Examining the association between autism spectrum disorder and atopic eczema: meta-analysis of current evidence.

OBJECTIVES: This study aims to investigate the association between autism spectrum disorder (ASD) and atopic eczema (AE), shedding light on potential associations and underlying mechanisms. METHODS: A comprehensive review of literature was conducted to identify relevant studies published up to August 2023. Various electronic databases, including PubMed, Embase, Scopus, Web of Science, and Cochrane, were searched using specific keywords related to ASD and AE. RESULTS: The meta-analysis covered a total of 30 studies. The first analysis included 23 studies with a combined total of 147430 eczema patients in the ASD group and 8895446 eczema patients in non-ASD group. We calculated the risk ratio of eczema in ASD and non-ASD groups, which revealed a significantly higher risk of eczema in patients with ASD (RR 1.34; 95% CI 1.03, 1.76). The second analysis included seven studies with a combined total of 3570449 ASD patients in the AE group and 3253973 in the non-Eczema group. The risk ratio of ASD in the Eczema and Non-Eczema groups showed an insignificantly increased risk of ASD in patients with eczema (RR 1.67; 95% CI 0.91, 3.06). CONCLUSION: This study underscores the possible link between ASD and atopic eczema, shedding light on their potential association. IMPACT: Our study conducted a meta-analysis on the association between autism spectrum disorder (ASD) and atopic eczema (AE), shedding light on potential associations and underlying mechanisms. The review we conducted covered a total of 30 studies. This study underscores the possible link between ASD and atopic eczema, shedding light on their potential association.

Survival characteristics of Wilms Tumor, a reference developed from a longitudinal cohort study.

BACKGROUND: Wilms tumor (WT) survival has been affected by the evolution in clinical and biological prognostic factors. Significant differences in survival rates indicate the need for further efforts to reduce these disparities. This study aims to evaluate the clinicopathological data impact on survival among patients after Wilm's diagnosis. METHODS: The study utilized the SEERStat Database to identify Wilms tumor patients, applying SEERStat software version 8.3.9.2 for data extraction. Selection criteria involved specific codes based on the International Classification of Diseases for Oncology (ICDO-3), excluding cases with unknown SEER stage, incomplete survival data, unknown size, or lymph node status. Statistical analyses, including Kaplan-Meier estimates and Cox regression models, were conducted using R software version 3.5. Standardized mortality ratios (SMR) were computed with SEER*Stat software, and relative and conditional survival analyses were performed to evaluate long-term survival outcomes. RESULTS: Of 2273 patients diagnosed with Wilms tumor, (1219 patients, 53.6% were females with an average age group of 3-8 years (50.2%). The overall mean survival after five years of diagnosis was 93.6% (2.6-94.7), and the overall mean survival rate was 92.5% (91.3-93.8) after ten years of diagnosis. Renal cancers were identified as the leading cause of death (77.3%), followed by nonrenal cancers (11%) and noncancer causes (11%). Additionally, robust relative survival rates of 98.10%, 92.80%, and 91.3% at one, five, and ten years, respectively, were observed, with corresponding five-year conditional survival rates indicating an increasing likelihood of survival with each additional year post-diagnosis. Univariate Cox regression identified significant prognostic factors: superior CSS for patients below 3 years (cHR 0.48) and poorer CSS for those older than 15 years (cHR 2.72), distant spread (cHR 10.24), regional spread (cHR 3.09), and unknown stage (cHR 4.97). In the multivariate model, age was not a significant predictor, but distant spread (aHR 9.22), regional spread (aHR 2.84), and unknown stage (aHR 4.98) were associated with worse CSS compared to localized tumors. CONCLUSION: This study delving into WT survival dynamics reveals a multifaceted landscape influenced by clinicopathological variables. This comprehensive understanding emphasizes the imperative for ongoing research and personalized interventions to refine survival rates and address nuanced challenges across age, stage, and tumor spread in WT patients.

Detection and characterization of autoreactive memory stem T-cells in children with acute immune thrombocytopenia.

