NT-proBNP in Children With Left to Right Shunt and Dilated Cardiomyopathy.

BACKGROUND: B-type natriuretic peptide (BNP) levels are elevated in children with congenital heart disease involving a left-to-right shunt (LRS) and are also raised in dilated cardiomyopathy (DCM). As far as we know, there are few reports in the literature comparing the change of the NT-proBNP in LRS and DCM especially in the pediatric age group. OBJECTIVES: The aim of the study was to compare the changes of the NT-proBNP in pediatric patients with LRS and DCM. Correlation between the levels of NT-proBNP and the echocardiographic parameters in both groups was determined. PATIENTS AND METHODS: A total of 30 children (13 males and 17 females) participated in the study. There were 11/30 (36.7%) DCM and 19/30 (63.3%) LRS. The control group consisted of 44 healthy infants and children. Manifestations of heart failure (decompensation) were recorded. The NT-pro BNP levels were measured. The following Echo parameters were assessed: systolic function (ejection fraction and fraction shortening), pulmonary to systemic flow (Qp/Qs) in LRS, pulmonary flow and pulmonary artery pressure (SPAP) and LV diastolic function (E-wave, A-wave, E/A ratio and deceleration time). RESULTS: Clinically 17/30 (56.7%) (11 of the LRS and 5 of the DCM) were decompensated. Significant shunt was present in 15/19 (78.9%) in LRS. Systolic dysfunction was presented in 5/30 (16.7%) cases (4 patients were DCM and one case was LRS). Two types of diastolic dysfunction, impaired relaxation in 5/22 (22.7%) patients and restrictive-like filling pattern in 5/16 (31.2 %) were observed. The NT-Pro BNP level was significantly elevated 11 and 16 times in the LRS and DCM groups respectively. Negative significant correlations were observed between the levels of NT-ProBNP and the following echo variables; EDD, LAD, E wave and E/A ratio in the LRS patients. Positive significant correlations were observed between the levels of NT-ProBNP and the following echo variables; PAP and QP/QS in the LRS. Both the PAP and QP/QS were higher in the elevated NT-Pro BNP group compared to the normal level group. The NT-Pro BNP level was elevated in all 17/30 (56.7%) decompensated patients (11 were LRS, 6 were DCM) (P = 0.002). However, the level was elevated in only 7/13 (23.3%) of the compensated patients (3 were LRS, 4 were DCM) (P = 0.002). The NT-Pro BNP level was also elevated in 18/19 cases with pulmonary hypertension (P = 0.01). Finally, we conclude that the NT-ProBNP level is elevated in both LRS and DCM in pediatric age. This elevation is more remarkable with heart failure and increased PAP in both diseased groups. The level was also elevated and correlated to Qp/Qs in the LRS patients. CONCLUSIONS: So, we recommend the use of NT-ProBNP as a routine marker for following up patients with heart failure and pulmonary hypertension in LRS and DCM.

Lymphocyte Activation Markers in Pediatric Kidney Transplant Recipients.

BACKGROUND AND OBJECTIVES: The role of CD4+CD25+ T regulatory cells (Tregs) in immune tolerance in experimental transplantation is very important but the clinical significance of circulating Tregs in the peripheral blood is undetermined. We evaluated the association between the frequency of T cell activation markers CD25 and CD71 and clinical parameters that may affect the level of these T cell markers. METHODS: In 47peditric kidney transplant (KT) recipients and 20 healthy controls, the frequency of T cell activation markers, CD25 and CD71 was measured with flow cytometry after transplantation. Two clinical protocols of induction immunosuppression were used: (1) anti-thymocyte globulin (THYMO) group (n =29) and Basiliximab (BSX) group (n=10). RESULTS: The percentage of circulating CD25 after KT was significantly lower than that in the controls. There is no significant difference between KT and the controls s regard to circulating CD71. The percentage of CD25 was significantly increased in children with acute rejection compared with those without acute rejection. Calcineurin inhibitors (CNIs) decreased the frequency of CD25 but mammalian target rapamycin (mTOR) inhibitor did not. The proportion of CD25 significantly decreased in THYMO group during the first year after transplantation. CONCLUSION: The frequency of circulating T cell activation marker CD25 in pediatric KT recipients is strongly affected by CNIs, and a high frequency of CD25 is associated with acute rejection during the early posttransplant period. The measurement of T cell activation markers, may become a useful immune monitoring tool after kidney transplantation.

Prevalence of Obesity Among Male Adolescents in Arar Saudi Arabia: Future Risk of Cardiovascular Disease.

