α:Non-α and Gγ:Aγ globin chain ratios in thalassemia intermedia patients treated with hydroxyurea

Objectives: To elucidate the possible ways by which hydroxyurea molecules affect globin chain (α or β-like) synthesis. Methods: A total of 23 thalassemia intermedia patients (13 male and 10 female) aged between 5 and 26 years were treated for five months with 15 mg/(kg-day) of hydroxyurea. Hemoglobins electrophoresis and globin chain electrophoresis was performed on each sample at different time points before and during the treatment. Results: Fetal hemoglobin increased significantly in most patients and average episodes of transfusion decreased. Both Gγ and Aγ-globin chains increased significantly and α-globin:Nonα-globin chain as well as Gγ-globin:Aγ globin chains ratios decreased. Conclusions: Improvement in α:non-α ratio and consequent decrease of free α-globin chain might be the cause of beneficial effects of hydroxyurea therapy. Two patients who felt better didn't show significant increase in their fetal hemoglobin level, and this is in contradiction with the hypothesis claiming that the HbF level increase is the cause of such therapeutic effect. In spite of the unclear mechanism of action of this drug, hydroxyurea therapy had noticeable impacts on thalassemia intermedia and also sickle cell disease and even patients suffering from thalassemia major. © 2014 by the Asian Pacific Journal of Tropical Biomedicine

The best obesity indices to use in a single factor model indicating metabolic syndrome: A population based study

Objective: Although metabolic syndrome (MetS) is a major health problem worldwide, there is no universal agreement on its definition. One of the major disagreements is dealing with the issue of obesity in this definition. This study was conducted to determine a preferably better index of obesity which can be interrelated with other components of MetS in a single factor model of MetS. Design: Out of 6140 participants of a cohort study of subjects aged 10-90 years in northern Iran, the baseline data of 5616 participants aged 18-75 was considered. Confirmatory factor analysis was conducted using AMOS software to evaluate a single factor model of MetS in which blood pressure, triglyceride (TG), high density lipoprotein (HDL), fasting blood sugar (FBS) and obesity measures including waist circumference (WC), body mass index (BMI), waist to hip ratio (WHR) and waist to height ratio (WHtR) were used as indicators of metabolic syndrome. Four single factor models differing from each other by obesity indices were evaluated. The models were evaluated in all 5616 subjects and 4931 subjects without diabetes mellitus according to sex separately. Results: All single factor models had appropriate fit indices with CFI > 0.95, GFI > 0.95 and RMSEA < 0.08 in non-diabetic population, wherein all models obtained the best values of fit indices in men and good fit indices in women. In the general population of men, the single factor models built based on WHR (Chi-square=6.9, df=2, P-value=0.031, RMSEA = 0.028, CI = 0.007-0.052, CFI = 0.994, GFI = 0.999 and AIC = 22.9) and WHtR (Chi-square = 9.97, df = 2, P-value = 0.007, RMSEA = 0.036, CI = 0.016-0.059, CFI = 0.992, GFI = 0.998 and AIC = 25.97) were fitted properly with data while in th general population of women, the model based on WHR obtained better fit indices (Chi-square = 7.5, df = 2, P-value = 0.023, RMSEA = 0.033, CI = 0.011-0.060, CFI = 0.994, GFI = 0.998 and AIC = 23.5). Models based on WHtR obtained better regression weights than WHR. Conclusion: While single factor validity of MetS was confirmed in almost all models, the best models were different according to sex and population of study. © 2016, Academy of Medical Sciences of I.R. Iran, INIA. All rights reserved