Primary immune thrombocytopenia (ITP) is an acquired autoimmune disorder characterized by an isolated decrease in platelets below 100 × 109/l after the exclusion of other conditions associated with thrombocytopenia. We investigated the role of different memory T-cell subsets, including T stem cell memory (TSCM), in children diagnosed with primary ITP and its association with therapeutic duration. This case-control study included 39 pediatric patients with acute ITP admitted to the Children's Hospital at Assiut University. Using a FACSCanto flow cytometer, CD8 + and CD4 + T-lymphocytes were gated. Five different subsets were characterized in each of these cells according to CD45RO and CD45RA expression. Afterward, gating was performed based on CCR7, CD95, and CD27. Examination of the CD8 + T cells subpopulation showed that Central memory T (TCM) and CD8+ Naïve T (TN) cells were significantly lower in ITP patients than in healthy children (p < 0.0001) and (p = 0.01), respectively. In addition, CD8 + TEMRA was significantly higher in ITP children than in controls (p = 0.001). CD4 + TCM cells were significantly lower in the ITP patient group (p = 0.04). However, CD4 + TEM was significantly higher in patients than controls (p = 0.04). Our research found that ITP patients had an imbalance in the ratio of CD4+ to CD8+ T cells in the peripheral blood and that TCM cells may be involved in the pathogenetic mechanism of ITP. TCMs could help in prediction of patients with higher risk of developing ITP.

Mapping the Grounds for Mortalities in Acute Myeloid Leukemia Through Registry Analyses: A Retrospective Cohort Study of Children, Adolescents, and Young Adults Patients.

Background: Our objective was to identify non-malignant factors that contribute to mortality in children, adolescents and young adults, aiming to improve patient follow-up and reduce mortality rates to achieve better survival outcomes. Methods: We analyzed 8,239 acute myeloid leukemia (AML) cases diagnosed between 2000 and 2019 in the USA. Using version 8.4.0.1 of the Surveillance, Epidemiology, and End Results (SEER)*Stat software, we calculated the standardized mortality ratios (SMRs) and 95% confidence intervals (CIs) for each cause of death. Results: Out of the 3,165 deaths observed in the study population, the majority (2,245;70.9%) were attributed to AML itself, followed by non-AML cancers (573; 18.1%) and non-cancerous causes (347; 10.9%). Conclusions: Patients with AML are at a higher risk of developing other types of cancer and granulocyte deficiencies, which increases the risk of death from non-cancerous causes such as infections. Moreover, treatment for AML carries the risk of cardiac problems. AML is commoner in males than females.

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.

Does ozaki procedure have a future as a new surgical approach for aortic valve replacement? a systematic review and meta-analysis.

Background: In 2014, Ozaki et al. introduced the neo-cuspidation (Ozaki procedure), a new valve from the pericardium, to reduce or even prevent the risk of chronic autoimmune inflammation and subsequent rejection or valve degeneration. Thus, the authors aimed to assess the safety and efficacy of the Ozaki technique in treating aortic valve diseases. Materials and methods: A comprehensive search was performed via PubMed, the Cochrane Library, Scopus, and the Web of Science up to 20 February 2022. Random-effects meta-analysis models were employed to estimate the pooled mean and SD or event to the total of the Ozaki procedure. Relevant records were retrieved and analyzed by OpenMeta analyst software. Results: A total of 2863 patients from 21 studies were finally included in our analysis. Ac. Ozaki technique showed statistical significance in terms of mean cardiopulmonary bypass time of 148 mins (95% CI 144-152.2, P<0.001), mean aortic cross-clamp time of 112.46 mins (95% CI 105.116, 119.823, P<0.001), reoperation with a low risk of 0.011 (95% CI 0.005, 0.016, P=0.047), conversion to aortic valve replacement with a low risk of 0.004 (95% CI -0.001, 0.008, P=0.392), finally ICU stay (days) and hospital length of stay (days) with a mean of 2.061 days (95% CI 1.535, 2.587, P<0.001) and 8.159 days (95% CI 7.183-9.855, P<0.001), respectively. Conclusion: The Ozaki procedure provides a safe surgical technique with low mean cardiopulmonary bypass time and aortic cross-clamp time; moreover, a mean of 2-day-postoperative hospital stay was observed with the Ozaki procedure with a low risk of conversion to aortic valve replacement, reoperation, ICU and hospital stay, and death.

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.

Sildenafil for congenital heart diseases induced pulmonary hypertension, a meta-analysis of randomized controlled trials.

