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Background: Child disability has significant implications on their well-being and healthcare systems., Aim: This survey aimed to assess the magnitude of seven types of disability among Egyptian children aged 1 < 6 years and their socio-demographic, epidemiological, and perinatal predictors., Methods: A national population-based cross-sectional household survey targeting 21,316 children from eight governorates was conducted. The screening questionnaire was derived from the WHO ten-question survey tool validated for identifying seven disability categories., Results: The percentage of children with at least one disability was 8.1% as follows: speech/communication (4.4%), Mobility/physical (2.5%), Seizures (2.2%), Comprehension (1.7%), Intellectual impairment (1.4%), Visual (0.3%) and Hearing (0.2%). Age was not found to affect the odds of disability except for visual disability (significantly increased with age (AOR = 1.4, 95% CI:1.1-1.7). Male sex also increased the odds of all disabilities except visual, hearing, and seizures. Convulsions after birth significantly increased the odds of disability as follows: hearing (AOR = 8.1, 95% CI: 2.2-30.5), intellectual impairment (AOR = 4.2, 95% CI: 2.5-6.9), and mobility/physical (AOR = 3.4, 95% CI: 2.3-5.0). Preterm delivery and being kept in an incubator for more than two days after birth increased the odds for visual disability (AOR = 3.7, 95% CI: 1.1-12.1 & AOR = 3.7, 95% CI: 1.7-7.9 respectively). Cyanosis increased the odds of seizures (AOR = 4.7, 95% CI: 2.2-10.3). Low birth weight also increased the odds for all disability domains except for visual and hearing. Maternal health problems during pregnancy increased the odds for all types of disability except hearing and seizures. Higher paternal education decreased the odds for all disabilities by at least 30% except for vision and hearing., Conclusion: The study found a high prevalence of disability among Egyptian children aged 1-6 years. It identified a number of modifiable risk factors for disability. The practice of early screening for disability is encouraged to provide early interventions when needed., (© 2023. BioMed Central Ltd., part of Springer Nature.)

Aim: This study aimed to determine the prevalence of disability domains among Egyptian children in the age group of 6-12 years as well as assess their socio-demographic, epidemiological, and perinatal predictors., Methods: A national population-based cross-sectional household survey targeting 20,324 children from eight governorates was conducted. The screening questionnaire was derived from the WHO ten-question survey tool validated for the identification of disabilities., Results: The prevalence of children with at least one type of disability was 9.2%. Learning/ comprehension was the most prevalent type (4.2%), followed by speech/communication (3.7%), physical/ mobility and seizures (2.2% for each), intellectual impairment (1.5%), visual (0.7%), and hearing (0.4%). The commonest predictors for disabilities were children who suffered from convulsions or cyanosis after birth and maternal history of any health problem during pregnancy. However, preterm and low birth weight (LBW) babies or being admitted to incubators for more than two days were strong predictors for all disabilities except hearing disability. A history of jaundice after birth significantly carried nearly twice the odds for seizures (AOR = 2.2, 95% CI:1.5-3.4). History of difficult labor was a predictor of intellectual impairment (AOR = 1.5, 95% CI:1.1-2.0). A disabled mother was a strong predictor for all disabilities except seizures, while a disabled father was a predictor for visual and learning/ comprehension disabilities (AOR = 3.9, 95% CI:2.2-7.1 & AOR = 1.6, 95% CI:1.1-2.4 respectively). Meanwhile, both higher maternal and paternal education decreased significantly the odds to have, physical/ mobility and Learning/ comprehension by at least 30%., Conclusion: The study found a high prevalence of disability among Egyptian children aged 6-12 years. It spotted many modifiable determinants of disability domains. The practice of early screening for disability is encouraged to provide early interventions., (© 2023. BioMed Central Ltd., part of Springer Nature.)

