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BACKGROUND: Iron overload is the main cause of oxidative stress in beta-thalassemia (βT) by the increased production of free radicals and reactive oxygen species. Antioxidants counteract the toxic effects of oxidative stress. AIM: This study aims to evaluate the total antioxidant capacity (TAC) and the possible impact of splenectomy, iron chelators, and hydroxyurea (hydra) on serum level of TAC. MATERIALS AND METHODS: Fifty children and adolescents with βT were studied in comparison to 25 healthy age- and sex-matched subjects. Complete medical history, clinical examination, and laboratory assessment of serum TAC, ferritin, hepcidin, and hemoglobin (Hb) were carried out. RESULTS: There was no statistically significant difference between the three groups; thalassemia major (TM), thalassemia intermedia (TI), and controls as regard age and sex. β-TM patients had significantly higher serum ferritin, serum hepcidin, and serum TAC (p < 0.000, 0.002, and 0.000, respectively) than controls. β-TI patients had significantly higher serum ferritin and serum TAC (p < 0.000) than controls. Serum TAC was lower in children with splenectomy, but this difference was not statistically significant. In addition, we observed no statistically significant difference in serum TAC of patients under different (deferasirox or deferiprone) medication. Serum TAC concentration was significantly higher in patients taking hydroxyurea (hydra) (p < 0.010). CONCLUSIONS: The study showed an increased level of serum TAC in patients with β-T in comparison with controls. Serum TAC was also increased in those taking hydroxyurea, however, it was low in βT patients under regular chelation therapy, while splenectomy had no significant effect on serum TAC.

BACKGROUND: Hepcidin, a small peptide hormone, is established as the main regulator of iron homeostasis. AIM: To estimate serum hepcidin, ferritin, and hepcidin: ferritin ratio in β-thalassemia patients and to determine the effect of splenectomy and hydroxyurea on serum hepcidin. METHODS: A study was conducted on 30 thalassemia major (βTM), 29 thalassemia intermedia (βTI) and 29 healthy children's controls. Data were collected by patient interviewing where detailed history-taking and thorough clinical examinations were carried out. Serum ferritin and hepcidin were measured by ELISA assay (Bioneovan Co. Ltd Beijing, China). RESULTS: Βeta-thalassemia patients had higher serum ferritin, serum hepcidin and lower Hb and hepcidin: ferritin ratio compared to the controls (p < 0.001, 0.010, 0.001, 0.001) respectively. Β-TM patients had higher mean serum hepcidin and serum ferritin compared to β-TI, with statistically significant difference (P = 0.042, P < 0.001, respectively). Twenty-one patients out of 29 βTI was on hydroxyurea therapy; these patients had significantly lower levels of serum ferritin (P < 0.004) and significantly higher levels of Hb (P < 0.004). Serum ferritin was statistically significantly higher in splenectomized patients P < 0.009. Serum hepcidin level was insignificantly higher in splenectomized patients than non-splenectomized patients (21.6 ± 14.75, 17.76 ± 10.01 ng/mL). Hepcidin showed a significantly positive correlation with hepcidin: ferritin ratio in all studied groups. CONCLUSION: Serum hepcidin was elevated in β-thalassemia children with more evident elevation in βTM patients. Splenectomy played no major role in hepcidin regulation. Knowing that hepcidin in serum has a dynamic and multi-factorial regulation, individual evaluation of serum hepcidin and follow up, e.g. every 6 months could be valuable, and future therapeutic hepcidin agonists could be helpful in management of iron burden in such patient.

