Erythrocytic phosphatidylserine exposure and hemostatic alterations in β-thalassemia intermediate patients

Introduction

Hypercoagulable state is one of the common findings in beta-thalassemia intermedia (β-TI), particularly in splenectomized patients, with infrequent blood transfusion. Abnormality of the red blood cells (RBC) membrane due to oxidative damage is suggestive of possible etiologies. Membrane lipid peroxidation increases the exposure of phosphatidylserine (PS) that plays a role in the activation of coagulation factors V and X, subsequently initiating thrombosis. Our aim of this study was to find the probable correlation of the alteration of the PS on the RBC outer membrane with the hypercoagulable state in the β-TI patients.

Materials and methods

Our cross-sectional study was conducted on 39 splenectomized β-TI patients and 38 age-matched healthy controls. The mean age was 37 years. Analysis of the PS exposure on the RBCs was performed by fluorescein isothiocyanate (FITC) conjugated AV protein .Measurement of the coagulation factors X, V and antithrombin III (AT-III) was performed. We also checked the D-dimer levels .Analysis was performed by SPSS16.

Results

Fluorescence of FITC-Annexin V labeling on patients RBCs were higher than healthy controls; (2.8 ± 2.2%) of the patients versus (0.4 ± 0.18%) in the control group and was statistically significant (P < 0.05). Mean levels of factor X and AT-III of the patients as compared with the control group decreased and showed significant difference (P < 0.05).

Conclusions

Circulation of thalassemic RBCs, which abnormally possess PS on RBC membrane outer surface, suggests the possibility of the gradual consumption of the coagulation factors in the presence of a chronic coagulability state.     

Advances in genetics show the need for extending screening strategies for autosomal dominant hypercholesterolaemia

Aims Autosomal dominant hypercholesterolaemia (ADH) is a major risk factor for coronary artery disease. This disorder is caused by mutations in the genes coding for the low-density lipoprotein receptor (LDLR), apolipoprotein B (APOB), and proprotein convertase subtilisin/kexin 9 (PCSK9). However, in 41% of the cases, we cannot find mutations in these genes. In this study, new genetic approaches were used for the identification and validation of new variants that cause ADH. Methods and results Using exome sequencing, we unexpectedly identified a novel APOB mutation, p.R3059C, in a small-sized ADH family. Since this mutation was located outside the regularly screened APOB region, we extended our routine sequencing strategy and identified another novel APOB mutation (p.K3394N) in a second family. In vitro analyses show that both mutations attenuate binding to the LDLR significantly. Despite this, both mutations were not always associated with ADH in both families, which prompted us to validate causality through using a novel genetic approach. Conclusion This study shows that advances in genetics help increasing our understanding of the causes of ADH. We identified two novel functional APOB mutations located outside the routinely analysed APOB region, suggesting that screening for mutations causing ADH should encompass the entire APOB coding sequence involved in LDL binding to help identifying and treating patients at increased cardiovascular risk.     

Designing trauma registry system using a logical framework approach

While trauma registries provide the mechanisms to collect comprehensive, timely and accurate data related to the injuries and evaluate trauma care systems, they have not been established in most developing countries. On the other hand, in complex projects that have large aims, a logical framework approach （LFA） can help summarize and describe the multiple branches of the project systematically, and elucidate the main goals, extensive objectives, activities and expected outcomes. Therefore a LFA can be used to design and guide trauma registry project management, to integrate the cultural, clinical and capacity variations among countries; and to ensure early alignment of the project＇s design and evaluation.     

Increased expression of PD-1 in CD8 + CD3 + T cells correlates with EBV viral load in MS patients

Journal of NeuroVirology - Multiple sclerosis (MS) is one of the common autoimmune diseases. The exact etiology of MS is still unclear, but recent studies have shown the possibility of infectious...     

Measurement of the mechanical properties of soccer balls using digital image correlation method

There are some features for an approved soccer ball by Fédération Internationale de Football Association (FIFA), such as properties of the material, mass, pressure, stitches, etc. Many of these features up to now have been studied; nevertheless, the mechanical properties of the soccer balls to date have not been well reported. The chief purposes of the current research, hence, were to calculate the mechanical properties of the soccer balls, i.e., linear elastic, nonlinear hyperelastic, and viscoelastic, at two different sizes, including 4 and 5 which are using for football and futsal, respectively. To do this, compressive and stress-relaxation loading were applied to 38 approved soccer balls to quantify the stress–strain as well as reduced relaxation function of the balls. The strain/displacement of the balls was also measured via a high-speed camera using Digital Image Correlation (DIC) technique. The results revealed the mean elastic modulus of 66 and 67 kPa for the football and futsal balls, respectively. In addition, the maximum stresses of the football and futsal balls were 16 and 13 kPa, respectively. The nonlinear mechanical response of the soccer balls were analyzed using hyperelastic material models, i.e., Mooney-Rivlin and Ogden. A Finite Element (FE) model was also developed to verify the hyperelastic data compared to the experimental ones and, remarkably, the numerical data were in consistence with the experimental data. Finally, Prony- series was employed to quantify the viscoelastic properties of the balls. During the game, a soccer ball can reach to a speed of 210 km/h that can damage the human eye; however, the injury detail still has not been studied.     

Effect of hydroxychloroquine on treatment and recurrence of acute brucellosis: a single-blind,randomized clinical trial

Brucellosis is associated with a high recurrence rate and requires more than one course of standard treatment; therefore, more research is required to find more effective treatments that lead to prompt recovery, and reduce the relapse of disease. This single-blind, randomized study was designed to evaluate the effect of the standard treatment for brucellosis in combination with hydroxychloroquine.A total of 177 patients with acute brucellosis were randomly assigned to one of two treatment groups: doxycycline-streptomycin (DS) and doxycycline-streptomycin-hydroxychloroquine (DSH). Clinical symptoms and signs, serological tests, and side effects of therapy were compared between the two groups during the treatment course and at three and six months after the end of drug therapy. Of the 177 patients, with a mean age of 40.5?±?16.9 years, 66.1% were males. The mean duration of clinical signs prior to admission was 43.4?±?41.1 days. Appropriate clinical responses, relapse, treatment failure, and adverse drug reactions were seen in 98.9%, 1.2%, 0.0%, and 12.6% of patients, respectively, in the DSH group vs. 86.7%, 11.6%, 2.3%, and 19.8% of patients, respectively, in the DS group. There were significant differences in clinical response and relapse rates between the two groups. The addition of hydroxychloroquine to a doxycycline-streptomycin regimen appears to increase the efficacy of treatment, accelerate improvement of clinical symptoms, and significantly reduce the rate of relapse of brucellosis.