Efficacy and Safety of Single-Dose Pregabalin in Preoperative Pediatric Sedation.

Introduction: This study aimed to investigate the anxiolytic and sedative effects of a single oral dose of 5 mg/kg pregabalin in pediatric patients undergoing elective surgery. It also assessed potential adverse effects and its impact on bispectral index (BIS) responses. Materials and Methods: This prospective randomized clinical trial enrolled 60 pediatric patients undergoing minor elective surgery. Patients were randomly assigned to receive either oral pregabalin (5 mg/kg) or a placebo one hour before induction of anesthesia. Anxiety levels were assessed using the Visual Analog Scale for Anxiety (VAS-A), and sedation levels were evaluated using the Ramsay Sedation Scale (RSS). Results: Pregabalin premedication significantly reduced preoperative anxiety, as indicated by lower VAS-A scores compared to the control group. Sedation levels, measured using the RSS, were significantly higher in the pregabalin group at various time points post-dose. During intubation, skin incision, and recovery, BIS responses were significantly lower in the pregabalin group. Conclusion: The use of single-dose pregabalin preoperatively in children recorded a significant decrease in anxiety and achieved a state of sedation without an increase in adverse effects.

Gluten-Free, Casein-Free Diet for Children with Autism Spectrum Disorder: A Case-Controlled Study.

Background and Objectives: Numerous therapeutic and dietary interventions have been examined in the last thirty years for pediatric patients diagnosed with autism spectrum disorder (ASD). Our interventional study aimed to assess the effectiveness of the gluten-free, casein-free (GFCF) diet in a cohort of Egyptian children with ASD. Materials and Methods: The present clinical trial was conducted as a prospective 12-month, open-label, case-controlled interventional study. Thirty-six ASD children who were newly diagnosed and had not taken any prior psychiatric or rehabilitation therapy were included in this study. The patients were randomly assigned into two groups: group A, which received the GFCF diet, and group B, which served as the control group and was not restricted to food containing gluten and casein for 12 months. All patients were followed up for 1 year. Results: Following the implementation of the GFCF diet in group A, significant improvements in CARS scores were observed compared to group B after 6-month and 1-year follow-up periods. Conclusions: The introduction of the GFCF diet could be helpful and promising for autistic children. Conclusive evidence regarding the effectiveness of the GFCF diet remains a subject of controversy. Nonetheless, our study contributes some evidence supporting its potential benefits for children with ASD. It is recommended that future research on the GFCF diet employ a more sophisticated research design, incorporating a consistent baseline measure that can effectively assess the therapeutic effects of these interventions for individuals with ASD.

Could the Crosstalk Between Myeloid-Derived-Suppressor Cells and Regulatory T Cells Have a Role in Beta-Thalassemia?

Background: Secondary iron overload, alloimmunization, and increased risk of infection are common complications in patients with transfusion-dependent thalassemia (TDT). Regulatory T cells (Tregs) and myeloid-derived suppressor cells (MDSCs) play an essential role in preventing excessive immune response. This research aimed to study the interaction between Tregs and MDSCs in TDT patients and to evaluate the association of these cell types with disease severity. Methods: This case-control study included 26 patients with TDT and 23 healthy, age- and sex-matched controls. All patients were investigated for complete blood count (CBC), serum ferritin, and flow cytometric analysis of peripheral blood to detect Tregs, MDSCs, and MDSC subsets. Results: A significant increase was observed in the frequencies of Tregs and MDSCs, particularly monocytic MDSCs (MO-MDSCs), in TDT patients compared with controls. The frequencies of these cells showed a direct association with ferritin level and total leukocyte count and an inverse association with hemoglobin level. Furthermore, a positive correlation was observed between Tregs and each of the total MDSCs and MO-MDSCs. Conclusions: Levels of Tregs and MDSCs increased in TDT and may probably have a role in suppressing the active immune systems of TDT patients.

Clinicoradiologic Correlation in 22 Egyptian Children With Megalencephalic Leukoencephalopathy With Subcortical Cysts.