IntroductionFamilial Mediterranean fever (FMF) is an autosomal recessive disease. It is characterized by recurrent crises of fever and serosal inflammation. Although FMF patients are symptom free in between attacks, subclinical inflammation continues during the attack-free period. Such patients with inflammatory status have an increased risk of atherosclerotic cardiovascular complications. We attempted to elucidate the role of arterial wall thickening as a predictor of early atherosclerosis in children affected by FMF and to clarify the links between carotid intima media thickness and the markers of subclinical inflammation serum amyloid A (SAA), erythrocyte sedimentation rate (ESR), neutrophil-to-lymphocyte ratio (NLR) and platelet lymphocyte ratio (PLR).Material and methodsIt is a case control study. The study comprised 45 Egyptian children diagnosed with FMF and 45 healthy children of matched age and sex who served as controls, without family history or clinical manifestations suggestive of FMF. Laboratory investigations included complete blood count, NLR, PLR, ESR, C-reactive protein and lipid profile. Serum amyloid A levels were determined in both groups using enzyme linked immunosorbent assay. Assessment of the common carotid artery intima media thickness (CIMT) in the FMF patients was carried out.ResultsThe level of SAA was significantly higher in patients than the control subjects with a mean value of 38.30 ng/ml and 23.43 ng/ml respectively (p < 0.001). Our patients showed significantly higher PLR when compared to controls (p < 0.001). The mean right and left carotid intima media thickness in patient and control groups showed a highly significant difference (p = 0.005 and 0.036 respectively).ConclusionsThe mean carotid intima media thickness is higher in cases than the control group. Hence carotid intima media thickness may be used as a tool in the prediction of any atherosclerotic burden in those children.

BACKGROUND: Obese children and adolescents are more prone to have metabolic syndrome (MS).MS is a cluster of cardiovascular risk factors associated with insulin resistance. Body round index [BRI], visceral adiposity index [VAI] and a body shape index [ABSI] are among the new obesity anthropometric parameters. AIM: To evaluate the new markers for obesity in children and their possible association with other laboratory and clinical variables of MS. METHODS: Eighty nine obese children and 40 controls aged 10-18 years were recruited. Full history taking, thorough clinical examination, anthropometric and biochemical features were performed in the studied groups. Subcutaneous fat thickness (SFT) and visceral fat thickness (VFT) were estimated by ultrasonography. RESULTS: Obese children, exhibited significantly higher values in all anthropometric measurements (P < 0.001). Diastolic and systolic blood pressure were significantly higher (P < 0.001) in the obese group. ABSI, BRI and VAI have been found to be significantly higher in obese subjects (P < 0.001), with no significant gender difference. BMI, WHtR, WC/HR, SBP, DBP, subcutaneous fat thickness and visceral fat thickness, Liver Span, ABSI, BRI, VAI and HOMA_IR were significantly higher among children with MS than those without MS. Positive significant correlations of VAI with BMI, WC/Ht, WC/Hip, SBP, DBP, SFT, VFT, Liver size and HOMA-IR (r = 0.384, 0.239, 0.268, 0.329, 0.516, 0.320, 0.254, 0.251, and 0.278 respectively) are shown. The area under the ROC curve (AUC) of BMI, VAI, ABSI, BRI for predicting MS was 0.802 (0.701-0.902), 0.737 (0.33-0.841), 0.737 (0.620-0.855), 0.816 (0.698-0.934). CONCLUSION: We suggest using the VAI and WHtR indexes, as they are better predictor of MS.

BACKGROUND: Fetuin-A and ghrelin have been implicated in cardiovascular diseases and mortality among end stage renal disease patients. The exact mechanisms have not been fully elucidated. There is robust data supporting an association between ghrelin and various cardiovascular conditions, and some common processes such as inflammation, oxidative stress, and endoplasmic reticulum stress have been implicated.AIM: This study was conducted to assay serum fetuin-A and ghrelin in chronic renal failure pediatric patients and to study changes in their level that may occur after a single hemodialysis.MATERIAL AND METHODS: Forty nine pediatric patients suffering from ESRD on maintenance hemodialysis (HD), 20 patients with chronic renal failure (CRF) not on dialysis and 35 healthy subjects as control group were included. The mean age of the study population was 10.58 ± 3.94, 10.62 ± 3.24 and 10.61 ± 3.97 years respectively. Serum fetuin-A and plasma acyl ghrelin levels were measured by using ELISA method.RESULTS: The present study revealed that predialysis serum fetuin-A level was significantly increased in pediatric HD patients compared with the normal population, while ghrelin levels were significantly reduced. Furthermore, serum levels of fetuin-A decreased significantly after a single HD session.CONCLUSION: Our study concluded that fetuin-A and acyl ghrelin may play a role in inflammatory process among HD pediatric patients which may account for cardiovascular insults and mortality but their use as biochemical markers among ESRD pediatric patients have limitations due to wide fluctuations.

