Serum-soluble suppression of tumourigenicity-2 as a biomarker in children with congestive heart failure.

BACKGROUND: We aimed to evaluate serum soluble suppression of tumorigenicity-2 in children with congestive heart failure, to assess the diagnostic and prognostic values of soluble suppression of tumorigenicity-2 in these patients, and to correlate its levels with various clinical and echocardiographic data. METHODS: We included 60 children with congestive heart failure as the patient group. Sixty healthy children of matched age and sex served as the control group. Patients were evaluated clinically and by echocardiography. Serum level of suppression of tumorigenicity-2 was measured for patients at admission. All patients were followed up for death or readmission for a period of one year. RESULTS: Soluble suppression of tumorigenicity-2 was significantly higher in children with congestive heart failure as compared to the control group. Soluble suppression of tumorigenicity-2 was significantly increased in patients with higher severity of congestive heart failure. There was a significant increase in soluble suppression of tumorigenicity-2 in patients with bad prognosis compared to those with good prognosis. There was a significant positive correlation between soluble suppression of tumorigenicity-2 and respiratory rate, heart rate, and clinical stage of congenital heart failure, while there was a significant negative correlation between soluble suppression of tumorigenicity-2 and left ventricular systolic and diastolic function. The best cut-off of soluble suppression of tumorigenicity-2 to diagnose congestive heart failure was > 3.6 with 87% sensitivity and 79% specificity. The cut-off point of soluble suppression of tumorigenicity-2 to diagnose congestive heart failure in children was ≥ 31.56 ng/ml, with 95% sensitivity and 91.37% specificity. Moreover, the cut-off point of soluble suppression of tumorigenicity-2 to predict bad prognosis in children with congestive heart failure was ≥ 255.5 ng/ml, with 92% sensitivity and 89.0% specificity. CONCLUSION: Soluble suppression of tumorigenicity-2 is a good diagnostic and predictive biomarker in children with congestive heart failure.

Therapeutic efficacy of different iron chelators in Egyptian children with Beta Thalassemia with iron overload.

BACKGROUNDS: Beta thalassemia is a hereditary blood disorder characterized by reduced or absent beta chains of hemoglobin resulting in imbalanced globin chain synthesis with early destruction of RBCs and anemia. Patients with thalassemia major become transfusion- dependent with subsequent iron overload. Effective iron chelation therapy remains the main target of management of thalassemia major. OBJECTIVES: 'The aim of this work was to compare the efficacy of different iron chelating agents' in the treatment of ' iron overload in children with beta thalassemia major'. PATIENTS AND METHODS: 'The current study was conducted on 120 children with beta thalassemia major with serum ferritin level of more than 1000 ng/ml who were divided into 4 groups': Group A: 30 patients were treated with 8 hours intravenous infusion of Desferrioxamine, '40 mg/kg/day, 6 days per week for 6 months'. Group B: 30 patients were treated with subcutaneous infusion of Desferrioxamine, 40 mg/kg/day, 6 days per week 8-12 hours per day at night using Desferal pump for 6 months. Group C: 30 patients were treated with oral Deferiprone 75 mg/kg/day in three divided doses daily for 6 months. Group D: 30 patients were treated with oral Deferasirox 30 mg/kg/day in single dose on empty stomach daily for 6 months. 'For all patients laboratory investigations were carried out including complete blood count (CBC), measurement of serum ferritin, serum iron, TIBC (total iron binding capacity), liver enzymes and kidney functions'. RESULTS: There were significant reductions in 'serum ferritin and serum iron' after treatment in all studied groups with the highest reduction in group A, group B, group D and group C but without statistically significant differences between the four studied groups before and after chelation therapy. 'There were no significant differences in' the mean values of the parameters of CBC, liver enzymes and kidney functions between the studied groups before and after chelation therapy. CONCLUSION: From this study we concluded that there was significant reduction in serum ferritin and serum iron after chelation therapy in studied groups with the highest reduction in group A (IV Desferrioxamine), group B (SC Desferrioxamine), group D (oral Deferasirox) and group C (oral Deferiprone) with no statistically significant differences between the studied groups of patients before and after 6 months of regular chelation.