BACKGROUND: Sildenafil was first prescribed for angina pectoris and then for erectile dysfunction from its effects on vascular smooth muscle relaxation and vasodilatation. Recently, sildenafil has been proposed for congenital heart diseases induced pulmonary hypertension, which constitutes a huge burden on children's health and can attribute to fatal complications due to presence of unoxygenated blood in the systemic circulation. Therefore, our meta-analysis aims to further investigate the safety and efficacy of sildenafil on children population. METHODS: We searched the following electronic databases: PubMed, Cochrane CENTRAL, WOS, Embase, and Scopus from inception to April 20th, 2022. Randomized controlled trials that assess the efficacy of using sildenafil in comparison to a placebo or any other vasodilator drug were eligible for inclusion. The inverse variance method was used to pool study effect estimates using the random effect model. Effect sizes are provided in the form of mean difference (MD) with 95% confidence intervals (CI). RESULTS: Our study included 14 studies with (n = 849 children) with a mean age of 7.9 months old. Sildenafil showed a statistically significant decrease over placebo in mean and systolic pulmonary artery pressure (PAP) with MD -7.42 (95%CI [-13.13, -1.71], P = 0.01) and -8.02 (95%CI [-11.16, -4.88], P < 0.0001), respectively. Sildenafil revealed a decrease in mean aortic pressure and pulmonary artery/aortic pressure ratio over placebo with MD -0.34 (95%CI [-2.42, 1.73], P = 0.75) and MD -0.10 (95%CI [-0.11, -0.09], P < 0.00001), respectively. Regarding post corrective operations parameters, sildenafil had a statistically significant lower mechanical ventilation time, intensive care unit stay, and hospital stay over placebo with MD -19.43 (95%CI [-31.04, -7.81], s = 0.001), MD -34.85 (95%CI [-50.84, -18.87], P < 0.00001), and MD -41.87 (95%CI [-79.41, -4.33], P = 0.03), respectively. Nevertheless, no difference in mortality rates between sildenafil and placebo with OR 0.25 (95%CI 0.05, 1.30], P = 0.10) or tadalafil with OR 1 (95%CI 0.06, 17.12], P = 1). CONCLUSION: Sildenafil is a well-tolerated treatment in congenital heart diseases induced pulmonary hypertension, as it has proven its efficacy not only in lowering both PAP mean and systolic but also in reducing the ventilation time, intensive care unit and hospital stay with no difference observed regarding mortality rates.

Safety and efficacy of tezepelumab vs. placebo in adult patients with severe uncontrolled asthma: a systematic review and meta-analysis.

Patients with severe uncontrolled asthma still experience acute asthma symptoms and exacerbations, particularly those with non-eosinophilic inflammation who take the maximum amount of standard drug therapy. Tezepelumab, a human monoclonal antibody, can improve lung function and enhance control of asthma symptoms in those patients, regardless of the disease's baseline characteristics. This study aims to investigate the safety and efficacy of using tezepelumab in controlling severe symptoms of uncontrolled asthma. We performed a comprehensive literature search in several databases, including PubMed, Scopus, Web of Science, Cochrane Library, and clinicaltrial.gov, using a well-established search strategy to include all relevant publications. According to our inclusion criteria, we searched for randomized controlled trials comparing tezepelumab versus placebo in patients with severe, uncontrolled asthma. We analyzed the data using The Revman 5.4 program software. The search identified 589 potential articles. After excluding studies inconsistent with selection criteria, four studies were included and analyzed qualitatively and quantitatively. The pooled effect demonstrated the better performance of tezepelumab over the placebo regarding the decrease in annualized asthma exacerbation rate (MD = - 0.74, (95% CI [- 1.04, - 0.44], p < 0.00001)), asthma control questionnaire-6 (ACQ-6) Score MD = - 0.32, (95% CI [- 0.43, - 0.21], p < 0.00001)), blood eosinophil count (MD = - 139.38 cells/mcL, (95% CI [- 150.37, - 128.39], p < 0.00001)), feNO (MD = - 10 ppb, (95% CI [- 15.81, - 4.18], p = 0.0008)) and serum total IgE (MD = - 123.51 UI/ml, (95% CI [- 206.52, - 40.50], p = 0.004)). All tezepelumab groups had higher pre-bronchodilator forced expiratory volume in 1 s than the placebo group (MD = 0.16, (95% CI [0.10, 0.21], p < 0.00001)). Higher efficacy and safety profile was detected for tezepelumab to control the exacerbations of severe uncontrolled adult asthmatics.

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.

Prevalence and associated risk factors of recurrent otitis media with effusion in children in Upper Egypt.

