GALLSTONES IN PATIENTS WITH INHERITED HEMOLYTIC DISEASES

The purpose is to provide an overview on the incidence of gallstone disease in patients with various types of inherited (chronic) hemolytic diseases at risk of cholelithiasis/choledocholithiasis with particular emphasis on its pathogenesis, genetic, risk factors and management. A detailed electronic literature search to determine the source of materials for this review article was done. The reported incidences of gallstones and choledocholithiasis vary according to the different types of inherited hemolytic diseases and the ethnicity of the studied populations. To date, no review article summarises the incidences of cholelithiasis in patients with various inherited haemolytic diseases was published. Regular ultrasound examination for the presence of gallstones recommended in patients with inherited haemolytic anaemias, particularly those with additional risk factors recommended. Further studies for evaluating the reasons for the higher incidence of cholelithiasis in thalassemia major and sickle cell anemia compared to hereditary spherocytosis; the effect of co inheritance of alpha thalassaemia on decreasing bilirubin level in patients with sickle cell disease and beta thalassaemia; the effect of the co inheritance of UGT1A1 and ABCG8 gene mutation on the incidence of gallstones in other blood diseases such as Hb-H disease, autoimmune haemolytic anaemias, congenital dyserythropoietic anaemia, hereditary elliptocytosis, Southeast Asian Ovalocytosis, glucose-6-phosphate and pyruvate kinase deficiency are recommended. Evaluation of the potential role of the solubility of the mutant proteins and haemoglobin subunit in the red blood cells as an additional mechanism for the development of gallstones in patients with inherited haemolytic anaemias recommended

Game-based student e-learning experience: Empirical evidence from private universities in Jordan

This study investigates the impact of game-based (gamification) e-Learning techniques on students' engagement, thereby, their satisfaction with e-learning in Jordanian private universities. A conceptual model was developed based on existing empirical evidence from the literature. Data was then collected through a self-administered questionnaire survey from 198 private university students, who were conveniently selected for the study. The data was analyzed using Structural Equation Modeling (SEM) with smart PLS 23. Data analysis revealed a positive effect of gamification on both student engagement and satisfaction, suggesting that incorporating gaming elements into the e-learning process in Jordanian private universities led to higher levels of student engagement, thereby greater student satisfaction with the e-learning experience. A mediating role was also found for student engagement on the effect of gamification on student satisfaction. The findings provide insights to practitioners on how gamification can be utilized as an effective strategy to deliver a more enjoyable and interactive e-learning experience. Research findings were discussed, and conclusions and implications were lastly provided

Demystifying Smoker's Paradox: A Propensity Score-Weighted Analysis in Patients Hospitalized With Acute Heart Failure.

Background Smoker's paradox has been observed with several vascular disorders, yet there are limited data in patients with acute heart failure (HF). We examined the effects of smoking in patients with acute HF using data from a large multicenter registry. The objective was to determine if the design and analytic approach could explain the smoker's paradox in acute HF mortality. Methods and Results The data were sourced from the acute HF registry (Gulf CARE [Gulf Acute Heart Failure Registry]), a multicenter registry that recruited patients over 10 months admitted with a diagnosis of acute HF from 47 hospitals in 7 Middle Eastern countries. The association between smoking and mortality (in hospital) was examined using covariate adjustment, making use of mortality risk factors. A parallel analysis was performed using covariate balancing through propensity scores. Of 5005 patients hospitalized with acute HF, 1103 (22%) were current smokers. The in-hospital mortality rates were significantly lower in current smoker's before (odds ratio, 0.71; 95% CI, 0.52-0.96) and more so after (odds ratio, 0.47; 95% CI, 0.31-0.70) covariate adjustment. With the propensity score-derived covariate balance, the smoking effect became much less certain (odds ratio, 0.63; 95% CI, 0.36-1.11). Conclusions The current study illustrates the fact that the smoker's paradox is likely to be a result of residual confounding as covariate adjustment may not resolve this if there are many competing prognostic confounders. In this situation, propensity score methods for covariate balancing seem preferable. Clinical Trial Registration URL: https://www.clinicaltrials.gov/. Unique identifier: NCT01467973.Gulf CARE (Gulf Acute Heart Failure Registry) is an investigator- initiated study conducted under the auspices of the Gulf Heart Association and funded by Servier, Paris, France; and (for centers in Saudi Arabia), by the Saudi Heart Association (The Deanship of Scientific Research at King Saud University, Riyadh, Saudi Arabia [research group number: RG -1436- 013]). This does not alter our adherence to policies on sharing data and materials; and the funders had no role in study design, data collection and analysis, decision to publish, or preparation of the manuscript. The publication of this article was funded by the Qatar National Library