BACKGROUND: Visfatin is an intracellular enzyme, known as nicotinamide phosphoribosyltransferase (Nampt) and pre-B-cell colony-enhancing factor (PBEF-1). It has insulin-mimetic effects and lowers plasma glucose levels.AIM: The aim of the work was to assess serum concentration of Visfatin in type 1 diabetic children and adolescents and study its relationships with duration of diabetes, body mass index (BMI), glycemic control, insulin dosage, lipid profile and microvascular complications.MATERIAL AND METHODS: Fifty children and adolescents with type 1 diabetes mellitus were recruited with 30 ages and gender-matched healthy subjects. They were subjected to history taking; anthropometric measurements and chronic diabetic complications were recorded if present. Laboratory analysis included urinary microalbumin, serum triglycerides, HDL, LDL, cholesterol, fasting blood glucose, glycosylated Hb (HbA1c) and serum visfatin which was measured with enzyme-linked immunosorbent assay.RESULTS: Diabetic patients showed highly significant decrease in the level of visfatin compared to the control group (P = 0.0001).There was significant further decrease in visfatin level in diabetics with microalbuminuria (n = 13) compared to normoalbuminuric patients (n = 37) (P = 0.015). There was highly significant inverse correlation between visfatin level with age (r = -0.379, p = 0.007), BMI (r = -0.418, p = 0.003), waist circumference (r = -0.430, p = 0.002), hip circumference (r = -0.389, p = 0.005) and microalbuminuria (r = -0.323, p = 0.022).CONCLUSIONS: Type 1 diabetic children and adolescents had a significantly lower visfatin level compared to controls. A marked decrease in the level of visfatin was shown in patients with microalbuminuria with an inverse correlation with BMI suggesting an important role of visfatin in the pathogenesis of type 1 diabetics and type 1 diabetic nephropathy.

Aim: To study serum progranulin (PGRN) level in children with obesity and its relationship to inflamamatory markers and visceral fat. Methods: Fifty obese children and 50 controls aged 10-18 years were recruited. Demographic, anthropometric and biochemical features were collected according to a standard protocol. Serum progranulin levels, serum IL-6 and hsCRP were measured using ELISA. Insulin resistance was calculated by the homeostasis model (HOMA-IR) using the following formula: HOMA-IR = fasting insulin (mU/L) × fasting glucose (mmoL/L)/ 22.5. The maximum visceral fat thickness (VFT) and the minimum subcutaneous fat thickness (SFT) were measured by ultrasonography. Results: In the obese group, a significant increase was found in serum PGRN (48.87 ± 42.33 ng/mL) compared to control group (30.18 ± 23.82 ng/mL). Progranulin correlated significantly with VFT (r = 0.475), IL6 (r = 0.368), Insulin(r = 0.440) and HOMA-IR (r = 0.379). The mean serum progranulin in the high tertile VFT group was significantly higher than those in the low tertile and middle tertile groups (P = 0.030 and P = 0.039 respectively). VFT was highly positively correlated to progranulin, SFT, IL6, insulin, HOMA-IR and hsCRP (P = 0.001, 0.000, 0.001, 0.001, 0.003 and 0.003). However, the correlation coefficient between SFT and progranulin was insignificant. Summary: we demonstrated for the first time that serum PGRN concentrations increased in Egyptian obese children. The concentrations of serum PGRN correlated closely with visceral fat and IL6. Conclusion: PGRN may contribute to the pathogenesis of chronic inflammation in obesity. It could be a novel marker of visceral fat in obesity. Thus PGRN could be a potential therapeutic target for management of chronic inflammation in obesity.