OBJECTIVE: We conducted this study to determine the associations of possible risk factors and prevalence of recurrent otitis media with effusion (OME) in a cohort of children in Upper Egypt. METHODS: This was a cross-sectional study undertaken in two tertiary referral centers in Upper Egypt. Associations of possible risk factors with prevalence of recurrent OME were studied. Multi-factor logistic regression analysis was done to recognize the statistically significant risk factors associated with recurrent OME. RESULTS: We collected the data of 2003 pediatric patients, of which 1016 were males (50.7%). A total number of 310 children have OME, including 159 males (51.3%). The prevalence rate of OME in our cohort was 15.5%. Multi-factor logistic regression analysis of the risk factors related to recurrent OME showed it was strongly associated with adenoid hypertrophy (P < 0.0001), tonsil hypertrophy (P < 0.0001), sinusitis (P < 0.0001), posterior nostril polyps (P = 0.009), allergic rhinitis (P < 0.0001), recurrent URTIs (P = 0.029) and gastroesophageal reflux (P = 0.031). CONCLUSIONS: Our study showed that recurrent OME in children in Upper Egypt is a common multifactorial problem, especially in young age. In our locality, allergic rhinitis, recurrent upper respiratory tract infections, gastroesophageal reflux, adenoid and tonsil hypertrophy were the most important associated factors related to the etiopathogenesis of OME.

Clinical profile and outcome of acute organophosphate poisoning in children of Upper Egypt: a cross-sectional study.

BACKGROUND: Organophosphates are one of the most common agents of poisoning in developing countries including Egypt. Due to lack of data about characteristics of organophosphates poisoning in our localities, we aimed to evaluate its clinical pattern and factors affecting outcome. METHODS: It was a cross-sectional study conducted in South valley University hospital between January 2019 and December 2019. It included all children ≤16 years of age presented with organophosphates poisoning. Diagnosis was performed from the history taken from the patient's relatives and presenting symptoms. Demographic data, mode and route of poisoning, time from exposure to presentation, clinical symptomatology, grading and routine investigations were evaluated in addition to treatment taken and outcome. RESULTS: During the study period, 108 children; mean age was 7.95 ± 4.11 years (range 1. 5-16 years) presented with organophosphorous poisoning. Sixty five (60%) cases were females and 43 (40%) were males. Unintentional acts (87%) were detected more than suicidal (13%) and inhalation route (63.8%) more than ingestion (36. 2%). Miosis was the most frequent clinical sign (100%) followed by respiratory distress (77.7%). Regarding time of presentation to emergency department, 43 (40%) cases were presented within 6 h while others presented more than 6 h post-exposure. Mechanical ventilation (MV) was needed for 14 (13%) cases and 6 (5.5%) cases died due to respiratory failure. Duration of hospital stay, mean time interval from toxic exposure to hospital presentation, leucocytosis, need for MV and cumulative dose of pralidoxime were significantly higher in non survivors than survivors while Pao2 (partial arterial oxygen) and GCS (Glasgow coma scale) were significantly lower. CONCLUSION: This study concluded that time consumed till presentation to hospital, low GCS, need for MV, leucocytosis, decreased PaO2 and increased cumulative dose of pralidoxime were independent risk factors of mortality.

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.

Polymorphism of interleukin-1ß and interleukin-1 receptor antagonist genes in children with autism spectrum disorders.

In this study, we first investigated interleukin-1 beta (IL-1ß) and IL-1 receptor antagonist (IL-1RA) levels in a cohort of Egyptian children with autism spectrum disorder (ASD) and in healthy controls. Second, we examined the single-nucleotide polymorphisms (SNPs) at positions -31 and - 511 of the IL-1ß gene promoter and IL1RA and assessed the association between IL1B and IL1RA polymorphisms with ASD. We examined IL1ß promoter polymorphism at -511 (IL-1ß-511) and - 31 (IL-1ß-31) and IL1RA gene polymorphism in 80 children with ASD and 60 healthy children. The children with ASD had significantly higher levels of IL-1ß and IL-1RA than the controls. The children with ASD also had significantly higher frequencies of homozygous (CC) and heterozygous (TC) genotype variants of IL-1ß-511, and IL-1RA than the controls. Moreover, the frequency of the IL-1ß-511 allele (C) was higher in the ASD group than in the controls (p = .001). The homozygous and heterozygous variants of IL-1RA allele II were also significantly higher in the ASD group than in the control group. There was no significant association between the IL-1ß-31 genotype and autism classes. However, there were significant differences in the distribution of the IL-1RA heterogeneous genotype and allele II among children with severe autism. The inflammatory role of cytokines has been implicated in a variety of neuropsychiatric pathologies, including autism. Our data show alterations in the IL-1ß system, with abnormally increased serum levels of IL-1ß and IL-1RA in the children with ASD. Further, polymorphisms in the IL-1ß-511 and IL-1RA genotype variants correlated positively with autism severity and behavioral abnormalities. IL-1ß-511 and IL-1RA gene polymorphisms could impact ASD risk and may be used as potential biomarkers of ASD. Variations in the IL-1ß and IL-1RA systems may have a role in the pathophysiology of ASD.