Gallstones in patients with inherited hemolytic diseases

The purpose is to provide an overview on the incidence of gallstone disease in patients with various types of inherited (chronic) hemolytic diseases at risk of cholelithiasis/choledocholithiasis with particular emphasis on its pathogenesis, genetic, risk factors and management. A detailed electronic literature search to determine the source of materials for this review article was done. The reported incidences of gallstones and choledocholithiasis vary according to the different types of inherited hemolytic diseases and the ethnicity of the studied populations. To date, no review article summarises the incidences of cholelithiasis in patients with various inherited haemolytic diseases was published. Regular ultrasound examination for the presence of gallstones recommended in patients with inherited haemolytic anaemias, particularly those with additional risk factors recommended. Further studies for evaluating the reasons for the higher incidence of cholelithiasis in thalassemia major and sickle cell anemia compared to hereditary spherocytosis; the effect of co inheritance of alpha thalassaemia on decreasing bilirubin level in patients with sickle cell disease and beta thalassaemia; the effect of the co inheritance of UGT1A1 and ABCG8 gene mutation on the incidence of gallstones in other blood diseases such as Hb-H disease, autoimmune haemolytic anaemias, congenital dyserythropoietic anaemia, hereditary elliptocytosis, Southeast Asian Ovalocytosis, glucose-6-phosphate and pyruvate kinase deficiency are recommended. Evaluation of the potential role of the solubility of the mutant proteins and haemoglobin subunit in the red blood cells as an additional mechanism for the development of gallstones in patients with inherited haemolytic anaemias recommended

Relative proteome quantification of alpha, beta, gamma and delta globin chains in early eluting peaks of Bio-Rad variant II® CE-HPLC of hemoglobin from healthy and beta-thalassemia subjects in Malaysia

This is the first report of QQQ-mass spectrometric identification and quantification of the Hb subunits, alpha, beta, delta and gamma globin peptides, derived from enzymatic-digestion of proteins in the early unknown peaks of the Bio-Rad cation-exchange chromatography of haemoglobin. The objectives were to assess the relationship of the quantity of the free alpha, beta, delta and gamma globin chains with the phenotypic diversity of beta-thalassaemias (β-thal). The results demonstrate that the pools of free globin chains in red blood cells were correlating with the severity of the disease in patients with different phenotypes of β-thal. The mechanism and the regulation of synthesis of free globin chains pool in a normal individual and in patients with different β-thal phenotypes could arise from several mechanisms which will require further investigation. The role of the free globin pool in patients with β-thal for development of novel therapeutic approaches based on these potential targets requires further investigation. Pertinent biomarkers improves the diagnosis of the β-thal, especially in low-income countries where they are most common and allows more effective therapeutic intervention leading to more successful therapeutic outcome. Keywords: Anaemia, Thalassemia, Mass spectrometry, Globin chain, Quantification, HPLC, Unknown peak

Reduction in Door-to-Needle Time after Transfer of Thrombolysis Site from CCU to Emergency Department

Objective. Early restoration of coronary perfusion by thrombolysis or percutaneous coronary intervention is the main modality of treatment to salvage the ischemic myocardium. The earlier the procedure is completed, the greater the benefit is in saving myocardium and restoring its functions. The aim of the study is to compare the door-to-needle time (DNT) in acute ST elevation myocardial infarction (STEMI) in the period prior to December 2008 when the site of thrombolysis was in coronary care unit (CCU) and the period after that when the site was shifted to emergency department (ED). Methods. A retrospective, descriptive study was conducted at Al Khor Hospital, Qatar, in patients with acute STEMI who underwent thrombolysis at CCU and ED from April 2005 until December 2011, to compare the DNT, duration of hospitalization, and mortality. Results. A total of 211 patients with acute STEMI were eligible for thrombolysis; 58 patients were thrombolysed in the CCU and 153 in ED. The median DNT was reduced from 33.5 minutes in the CCU to 17 minutes in the ED representing a reduction of more than 50% with a P value of < 0.0001. Conclusion. The transfer of the thrombolysis site from CCU to the ED was associated with a dramatic and significant reduction in median door-to-needle time by more than half

Immediate and one-year outcome of patients presenting with Acute Coronary Syndrome complicated by stroke: Findings from the 2ndGulf Registry of Acute Coronary Events (Gulf RACE-2)

BACKGROUND: Stroke is a potential complication of acute coronary syndrome (ACS). The aim of this study was to identify the prevalence, risk factors predisposing to stroke, in-hospital and 1-year mortality among patients presenting with ACS in the Middle East. METHODS: For a period of 9 months in 2008 to 2009, 7,930 consecutive ACS patients were enrolled from 65 hospitals in 6 Middle East countries. RESULTS: The prevalence of in-hospital stroke following ACS was 0.70%. Most cases were ST segment elevation MI-related (STEMI) and ischemic stroke in nature. Patients with in-hospital stroke were 5 years older than patients without stroke and were more likely to have hypertension (66% vs. 47.6%, P = 0.001). There were no differences between the two groups in regards to gender, other cardiovascular risk factors, or prior cardiovascular disease. Patients with stroke were more likely to present with atypical symptoms, advanced Killip class and less likely to be treated with evidence-based therapies. Independent predictors of stroke were hypertension, advanced killip class, ACS type –STEMI and cardiogenic shock. Stroke was associated with increased risk of in-hospital (39.3% vs. 4.3%) and one-year mortality (52% vs. 12.3%). CONCLUSION: There is low incidence of in-hospital stroke in Middle-Eastern patients presenting with ACS but with very high in-hospital and one-year mortality rates. Stroke patients were less likely to be appropriately treated with evidence-based therapy. Future work should be focused on reducing the risk and improving the outcome of this devastating complication