Bleeding phenotype is reported in β-thalassemia patients. However, the underlying etiology remains elusive. We aimed to assess coagulation profile and the platelet aggregation in β-thalassemia children with bleeding diathesis. Fifty β-thalassemia children with a positive bleeding history were recruited. Bleeding phenotype was explored through full history taking and thorough clinical examination. Complete blood count, prothrombin time, international normalized ratio, and platelets aggregometry were performed for children with negative workup. Mucosal bleeding was manifest among most of our patients (96%). Two-third of patients had decreased aggregation with ristocetin (68%), adenine di-phosphate (64%), and arachidonic acid (64%). While half of the patients (48%) had deficient response to epinephrine. Collagen, ristocetin, and arachidonic acid induced aggregation were negatively correlated to frequency of blood transfusion (P=0.021, r=-0.325; P<0.001, r=-0.465; P=0.018, r=-0.333, respectively). Aggregation to collagen and epinephrine demonstrated a negative correlation with age (P=0.04, r=-0.287; P=0.03, r=-0.315). Deferiprone was associated with a deficient response to ristocetin and collagen when compared with deferasirox or no chelation (P=0.021 and 0.006, respectively). Impaired ristocetin response was linked to hydroxyurea (P=0.035). Platelets function defect should be considered in β-thalassemia patients with bleeding symptoms., Competing Interests: The authors declare no conflict of interest., (Copyright © 2022 Wolters Kluwer Health, Inc. All rights reserved.)

Epilepsy is a serious childhood disease associated with cognitive impairment. Our aim was to investigate the possible association of serum folic acid, vitamin B12, and intelligence scores in epileptic children. A group of 30 children with established diagnosis of idiopathic epilepsy for at least one year as well as another group of 30 nonepileptic healthy children as the control group were recruited for analysis. Cognitive performance was assessed by a battery of psychological tests that covers verbal and nonverbal intelligence. Serum B12 level was significantly lower in patients than the control group (264.17 ± 58.07, 450.55 ± 134.9, respectively). No significant difference was detected between patients and the control group regarding serum folic acid level. Verbal, performance, and total IQ were significantly lower in patients than the control group (83.2 ± 3.08 vs. 95.8 ± 6.22, 78.4 ± 10.68 vs. 91.3 ± 2.45, and 180.6 ± 6.58 vs. 93.5 ± 3.02, respectively). However, no significant correlation was detected in folic acid, vitamin B 12, and cognitive scores. Epileptic children were five times more at risk of having low IQ (verbal, performance, and total) < 85 than the control group (OR = 4.754, 95% CI 13.047-1031.316, p  = .000). In conclusion, children with epilepsy might be at higher risk for cognitive dysfunction than normal children. No significant association was detected between cognitive performance and either folic acid or vitamin B12 in epileptic children receiving sodium valproate. Supplementation of those vitamins should be restricted to those with documented deficiency.

Background: The global pandemic of COVID-19 has led to unprecedented psychological stress on frontline health professionals. The objective of our study was to determine the prevalence of perceived stress and its association with having children among physicians in Al Madinah city, Saudi Arabia, during the COVID-19 pandemic., Methods: A web-based analytical cross-sectional study was conducted among the physicians working in Al-Madina, Saudi Arabia. Physicians living in Madina city were invited to participate in this survey by using an online questionnaire that consisted of socio-demographic information, and Perceived Stress Scale (PSS) 10 items questionnaire. The outcome measure was perceived stress score and levels among participants, while exposure factors were having children and the number of children of each participant., Results: Low, moderate and high levels of perceived stress were found in 39.3%, 56.4% and 4.3% of the physicians, respectively. 53.6% of those physicians who have children, had significant moderate perceived stress as compared to 93.3% of those who do not have any children (odds ratio (OR) = 0.08, 95% confidence interval (CI) 0.01-0.65, p=0.004). There was an inversed significant correlation between the number of children a participant had and the perceived stress scale score (R = -0.21, p=0.026)., Conclusion: COVID-19 pandemic resulted in perceived stress among physicians in Saudi Arabia, mostly moderate level. Having children was found to be a protective factor.