The advantages of measuring hepatic oxidative status in liver biopsy are that it helps in diagnosis of hepatic dysfunction, reflects the degree of deterioration in the liver tissues, and helps to determine the severity of hepatic injury. We aimed to study the oxidative stress state in children with chronic hepatitis by using indirect approach in which antioxidant enzymes such as glutathione peroxidase (GPX), superoxide dismutase (SOD) and catalase (CAT) are determined in the liver tissue. The present study included 21 children and adolescents (12 males, 9 females) suffering from chronic hepatitis. Patients were selected from the Hepatology Clinic, New Children’s Hospital, Cairo University from November 2006 till 2009 and compared with a group of 7 children who happened to have incidental normal liver biopsy. Children with chronic hepatitis had mean age 8.12 ± 1.15 years. It was further subdivided into 2 subgroups: chronic viral heaptitis (n = 13) and cryptogenic hepatitis (n = 8). GPX, SOD and CAT levels were measured in fresh liver tissue (cell free homogenates) using ELISA. In chronic hepatitis group; there was a significant increase in the hepatic GPX activity (38.59 ± 35.82 nmol/min/ml) as compared to the control group (10.62 ± 6.68 nmol/min/ml). Also a significant correlation was observed between SOD and both ALT (r = 0.87, p < 0.05) and AST (r = 0.74, p < 0.05). GPX correlated with ALT (r = 0.80, p < 0.05) level in the chronic viral hepatitis subgroup. Our findings suggest that oxidative stress could play a role in the pathogenesis of chronic hepatitis. These preliminary results are encouraging to conduct more extensive clinical studies combining antioxidant therapy with various treatments.

Introduction: Fetuin-A is an important player in the enhancement of insulin resistance. There are very limited data available concerning the relationships between fetuin-A, weight status and features of the metabolic syndrome (Met S) in obese Egyptian subjects, and especially in children. The aim of the study was to evaluate fetuin-A serum level in subjects with obesity and its possible asso- ciation with other laboratory and clinical variables. Material and methods: A total of 140 obese subjects and 50 controls aged 10-40 years were recruited. Demographic, anthropometric and biochemical features were collected according to a standard protocol. Serum fetuin-A levels were measured using ELISA and the modified Third Report of the National Cholesterol Education Program (NCEP-ATP III) criteria were adopted to diagnose Met S. Results: A higher level of serum fetuin-A was detected in obese subjects. Met S cases were also significantly associated with higher serum fetuin-A. Fetuin-A correlated significantly with BMI (r = 0.437), systolic (r = 0.228) and diastolic blood pressure (r = 0.295), waist circumference (r = 0.332), insulin resist- ance calculated by the homeostasis model (HOMA-IR) (r = 0.295) and high-den- sity lipoprotein (HDL) (r = 0.362). Conclusions: Fetuin-A levels were higher in adults and children with obesity and Met S. They were related to insulin resistance and to features of the Met S in cross-sectional analyses. Our study demonstrates a novel association between human fetuin-A and the Met S among obese subject. Therefore, fetuin-A might be a new promising link between obesity and its comorbidities.

Introduction Dietary control of classic phenylketonuria (PKU) needs restriction of natural proteins; adequate protein intake is achieved by adding low phenylalanine (phe) formulae. The adequacy of this diet for normal bone mineralization had not been sufficiently evaluated. Our aim was to evaluate and follow up bone mineral density (BMD) in children and adolescents with PKU within a 2-year time interval to assess the adequacy of a phenylalanine restricted diet for bone mineralization and to search for a possible relationship between BMD, dietary control and blood phenylalanine (phe) concentrations. Material and methods Thirty-two patients with classic PKU (3-19 years) were evaluated for their bone mineral status using dual energy X-ray absorptiometry (DEXA) both at the beginning (baseline) and the end (follow-up) of the study. Results Low BMD was detected in 31.25% at the start and in 6.25% of patients after 2 years follows-up. No relationship was found between BMD and the duration of diet compliance and phe level as well. Conclusions In this study the low BMD detected in our patients was both at baseline and follow-up independent of diet restriction. A yearly DEXA would be highly beneficial for early detection and treatment, thus preventing osteoporosis and decreasing the risk of fractures. We also suggest the importance of searching for new emerging therapies such as enzyme substitution or gene therapy as low protein diet compliance was not enough to maintain normal bone mineral density.