BACKGROUND: Obesity in adolescence is crucial as it represents an important stage in human life. Dietary habits are greatly associated with lifestyle. Many reports suggested direct relationship between adolescent fatness and increased risk of cardiovascular diseases (CVD) which will be found in young adult population. AIM: Determine the prevalence of overweight and obesity among male adolescents in Arar city, Saudi Arabia (KSA). We estimated the future risk of developing cardiovascular diseases in this age-group and its possible correlation to different lifestyles and dietary habits. RESULTS: A total of 523 male students with a mean age of 16.7 ± 0.9 years participated in the current study in which 30.4% of those students were obese and 17.2% were overweight. A direct relationship was found between body weight and different dietary and lifestyle habits. The risk of CVD based on waist height ratio (WHtR) was found in 33.5% of participants (30.4% obese, 2.1% overweight and 1% normal weight); moreover, the risk of CVD was strongly related to different dietary and lifestyle habits. CONCLUSION: Overweight and obesity were high among adolescent male students in Arar, who became susceptible to the risk of CVD. Arar showed the highest rate of obesity all over KSA. Both obesity and risk of CVD were strongly related to bad dietary habits and lifestyle.

Relationship between Biochemical Bone Markers and Bone Mineral Density in Patients with Phenylketonuria under Restricted Diet.

OBJECTIVE: Most of phenylketonuria (PKU) develops bone turnover impairment and low bone mineral density (BMD). Measurements of BMD reflect only bone mineral status but not the dynamics of bone turnover. Bone markers are a noninvasive tool useful for the assessment of bone formation and bone resorption processes. Our study was to assess the levels of bone markers in PKU in order to select a screen marker and detect the most specific marker which can be combined with BMD for appropriate follow up. METHODS: Thirty three classic PKU patients were studied. BMD and bone mineral content (BMC) were measured. Total alkaline phosphatase (ALP), osteocalcin (OC) and carboxy-terminal propeptide of type I collagen (CICP), osteoprotegerin (OPG), receptor activator of nuclear factor κß ligand (RANKL) and Deoxypyridinoline (DPD) were measured. Findings : Nineteen (57.6%) male and fourteen (42.4 %) female PKU patients were involved in the current study. Their mean age was 8.4±4.6 yrs and the age range 3-19 yrs. The control group consisted of twenty two (52.4%) males and twenty (47.6%) females. Their mean age was 8.5±3.3 yrs and th age range 2-17 yrs. Using the Z score values, there was a significant decrease of total BMC (TBMC-Z), BMD of the femoral neck BMD-FN-Z, BMD of lumbar vertebrae (BMD-L-Z), BMD-FN and DPD while RANKL increased. There was a negative correlation between CICP and TBMC and between CICP and BMD-L in these patients. Also, a negative correlation between ALP and TBMC and between ALP and BMD-L was observed. It was concluded that the ALP provides a good impression of the new bone formation in the PKU patients and it has a highly significant negative correlation with the many parameters of the bone mineral status beside the wide availability of inexpensive and simple methods. So a screening test and/or follow up for the PKU patients using ALP would be available. Once the level of ALP decrease is detected, one can combine it with BMD to explore the bone mineral status and with specific bone markers (OC, RANKL and DBD), to verify the dynamics of bone turnover. CONCLUSION: This schedule will reduce the risk of exposure of these patients to the risk hazards of DXA and limit its use only to a limited number of the highly suspected cases.

Fetuin-A levels in obesity: differences in relation to metabolic syndrome and correlation with clinical and laboratory variables.

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 association 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 resistance calculated by the homeostasis model (HOMA-IR) (r = 0.295) and high-density 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.

Genetic polymorphism of ACE and the angiotensin II type1 receptor genes in children with chronic kidney disease.

AIM AND METHODS: We investigated the association between polymorphisms of the angiotensin converting enzyme-1 (ACE-1) and angiotensin II type one receptor (AT1RA1166C) genes and the causation of renal disease in 76 advanced chronic kidney disease (CKD) pediatric patients undergoing maintenance hemodialysis (MHD) or conservative treatment (CT). Serum ACE activity and creatine kinase-MB fraction (CK-MB) were measured in all groups. Left ventricular mass index (LVMI) was calculated according to echocardiographic measurements. Seventy healthy controls were also genotyped. RESULTS: The differences of D allele and DI genotype of ACE were found significant between MHD group and the controls (p = 0.0001). ACE-activity and LVMI were higher in MHD, while CK-MB was higher in CT patients than in all other groups. The combined genotype DD v/s ID+II comparison validated that DD genotype was a high risk genotype for hypertension .~89% of the DD CKD patients were found hypertensive in comparison to ~ 61% of patients of non DD genotype(p = 0.02). The MHD group showed an increased frequency of the C allele and CC genotype of the AT1RA1166C polymorphism (P = 0.0001). On multiple linear regression analysis, C-allele was independently associated with hypertension (P = 0.04). CONCLUSION: ACE DD and AT1R A/C genotypes implicated possible roles in the hypertensive state and in renal damage among children with ESRD. This result might be useful in planning therapeutic strategies for individual patients.

A long-term study of bone mineral density in patients with phenylketonuria under diet therapy.

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.