Background: Informed consent (IC) is a healthcare standard emphasizing the meaning of human dignity as clarified in the Universal Declaration of Human Rights. Data about IC practices in Egypt is insufficient. This study aimed to assess the Egyptian patients'/guardians' experiences about IC and their expectations about its practices' purposes in general and according to the type of the healthcare facility., Methods: Self-administered questionnaire was carried out for 1092 participants who had undergone or were scheduled to a procedure requiring an IC at three studied types for Egyptian health care facilities. Ten statements were ranked twice by the participants to reflect their perception of IC purpose as per what is currently practiced and what they believe should be practiced., Results: IC implementation varies significantly (p<0.05) across the health care facilities in Egypt. The percentage of its implementation at the non-governmental facilities, governmental facilities, and university hospital was 85.9%, 77.8%, and 63.8 respectively. The first three ranked purposes of the current IC practices were: "Helping patient/guardian decide (64.9%)", "Documenting patient's/guardian's decision (59.3%)", and "Having shared decision (57.3%)". The perceived purposes of IC to be practiced were: "Informing the patient/guardian (68.4%)", "Making sure patient/guardian understand (65.3%)" and "Documenting patients/guardians decisions (65.1%)". "Being a meaningless routine" was reported by the majority to be ranked as a low purpose for IC current and preferred practices., Conclusion: The practice of IC is common within the Egyptian medical community. Participants believe that information disclosure "Making sure patients understand" has to help in IC decision making and its main purpose. However, unfortunately, this is not perceived as a current purpose of IC. There was consensus agreement that documenting the patient's/guardian's decision and informing the patient/guardian are perceived as both important current and preferred purposes for IC practices., Competing Interests: The authors have declared that no competing interests exist.

Aim of the Work: This study aimed at assessing the dominance of risk practices associated with HCV endemicity in Egypt and detecting the behavioral development level concerning different aspects of HCV risk behaviors with respect to age and gender. The survey highlights the most cost-effective strategies that could accelerate HCV elimination in Egypt., Subjects and Methods: A national household survey targeted 3780 individuals (age range: 10-85 years). The sample was a systematic probability proportionate to size from 6 governorates representing the six major subdivisions of Egypt. The indicators used for assessing the behavioral development level towards HCV included six domains: awareness (7 indicators), perceived risk (5 indicators), motivation with the intention to change (4 and 5 indicators for males and females respectively), trial, rejection or adoption (6 and 5 indicators for males and females respectively)., Results: The study revealed that along the continuum of behavior development, the percentage of the participants who acquired half of the scores was as follows: 73.1% aware, 69.8% developed perceived risk, 80.6% motivated with only 28.9% adopting the recommended behaviors, 32% rejected them, 2.3% were in the trial stage versus 35.8% who did not try any. Adolescents had significantly lower levels of development for almost all domains when compared to adults. Statistical higher significance was detected in favor of adults, employees, married, Lower Egypt governorates, and university-educated participants (p<0.001) regarding awareness, perceived risk, and motivation scores. More than half of the participants incorrectly believed that contaminated food, sharing food utilities, contaminated water, mosquitoes, and schistosomiasis would lead to HCV transmission., Conclusion: Egypt would be closer to HCV elimination when cost-effective strategies are directed not towards creating awareness, perceived risk or motivation to change- (at an acceptable level)- but towards motivating adopting risk-reduction behaviors for HCV, tackling misconceptions and reinforcement of social support., Competing Interests: All authors report no conflict of interest. “No financial or non-financial benefits have been received or will be received from any party related directly or indirectly to the subject of this manuscript.

Background: COVID-19 (Corona Virus Disease 2019) is showing a wide global spread, and urgent joint international efforts is required to the control of this pandemic, the awareness of people towards infectious viruses still the main factor to limit the widespread of disease. The aim of this study is to assess the level of awareness and attitude towards COVID-19 among a sample of Egyptian school children, using a web-based questionnaire., Results: A total of 708 participants were involved in this online survey study, representing different areas in Egypt, 378 males (53.4%), 330 females (46.5%); their age range between 6 and 18 years. Regarding the residence, 576 (81.4%) were from urban areas, the remaining 132 (18.6%) were from non-urban areas. Internet and media were more frequent used as a source of information in urban students. The knowledge level score of risk and prevention of the disease were significantly higher in urban students than in non-urban students. Healthy practice score ≥ 50 were significantly more frequent in urban students. Healthy practice score was non-significantly higher in urban students., Conclusions: Most of the study participants of school students are knowledgeable about basic information, and have cautious preventive practices towards COVID-19, denoting the efficacy of the public health efforts. However, the lower level of awareness in non-urbans, indicating a need to address alternative channels to communicate with these populations., Competing Interests: Competing interestsThe authors declare that they have no competing interests" in this section., (© The Author(s) 2021.)

