and receives immigration from the East. Detection and reporting of novel mutations and CFTR mutations that occur at very low frequencies from different populations living in various geographical areas are essential for identifying modulatory medicines that cover a broader range of mutations and also help genetic diagnosis of CF in newborn screening.

Background: Early stages of chronic kidney disease (CKD) are usually asymptomatic, but kidney damage that starts in childhood may persist until adulthood and can lead to serious morbidity and mortality in the following years. Therefore, preventive practices that can slow the progression of kidney damage are strongly needed. In this study, we evaluated the relationship of urinary chloride concentrations and fractional excretion of chloride (FeCl) with proteinuria and estimated glomerular filtration rate (eGFR).

Methods: This study is a retrospective descriptive study. Patients were divided into subgroups based on eGFR values and proteinuria status. It was investigated whether there was a difference between the groups in terms of urine chloride and FeCI levels.

Results: A total of 176 patients with a mean age of 11.5 ± 5.0 (2-18) years were included in the study. The most common primary diagnosis was CAKUT (n = 56, 31.8%), followed by glomerular diseases (n = 45, 25.5%). The number of patients with eGFR <90 mL/min/1.73 m² was 20 (11.4%). Urinary chloride levels were significantly lower in patients with proteinuria compared to those without (95.5 ± 74.8 mEq/L vs. 146.7 ± 85.7 mEq/L, p < 0.001). Regression analysis showed that higher chloride and FeCI levels were associated with less proteinuria. Urinary chloride was lower in stage 3 CKD compared to stage 2 (34.20 ± 20.2 mEq/L vs. 70.9 ± 60.5 mEq/L, p = 0.087), while FeCI was higher in patients with decreased eGFR (1.73 ± 0.91%, p < 0.001).

Conclusion: Strategies towards increasing urinary chloride excretion may help to slow the progression of CKD by providing a renoprotective effect via tubuloglomerular feedback (TGF), which inhibits the increase in glomerular pressure.

Background: The increasing frequency and scale of flood disasters pose significant challenges for evacuating children with medical complexity. This study aimed to clarify the challenges and necessary support for safely evacuating these children in Japan during floods.

Methods: A qualitative interview-based study was conducted with 10 caregivers of children with medical complexity. Interview data were analyzed using inductive thematic analysis following Braun and Clarke's six-phase approach.

Results: Four main themes related to challenges were identified: (1) Shortage of support personnel, (2) Inadequate response from the administration, (3) Specificity of medical needs, and (4) Limitations in coordination and communication. Four themes related to support needs were also identified: (1) Assistance, (2) Support by the administration, (3) Medical care, and (4) Information. These findings highlight the multifaceted nature of evacuation challenges for children with medical complexity, emphasizing the need for tailored support systems that address both social and medical aspects of disaster preparedness.

Conclusion: This study highlights the complex challenges in evacuating children with medical complexity during floods in Japan, addressing the increasing concerns raised by more frequent and severe flood disasters. These findings emphasize the urgent need for comprehensive community-based disaster preparedness involving various stakeholders, improved coordination, and tailored support systems for vulnerable children.

Background: Epilepsy often begins in childhood or adolescence, a period marked by significant hormonal changes. Antiepileptic drugs (AEDs) like valproic acid (VPA) and levetiracetam (LEV) are commonly used, but VPA is associated with notable endocrine side effects, particularly in adolescent girls. This raises concerns about drug choice during puberty.

Objective: To compare the effects of VPA and LEV monotherapy on pubertal hormone profiles, menstrual characteristics, physical development, and gynecologic ultrasound findings in adolescent girls with primary epilepsy.

Methods: This prospective, cross-sectional study included 61 adolescent girls (16 on VPA, 15 on LEV, 30 controls) at a tertiary hospital from January 2018 to January 2019. Participants underwent anthropometric measurements, hormonal evaluations (FSH, LH, estradiol, testosterone, SHBG, TSH, 17-OH progesterone, DHEASO₄), menstrual assessments, and pelvic ultrasound. Statistical analysis was performed with a significance level of p < 0.05.

Results: VPA users had significantly higher TSH levels than LEV users and controls (p = 0.036), suggesting possible subclinical thyroid dysfunction. They also had lower 17-OH progesterone and DHEASO₄ levels (p = 0.008 and p = 0.022, respectively). No significant differences were observed between groups in weight, height, BMI, pubertal hormone levels, menstrual cycle characteristics, or ultrasound findings. Treatment duration did not significantly affect hormone levels.

Conclusion: VPA appears to negatively impact thyroid and adrenal hormones in adolescent girls, whereas LEV has a safer endocrine profile. Given the hormonal sensitivity during puberty, LEV may be a safer alternative for adolescent girls with epilepsy to reduce potential endocrine disturbances.