Vitamin D Status in Egyptian Children With Allergic Rhinitis.

Objective: This study aimed to assess the serum levels of vitamin D in an Egyptian cohort of children with allergic rhinitis (AR) and to evaluate any correlation of vitamin D status with the disease severity. Patient and methods: One hundred twenty children with AR and 100 healthy children were included in our study. We studied the serum levels of vitamin D 25(OH)D and 1,25(OH)2D in all participants. The associations between vitamin D levels and clinical characteristics of AR were examined. Results: In AR group, the serum levels of calcium, (25(OH)D and 1,25(OH)2D levels were significantly lower (p < .0001, p < .001, and p < .0001, respectively) in AR children than in controls. Furthermore, the mean 25-OHD3 levels in patients with moderate/severe AR were significantly lower than those with mild AR (p < .001). We found significant negative correlations between mean 25(OH)D levels and total nasal symptom score (r = -.62, p = .002) and total immunoglobulin E levels (r = -.27, p = .013) in AR group. Conclusions: Vitamin D deficiency is a frequent finding among Egyptian children with AR when compared to the healthy group. A significant inverse association was observed between vitamin D levels and AR disease severity.

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.

Iron deficiency and cyanotic breath-holding spells: The effectiveness of iron therapy.

Aim: Frequent cyanotic breath holding spells cause fear and severe anxiety to parents. This study aimed to evaluate clinical, laboratory and treatment characteristics of children with cyanotic breath holding spells. Methods: Included were 180 children (mean age: 1.82 ± 0.53 years) with cyanotic breath holding spells. They were divided into three groups: with iron deficiency, with iron deficiency anemia and without iron deficiency. Blood hemoglobin (HB), ferritin and iron concentrations were measured at baseline and after 3 and 6 months of iron treatment. Results: The mean spell frequency was 24.57 ± 7.31/months, 83% had spells after the age of 1 year, 37% had daily spells, 16% had family history of spells, and 61% had Iron deficiency/Iron deficiency anemia (p = .001). No significant difference in the frequency of spells between children with iron deficiency and those with Iron deficiency anemia. Compared to patients without iron deficiency, there was significant reduction of spells frequency, increased hemoglobin, ferritin and iron levels after 3 and 6 months of iron therapy (p = .0001). Negative correlations were observed between spell frequency with hemoglobin (p = .001), ferritin (p = .0001) and iron (p = .001) levels. Conclusion: Not only Iron deficiency anemia but also iron deficiency alone without anemia is associated with a risk of high-frequency cyanotic breath holding spells. Iron therapy results in reduction in spells' frequency which was correlated with increasing ferritin and iron levels.

Glomerular Glucocorticoid Receptors Expression and Clinicopathological Types of Childhood Nephrotic Syndrome.

Glucocorticoids are primary therapy of idiopathic nephrotic syndrome (INS). However, not all children respond to steroid therapy. We assessed glomerular glucocorticoid receptor expression in fifty-one children with INS and its relation to response to steroid therapy and to histopathological type. Clinical, laboratory and glomerular expression of glucocorticoid receptors were compared between groups with different steroid response. Glomerular glucocorticoid expression was slightly higher in controls than in minimal change early responders, which in turn was significantly higher than in minimal change late responders. There was significantly lower glomerular glucocorticoid receptor expression in steroid-resistance compared to early responders, late responders and controls. Glomerular glucocorticoid expression was significantly higher in all minimal change disease (MCD) compared to focal segmental glomerulosclerosis. In INS, response to glucocorticoid is dependent on glomerular expression of receptors and peripheral expression. Evaluation of glomerular glucocorticoid receptor expression at time of diagnosis of NS can predict response to steroid therapy.