Megalencephalic leukoencephalopathy with subcortical cysts (MLC) is a rare genetic form of cerebral white matter disease whose clinicoradiologic correlation has not been completely understood. In this study, we investigated the association between clinical and brain magnetic resonance imaging (MRI) features in 22 Egyptian children (median age 7 years) with MLC. Gross motor function was assessed using the Gross Motor Function Classification System, and evaluation of brain MRI followed a consistent scoring system. Each parameter of extensive cerebral white matter T2 hyperintensity, moderate-to-severe wide ventricle/enlarged subarachnoid space, and greater than 2 temporal subcortical cysts was significantly associated (P < .05) with worse Gross Motor Function Classification System score, language abnormality, and ataxia. Having >2 parietal subcortical cysts was significantly related to a worse Gross Motor Function Classification System score (P = .04). The current study indicates that patients with MLC manifest signification association between certain brain MRI abnormalities and neurologic features, but this should be confirmed in larger studies.

Dendritic cells and monocyte subsets in children with Gaucher disease.

BACKGROUND: There are minimal data on the frequencies of monocyte subsets and dendritic cells (DCs) in children with Gaucher disease (GD), as nearly all previous studies have involved adult patients. Consequently, we aimed to describe the changes in these cell subpopulations in children with GD type 1 who were on regular enzyme replacement therapy (ERT). METHODS: This case-control study included 25 children with GD1 and 20 healthy controls. All participants underwent investigations such as complete blood count and flow cytometric assessment of DC and monocyte frequencies and phenotype. RESULTS: We found that GD1 children had significantly reduced percentages of both types of DCs, i.e., plasmacytoid DCs and myeloid DCs, compared to the control group. There was also a significant reduction in absolute monocyte numbers and percentage of classical monocyte. Moreover, the GD1 children had higher frequencies of non-classical and intermediate monocytes than the control group. CONCLUSIONS: Our results so far indicate that, when compared to the control group, the GD1 children had significantly reduced total and classical monocyte, with significantly decreased frequencies for both types of DCs. These changes can contribute to immunological abnormalities in pediatric patients with GD1. IMPACT: Children with Gaucher disease type 1 (GD1) have significantly reduced total and classical monocyte frequencies, with decreasing percentages for both types of dendritic cells. GD1 children had significantly reduced frequencies of myeloid and plasmacytoid dendritic cells as compared to the controls. The GD1 children also had significant changes in monocyte subsets when compared to the controls. Our results show that monocytes and dendritic cells' significant changes could contribute to immunological abnormalities in pediatric patients with GD1.

Down-regulation of Regulatory T-cells in Children With Gaucher Disease Under Enzyme Replacement Therapy.

Gaucher disease (GD) is one of the most important lysosomal storage disorders. T-lymphocytes perform and regulate many of the immune processes and play a major role in immune homeostasis. Studies have shown that GD causes impairment in T-lymphocyte functions, although the role and status of T-lymphocytes in GD are still under investigation. It is still not fully known how GD leads to the altered biochemical and immunological cellular functions observed in the disease. Our study aimed to evaluate the variations of regulatory T-lymphocytes (Tregs) in 20 Egyptian children with GD under enzyme replacement therapy, managed in Assiut University Hospitals. Tregs were detected using 3-color flow cytometric immunophenotyping, in which subpopulations of T-lymphocytes and the expression of CD4+ on their surfaces were gated. The expression of CD25+ was assessed on CD4+ cells with different gates to define CD4+CD25, CD4+CD25+high, and CD4+CD25+ low cells. Then, CD4+CD25+highFoxp3+cells and MFI of Foxp3+ expression on CD4+CD25+ high were determined. We found the levels of CD4+CD25+/CD4+, CD4+CD25+high/CD4+, CD4+CD25+highFoxp3+ Tregs, and median fluorescence intensity of Foxp3+ expression on CD4+CD25+high were significantly lower in children with GD compared to healthy controls. In conclusion, our data showed significantly decreased regulatory T-lymphocytes in children with GD. The reduced effect of Tregs may have a role in the pathogenesis of immune dysregulation in children with GD. The relationship of these cells to immune disorders in GD children remains to be determined. Therefore, we recommend further studies to elucidate the role and function of Tregs in GD and its potential role in the disease phenotype, as well as how it is affected by electrical resistivity tomography.