Fibrosis severity and mannan-binding lectin (MBL)/MBL-associated serine protease 1 (MASP-1) complex in HCV-infected patients.

BACKGROUND AND STUDY AIMS: Mannan-binding lectin (MBL) is a collectin synthesised in the liver and secreted into the bloodstream. It binds micro-organisms via interactions with glycans on the target surface. Bound MBL subsequently activates MBL-associated serine protease proenzymes (MASPs). Several studies have investigated the possible role for MBL in hepatitis C virus (HCV) infection by examining MBL levels and polymorphisms in relation to disease progression and in response to treatment. The aim of this study was to investigate the relation of the activity of MBL and MBL/MASP-1 complex in sera of patients with mild and severe chronic HCV infection and outcome of HCV infection. PATIENTS AND METHODS: Serum level of MBL and functional assays for MBL/MASP-1 complex activity were assayed in sera of 80 patients with chronic HCV infection. Patients were divided into two groups according to the results of the liver biopsy, group I (40 HCV patients had mild hepatic fibrosis, Ishak fibrosis stages 0-1) and group II (40 HCV patients had severe hepatic fibrosis, Ishak fibrosis stages 5-6), in addition to 20 control subjects as group III. The analysis of the MBL/MASP-1 complex activity at 0, 3 and 6 months was performed in all patients. RESULTS: Serum levels of MBL and MBL/MASP-1 complex activity were higher in sera of patients with chronic HCV liver disease compared to those in control subjects. There was a correlation between the activity of the MBL/MASP-1 complex and the severity of fibrosis (P=0.003). MBL/MASP-1 complex activity was associated more significantly with severe fibrosis in comparison to MBL concentration. CONCLUSION: MBL and MBL/MASP-1 complex activities play a key role in first-line host defence mechanism against certain infectious agents including HCV infection. However, it is also likely that the role of MBL and MBL/MASP-1 complex activity extends beyond this restricted infection-related view in that it appears to be a key regulator of inflammation.

Regulated upon activation, normal T-cell expressed and secreted chemokine and interleukin-6 in rheumatic pulmonary hypertension, targets for therapeutic decisions.

BACKGROUND: Recent studies have highlighted the possible influence of chemokines and cytokines on several types of pulmonary arterial hypertension (PAH). Increasing interest has also been focussed on their role as a cause of post-cardiopulmonary bypass (CPB) organ dysfunction. HYPOTHESIS: Chemokines and cytokines are involved in the pathobiology of rheumatic PAH. METHODS: Serum levels of the chemokine, regulated upon activation, normal T-cell expressed and secreted (RANTES) and the cytokine interleukin-6 (IL-6) were measured by enzyme-linked immunosorbent assay (ELISA) in 35 patients with rheumatic mitral valve disease and 10 matched healthy subjects (control group). Eleven patients (31.4%) had severe pulmonary hypertension. Subsequently, 23 patients underwent mitral valve replacement. The relation of RANTES and IL-6 circulating level with postoperative organ dysfunction was analysed through multiple organ dysfunction score (MODS). RESULTS: Patients with severe PAH have a significantly higher mean serum level of RANTES compared with other patients (6138.6+/-3543.8 pg/ml vs 1818.2+/-475.2 pg/ml, p=0.0003). The serum level of IL-6 in the patients was statistically different from that of the control (378+/-50.8 pg/ml vs 262+/-90.5 pg/ml, respectively, p=0.002). Patients who required postoperative inotropes had higher preoperative and post-CPB levels of both RANTES and IL-6. While patients with postoperative lung dysfunction had higher levels of IL-6 preoperatively and post-CPB and lower levels of RANTES post-CPB. CONCLUSIONS: RANTES and IL-6 should be investigated as potential therapeutic targets in the control of rheumatic PAH. Improved understanding of the contribution of RANTES and IL-6 to adverse postoperative complications can lead to improved patient outcome.