Introduction Obesity and associated metabolic disorders are a worldwide epidemic. Recent evidence suggests that the microbial community in the human intestine may play an important role in the pathogenesis of obesity. The aim of this study was to assess the differences in the composition of the intestinal microbiota between obese and normal weight Egyptian children and adults. Material and methods The study included 79 subjects among whom 51 were obese (23 children and 28 adults), and 28 were subjects of normal weight (17 children and 11 adults). Faecal samples were collected from all subjects. Total DNA was extracted from collected stool samples and submitted to conventional PCR for detection of Firmicutes and Bacteroidetes. All the studied group was subjected to clinical and anthropometric evaluation. Laboratory assessment of fasting glucose, high-sensitivity C-reactive protein (hsCRP) and lipid profile was performed. Results The proportions of the phyla Firmicutes and Bacteroidetes were statistically significantly increased in the obese group compared to the normal weight group (p < 0.001, p = 0.003 respectively). The study also found a statistically significant positive trend for higher hsCRP in subjects with positive Firmicutes (p = 0.004). However, no associations were found between positive Bacteroidetes and hsCRP. Conclusions The results of this study indicate that obesity in Egyptian children and adults is associated with compositional changes in faecal microbiota with increase in the phyla Firmicutes and Bacteroidetes. This could be considered when developing strategies to control obesity and its associated diseases by modifying the gut microbiota.

INTRODUCTION: The International Society for Clinical Densitometry recommended that the lumbar spine and total body less head (TBLH) are the most accurate and reproducible skeletal sites for performing areal bone mineral density (BMD) measurements. Our objective is to evaluate the role of measurement of femoral neck BMD in avoiding the under-diagnosis of low BMD being a risk for fractures in subjects with chronic medical conditions that might affect bone health. MATERIAL AND METHODS: Subjects with chronic medical conditions that might affect bone health were studied (n = 468) and 36 healthy children were recruited as control subjects. Physical examinations, height, weight measurements and BMI were calculated. Dual-energy radiographic absorptiometry of the lumbar spine and femoral neck were measured. RESULTS: Bone mineral density z scores in both sites were significantly reduced in chronic patients, compared with control subjects. Prevalence of very low BMD z scores (-2 or more) using lumbar DXA, femoral DXA, and either of the sites were 1.38%, 3.37%, and 3.96%, respectively, while low BMD Z scores (-1 to less than -2) were 9.52%, 18.05% and 21.14% respectively. CONCLUSIONS: We identified a significant decrease in both lumbar and femoral BMDs in studied children. Sometimes femoral BMD is decreased while lumbar BMD is still within the normal range. For this reason we recommend that, when technically feasible and there is no facility to measure TBLH, all those patients should have lumbar spine and femoral neck bone mineral density measurements to avoid under-diagnosis of low BMD being a risk for fractures.

Systemic-onset juvenile idiopathic arthritis (SoJIA) is a chronic auto-inflammatory disease of childhood, with a complex genetic trait, which is characterized by arthritis associated with systemic manifestations. Familial Mediterranean fever (FMF) is another auto-inflammatory disorder that is monogenic. There are speculations as to whether Mediterranean fever (MEFV) mutations are among the genetic determinants of SoJIA. Our aim was to explore the frequency and clinical significance of MEFV mutations in Egyptian SoJIA patients. A group of healthy children were assigned to the control group in an attempt to estimate the carrier rate of MEFV mutations in Egypt. Eighty-four children were recruited in this study; 54 children, age (mean ± standard deviation; 8.31 ± 2.85 years), diagnosed as having SoJIA with no typical symptoms of FMF; 30 healthy age- and gender-matched children served as the control group. All recruited children were screened for 12 common MEFV mutations using a reverse hybridization assay of biotinylated PCR products. SoJIA patients had a significantly higher frequency of MEFV mutations (66.7 %) than in the healthy control population (16.7 %). V726A was the leading mutation in SoJIA patients, with an allelic frequency of 15.74 %, followed by E148Q, with an allelic frequency of 7.4 %. Children who were carriers of MEFV mutations had an 18 times higher risk of developing SoJIA than wild-type carriers [odds ratio 18.0 (95 % CI 5–69), P