Background: School feeding program (SFP) increases access to education and to better health. This study aims to evaluate the effects of SFP on physical growth, cognitive development, psychosocial behavior, and learning achievement of school children., Methods: A quasi-experimental study was conducted. The intervention group included 903 pupils in the fifth grade receiving the school meal, while the control group included 886 pupils, matched for age and sex, without meal. The meal consisted of a pie made of flour fortified with vitamins A, B6, B12, C, thiamin, riboflavin, niacin, folate, calcium, iron, zinc, and phosphorus. Socioeconomic position, nutritional status, and dietary behavior were evaluated. Neuropsychological tests were done. Psychosocial behavior was rated and educational achievement was recorded. Post hoc and independent sample t tests were used to detect the association of the studied parameters with the intake of school snack., Results: Children who took the meal had better scores on visual memory, auditory vigilance tests (9.71 ± 2.80 vs. 7.45 ± 3.25; 25.02 ± 3.36 vs. 10.82 ± 8.92, respectively, P < 0.001), the afternoon attention and working memory test (8.20 ± 2.21vs. 7.75 ± 3.05) (P < 0.001), but less score of externalizing behavior (P < 0.001) than the control group. No significant changes of children's nutritional status were detected between the two groups. School meal was the main predictor of visual memory and auditory vigilance (P < 0.001), and was the strongest predictor of academic achievements when combined with family size and meals' frequency (P < 0.001)., Conclusion: School meal improves academic achievements of school children.

Background: Provision of emergency obstetric care is considered the key for maternal mortality reduction worldwide. This study evaluated the impact of community- and facility-based educational programs on provision of emergency obstetric care in Egypt. The study focused on evaluating utilization of the available health services and care seeking behaviors of mothers in the childbearing period., Methods: We implemented a package of community- and facility-focused educational interventions in two of Egypt's lowest income governorates. At facility level, health professionals at rural health units from 21 villages over 5 years were trained. Mass media gathering, individual teaching at health facilities, printed materials and home-based care sessions were provided. Collectively, these interventions were designed to focusing on recognition of the early warning signs during pregnancy, delivery and postpartum period for timely referral to hospitals for 20,494 women and adolescents mothers., Results: The impact of the interventions was highly reflected on the percent of mothers received care during their pregnancy period. Proper antenatal care at governmental or private health facilities was raised dramatically from 0.6 to 59.3% and those who utilized at least one family planning method from 61.4 to 74.4%. Accordingly, the rate of complications significantly reduced during pregnancy (38.1 to 15.1%), during delivery (24.1 to 13.1%) and during postpartum (81.7 to 7.0%). As an impact to the improvement, there was a marked reduction in adolescent pregnancy by 55% and better birth outcome with a reduction in the percent of stillbirth by 11.5%., Conclusion: It is important to provide a comprehensive package that works at both improving qualities of care as well as empowering women by knowledge to first aid measures at the community level. The cost-effective way to empower mothers to provide first aid measures as emergency obstetric care is to adopt the outreach approach which could be more influential than mass media campaigns for the at-risk and vulnerable and low-income communities.

The study aimed to investigate the feasibility of incorporation of metoclopramide hydrochloride (MCP HCl) in mucoadhesive thermoreversible liquid suppository (MCP HCl -LS ) to bypass the first-pass metabolism and maximize its efficacy to be a promising substitute for parenteral therapy. MCP HCl -LS was formulated using Pluronic (PF127/PF68) and hydroxypropylmethylcellulose (HPMC) and in vitro evaluated through their gelation temperature, gel strength (GS), mucoadhesive force, and in vitro release studies. Also, the MCP HCl -LS was evaluated for its rheological behavior and examined for rectal mucosal integrity after administration. The results revealed that the MCP HCl -LS ; composed of PF127/PF68/HPMC (20/7/0.5% w/w) was in the liquid state at room temperature, experienced gelation at 30.23 °C, with suitable GS of 28.66 s and rectal retention force of 43.03 × 10 2  dyne/cm 2 . The pharmacokinetic data showed that the MCP HCl -LS exhibited a significant increase ( p  < 0.05) in AUC 0-480 (219.688 vs 172.333 ng.h.mL -1 of the marketed) and 1.3-fold increase in relative bioavailability compared to Primperan ® suppository, indicating that LS formula bypassed the first-pass metabolism. Moreover, MCP HCl -LS did not cause any morphological harm to the rectal tissues suggested that the developed formulation was safe and could be a potentially useful alternative drug carrier for rectal delivery of MCP HCl in patients experiencing chemotherapy-induced nausea and vomiting.

Background: Functional constipation (FC) is a common health problem in paediatrics that causes significant physical and emotional distress to patients and their families., Aim: In the current work, we assessed the presence of behavioural problems in children with functional constipation and their pattern and relation to various demographic and disease-associated factors., Methods: A cross-sectional case-control study was conducted, including 55 consecutive children aged 4-16 years diagnosed with functional constipation and 55 healthy age and sex-matched controls. Psychological assessment was done using the Pediatric Symptom Checklist - 17 (PSC-17)., Results: Twenty-six (47.3%) patients with FC had positive total PSC-17 scores while none of the controls had positive scores (p-value < 0.001). Positive internalising and externalising behaviours scores and attention problems were found in 36 (65.5%), 15 (27.3%) and 12 (21.8%) of the patients respectively in contrary to controls where only 6 (10.9%) had positive scores in internalising behaviour, and non-showed externalising behaviour and 4 (7.3%) were inattentive. Older age, longer duration of illness, residency in rural areas and presence of encopresis were found to have a significant association with the presence of such problems., Conclusion: Children with FC have more behavioural disorders compared to healthy controls. Integration of psychosocial aspects and their management is recommended during dealing with patients with FC., (Copyright: © 2019 Marwa M. El-Sonbaty, Ahmed Fathy, Amal Aljohani, Abeer Fathy.)

Background: Klotho G-395-A gene polymorphism may impact children with end-stage renal disease (ESRD). We investigated the relevance of Klotho G-395-A on ESRD development and progression, and its relationship with evolution of cardiovascular complications in pediatric dialysis patients., Methods: Fifty-five children with chronic kidney disease (CKD) and seventy healthy children were genotyped for Klotho G-395A., Results: Incidence of GA/AA genotypes and A allele were higher in ESRD patients compared with controls (54.5 vs. 7.1%, P < 0.001; 30.9 vs. 13.6%, P = 0.001, respectively). Also, children with GA/AA genotypes were 15.6 times more likely to develop ESRD than with GG genotype (95% CI 5.4-44.7, P < 0.001). A allele carriers have 2.8 times higher risk of developing ESRD than those with G allele (95% CI 1.5-5.35, P = 0.001). Also, the A allele could be considered a predictor of cardiovascular disease (CVD), as carriers have 161 times higher risk of cardiovascular complications than non-carriers (95% CI 21-1233, P < 0.001). All ESRD patients with CVD presented with left ventricular hypertrophy (LVH) and the frequency of A allele was significantly higher among ESRD children with LVH, whereas G allele frequency was significantly higher among ESRD children without LVH., Conclusions: The A allele of the G-395A Klotho gene polymorphism shows a significantly higher frequency among children with CKD and those with CVD and LVH. This mutant allele could be used as a risk marker for the development of ESRD as well as a predictor of CVD in these children.

Background/aims: Celiac disease (CD)-associated cardiologic disorders is a growing concern. However, data regarding cardiac affection in children with CD are few. This study aimed at assessing the subclinical impact of CD on the global myocardial performance in Saudi children with CD using Doppler tissue imaging (DTI)., Patients and Methods: Conventional two-dimensional echocardiography was performed among 20 Saudi children with CDas well as 20 age and sex-matched healthy controls. DTI were used to determine right ventricular (RV) and left ventricular (LV) Tei indexes. These findings were correlated with the Modified Marsh Classification of the histologic findings in CD., Results: LV and RV Tei indexes were significantly higher in children with CD than the control group (mean ± standard deviation: 0.47 ± 0.05 vs. 0.31 ± 0.18; P< 0.0005 and 0.51 ± 0.04 vs. 0.32 ± 0.05; P< 0.0001, respectively). RV Tei index was found to be positively correlated with the Modified Marsh Classification of CD (r = 0.7753, P< 0.0001). LV Tei index tended to be more affected in patients with more severe histologic findings, however, such relation did not reach statistical significance (r = 0.2479, P = 0.292). Fractional shortening did not correlate with the Modified Marsh Classification of histologic findings in CD patients (r= -0.11, P = 0.641)., Conclusions: Subclinical myocardial dysfunction of both ventricles occurs in children with CD. The DTI method appears to be more sensitive than conventional two-dimensional echocardiography in the early detection of myocardial dysfunction in children with CD., Competing Interests: There are no conflicts of interest.

Introduction: Serum procalcitonin (PCT) levels are known to be low in healthy individuals in healthy subjects but are increased in patients with a severe bacterial infection. It has not been extensively studied in children with chronic kidney disease (CKD), treated either with hemodialysis (HD) or with renal transplantation., Material and Methods: During a 6-month period, blood samples were taken from 102 (55 HD children and 47 renal transplant recipients) children with a strong clinical suspicion of infection. Procalcitonin levels were measured by ELISA., Results: Thirty-four/102 cases had proven infections as defined previously. Children with proven infections had a significantly higher PCT (0.920 ±0.24 ng/ml) than those without (0.456 ±0.53 ng/ml), p = 0.04. The ideal cutoff value derived for serum PCT was 0.5 ng/ml. This threshold value established a sensitivity of 94.1% and a specificity of 87.9%., Conclusions: This study indicates that significantly increased PCT concentration is a promising predictor of systemic bacterial infection in children with CKD, with good sensitivity and specificity. This study proposes that serum PCT is a convenient index of infection in CKD children at a cutoff value of 0.5 ng/ml., Competing Interests: The authors declare no conflict of interest.

Vascular access represents a lifeline for children undergoing hemodialysis. A failure of vascular access among patients receiving regular hemodialysis is associated with increased morbidity, mortality and costs. We assessed the possibility of using soluble adhesion molecules as reliable predictors of vascular access failure in children on hemodialysis. Moreover, we evaluated whether there is an association among the different studied adhesion molecules in hemodialysis patients with thrombosed and non-thrombosed arteriovenous fistula fistulas (AVFs). This study included 55 hemodialysis children, 36 with good access and 19 with access failure, and 20 healthy volunteers. Forty-four patients had native AVFs and 11 patients had tunneled permanent catheter (11with thrombosed and 33 with non-thrombosed AVFs). Serum-soluble vascular cell adhesion molecule-1 (sVCAM-1), soluble intercellular adhesion molecule-1 (sICAM-1), soluble E-selectin (sE-selectin) and soluble P-selectin (sP-selectin) were measured using ELISA technique. A significant increase was found in the levels of sVCAM-1, sICAM-1, sE-selectin and sP-selectin versus controls and all hemodialysis patients, hemodialysis patients with good access and hemodialysis patients with access failure (P=0.001 for sVCAM-1 and sICAM-1 and P=0.0001 for sE-selectin and sP-selectin). A significant increase was found in the levels of sVCAM-1, sE-selectin and sP-selectin in both chronic hemodialysis patients with thrombosed and non-thrombosed native AVFs versus controls (P=0.0001 for all parameters). There was significant difference between both chronic hemodialysis patients with thrombosed and non-thrombosed native AVFs as regard to sVCAM-1 (54.64±30.82 versus 25.69±27.96ng/ml, P=0.04). Both sICAM-1 and sP-selectin were positively correlated with the erythropoietin (EPO) dose in hemodialysis children (r=0.31, P=0.04 and r=0.32, P=0.04, respectively). A significant positive association was found between E-selectin and sP-selectin in hemodialysis patients with thrombosed AVFs (r=0.83, P=0.04). There was a significant correlation between sVCAM-1 and EPO dose in thrombosed AVF group (r=0.84, P=0.01). The assessment of serum sVCAM-1 might be useful for the identification of the chronic hemodialysis patients at an increased risk for native AVFs thrombosis. The role of EPO in vascular access failure should be taken into consideration. The clinical relevance of these observations warrants further investigations.

Objective: the present study was conducted to investigate the oxidant-antioxidant status in Egyptian children with sickle cell anemia., Methods: the serum levels of total antioxidant capacity (TAO), paraoxonase (PON), vitamin E, nitrite, and malondialdehyde (MDA) were measured in 40 steady state children with homozygous sickle cell anemia (24 males and 16 females) and 20 apparently healthy age- and gender-matched controls., Results: mean serum TAO, PON, vitamin E, and nitrite levels were significantly lower in the group with sickle cell anemia, whereas mean serum MDA was significantly higher in these children compared to controls. No significant differences in mean levels of TAO, PON, nitrite, vitamin E, and MDA were found in sickle cell anemia patients receiving hydroxyurea when compared with those not receiving hydroxyurea. A significant negative correlation between serum nitrite and the occurrence of vaso-occlusive crises (VOC) was observed (r=-0.3, p=0.04). PON level was found to be positively correlated with patients' weight and BMI (r=-0.4, p=0.01; r=-0.7, p<0.001, respectively), but not with frequency of VOC. The area under the curve of serum nitrite in predicting occurrence of VOC was 0.782, versus 0.701 for PON, and 0.650 for TAO (p=0.006). Serum MDA was not correlated with nitrite, PON, TAO, or vitamin E levels. No significant correlations were detected between serum nitrite and hemoglobin or antioxidant enzymes., Conclusion: children with sickle cell anemia have chronic oxidative stress that may result in increased VOC, and decreased serum nitrite may be associated with increases in VOC frequency. A novel finding in this study is the decrease in PON level in these patients, which is an interesting subject for further research., (Copyright © 2014 Sociedade Brasileira de Pediatria. Published by Elsevier Editora Ltda. All rights reserved.)

Background: Thyroid dysfunction is a known complication of transfusion-dependent β-thalassemia. However, information on its frequency and risk factors among Egyptian Children is still unclear., Objective: We aimed to determine the frequency of functional thyroid abnormalities among young patients with β-thalassemia and compare the thyroid function status among patients with β-thalassemia major (TM) and β-thalassemia intermedia (TI)., Materials and Methods: This was a cross-sectional study that included 52 β-thalassemia children [27 boys and 25 girls; 34 (65.4%) with TM and 18 (34.6%) with TI]. Their mean age was 16.0±1.91 (range: 12-18) years. Thyroid function and iron load status were assessed by measurement of free tetraiodothyronine, free triiodothyronine, thyroid stimulating hormone (TSH), and serum ferritin concentrations., Results: Serum TSH of the studied cases ranged from 0.28 to 25 μIU/ml with a mean of 4.5±4.8 μIU/ml. None of the studied cases had overt primary hypothyroidism and the frequency of subclinical hypothyroidism was 19.2%. No risk factors for thyroid dysfunction could be identified among our cases. The thyroid profile was comparable in TM and TI patients (P>0.05) and the frequency of subclinical hypothyroidism among TM cases was 20.6% and it was comparable to the 16.7% found among TI patients (P>0.05). No correlations were found between TSH, serum ferritin, chelation therapy, and frequency of blood transfusion., Conclusion and Recommendations: Both TM and TI patients are at risk for subclinical thyroid failure regardless of their iron overload status. Early evaluation of thyroid function in β-thalassemia children and thyroid replacement therapy for subclinical hypothyroidism should be introduced in the treatment protocols.

Background: The prevalence of cardiovascular disease (CVD) and inflammation is high in patients with chronic kidney disease (CKD). Adiponectin (ADPN) is an adipocytokine that may have significant anti-inflammatory and anti-atherosclerotic effects. Low adiponectin levels have previously been found in patients with high risk for CVD., Methods: On seventy eight advanced CKD (stages 4 and 5) pediatric patients undergoing maintenance hemodialysis( MHD) or conservative treatment (CT) the following parameters were studied: body mass index, left ventricular mass index(LVMI), serum adiponectin , cholesterol, HDL-cholesterol, high sensitivity C-reactive protein (hs CRP),interleukin 6(IL6) and single-nucleotide polymorphisms (SNPs) in the ADIPOQ gene at positions 45, and 276. Seventy age-and gender-matched healthy subjects served as control subjects., Results: Markedly (P = 0.01) elevated plasma adiponectin levels were observed in CKD patients, especially CT patients, compared to control subjects. The wild type of ADIPOQ 45T > G (T) allele is the main gene for patients and controls. MHD and CT patients had significantly higher frequency of the TT genotypes of +276G > T gene (P = 0.04) compared with control subjects. A significant positive correlation was observed between plasma adiponectin and IL6 level, whereas negative correlations were found between adiponectin level, cholesterol, HDL cholesterol and hs CRP. In a stepwise backward multiple regression model only IL6 (P = 0.001) was independently associated with plasma adiponectin levels. The adiponectin gene the 276 GT+TT genotypes were associated with a higher level of adiponectin ., Conclusions: The present study demonstrated that ADPN is related to several metabolic and inflammatory CV risk factors in a manner consistent with the hypothesis that this protein might have a protective role against these factors. We observed an association between the +276G>T SNP in the adiponectin gene and CKD in children. Genetic variation of +276 gene seemed to have a positive impact on circulating adiponectin levels in CKD patients.