In this Editorial,we highlight the possible role that metabolism dysfunction-associated steatotic liver disease(MASLD)may play in the future,regarding liver disease in patients with transfusion-dependent β-thalassemi...In this Editorial,we highlight the possible role that metabolism dysfunction-associated steatotic liver disease(MASLD)may play in the future,regarding liver disease in patients with transfusion-dependent β-thalassemia(TDBT).MASLD is characterized by excessive accumulation of fat in the liver(hepatic steatosis),in the presence of cardiometabolic factors.There is a strong correlation between the occurrence of MASLD and insulin resistance,while its increased prevalence parallels the global epidemic of diabetes mellitus(DM)and obesity.Patients with TDBT need regular transfusions for life to ensure their survival.Through these transfusions,a large amount of iron is accumulated,which causes saturation of transferrin and leads to the circulation of free iron molecules,which cause damage to vital organs(primarily the liver and myocardium).Over the past,the main mechanisms for the development of liver disease in these patients have been the toxic effect of iron on the liver and chronic hepatitis C,for which modern and effective treatments have been found,resulting in successful treatment.Additional advances in the treatment and monitoring of these patients have led to a reduction in deaths,and an increase in their life expectancy.This increased survival makes them vulnerable to the onset of diseases,which until recently were mainly related to the non-thalassemic general population,such as obesity and DM.There is insufficient data in the literature regarding the prevalence of MASLD in this population or on the risk factors for its occurrence.However,it was recently shown by a study of 45 heavily transfused patients with beta-thalassemia(Padeniya et al,BJH),that the presence of steatosis is a factor influencing the value of liver elastography and thus liver fibrosis.These findings suggest that future research in the field of liver disease in patients with TDBT should be focused on the occurrence,the risk factors,and the effect of MASLD on these patients.展开更多
This study focused on the way that Adolescents with Transfusion- dependent thalassemia explained negative or positive events in their life （Attributional Styles）. It is defined by three dimensions describing the cog...This study focused on the way that Adolescents with Transfusion- dependent thalassemia explained negative or positive events in their life （Attributional Styles）. It is defined by three dimensions describing the cognitive appraisal of the events： internal-external, stable-unstable, and global-specific. With cross-sectional research design, the observations consist of 102 adolescents （48 males, 54 females） who diagnosed with Transfusion-dependent thalassemia （more than 50 times for blood transfusions） completed the measure of Attributional Styles and Anxiety Questionnaires. The correlations in the predicted directions among variables examine with Pearson product-moment correlation coefficients, t-test, and One-way ANOVA to ascertain a significant between the group differences on attributional factors and levels of anxiety symptoms. The results show that Adolescent samples with higher levels of anxiety revealed statistically significant relationship among three negative attributional dimensions （overall composite F = 4.5, p 〈 0.05; negative composite F = 4.99, p 〈 0.01; negative-internality F = 4.99 p 〈 0.01; negative-stability F = 3.42, p 〈 0.05 and negative-globality F = 3.77, p 〈 0.05）. In addition, significant age- group differences were found for the total negative-globality （t = 2.05, p 〈 0.05） and negative- globality （t = -2.22, p 〈 0.05）. These data are consistent with the reformulated learned helplessness model of depression. In finding, the individuals who attribute negative life events to internal, stable, and global causes will be more vulnerable to anxiety than those who make external, unstable, and specific attributions. Most interestingly, those adolescents more than 17 years evidence more negative-globality attfibutional style than group less than 16 years, and female adolescents may influence this pattern. These results suggest that targeting Adolescents with Transfusion-dependent thalassemia may be important for improving aspect of coping on psychological adjustment to their chronic illness.展开更多
Objective To determine whether cyclosporine A(CsA)plus andronges was as effective as the current standard immunosuppressive therapy(IST)for transfusion-dependent nonsevere aplastic anemia(TD-NSAA).
Survival in patients with transfusion-dependent thalassemias(TDT)has increased,and complications such as hepatocellular carcinoma(HCC)are emerging.Risk factors include viral infection,mainly hepatitis C virus(HCV),iro...Survival in patients with transfusion-dependent thalassemias(TDT)has increased,and complications such as hepatocellular carcinoma(HCC)are emerging.Risk factors include viral infection,mainly hepatitis C virus(HCV),iron overload,the presence of cirrhosis,and immune dysregulation.Median survival after HCC occurrence has been estimated at 12 months,while data regarding the incidence of HCC in this population are minimal.Implementing effective hepatitis B virus(HBV)/HCV antiviral treatment and universal HBV vaccination programs is expected to decrease the risk for hepatocarcinogenesis substantially.Significant hemosiderosis and hepatic fibrosis are common in patients with TDT despite chelation therapy and have been correlated with HCC development.Thus,iron overload should be monitored with liver iron concentration and ferritin levels,and effective chelation therapy should be applied.In addition,all TDT patients,particularly those with cirrhosis,should be under surveillance every six months with abdominal ultrasound±alpha-fetoprotein levels,as this combination seems to provide better sensitivity for early HCC detection.展开更多
文摘In this Editorial,we highlight the possible role that metabolism dysfunction-associated steatotic liver disease(MASLD)may play in the future,regarding liver disease in patients with transfusion-dependent β-thalassemia(TDBT).MASLD is characterized by excessive accumulation of fat in the liver(hepatic steatosis),in the presence of cardiometabolic factors.There is a strong correlation between the occurrence of MASLD and insulin resistance,while its increased prevalence parallels the global epidemic of diabetes mellitus(DM)and obesity.Patients with TDBT need regular transfusions for life to ensure their survival.Through these transfusions,a large amount of iron is accumulated,which causes saturation of transferrin and leads to the circulation of free iron molecules,which cause damage to vital organs(primarily the liver and myocardium).Over the past,the main mechanisms for the development of liver disease in these patients have been the toxic effect of iron on the liver and chronic hepatitis C,for which modern and effective treatments have been found,resulting in successful treatment.Additional advances in the treatment and monitoring of these patients have led to a reduction in deaths,and an increase in their life expectancy.This increased survival makes them vulnerable to the onset of diseases,which until recently were mainly related to the non-thalassemic general population,such as obesity and DM.There is insufficient data in the literature regarding the prevalence of MASLD in this population or on the risk factors for its occurrence.However,it was recently shown by a study of 45 heavily transfused patients with beta-thalassemia(Padeniya et al,BJH),that the presence of steatosis is a factor influencing the value of liver elastography and thus liver fibrosis.These findings suggest that future research in the field of liver disease in patients with TDBT should be focused on the occurrence,the risk factors,and the effect of MASLD on these patients.
文摘This study focused on the way that Adolescents with Transfusion- dependent thalassemia explained negative or positive events in their life （Attributional Styles）. It is defined by three dimensions describing the cognitive appraisal of the events： internal-external, stable-unstable, and global-specific. With cross-sectional research design, the observations consist of 102 adolescents （48 males, 54 females） who diagnosed with Transfusion-dependent thalassemia （more than 50 times for blood transfusions） completed the measure of Attributional Styles and Anxiety Questionnaires. The correlations in the predicted directions among variables examine with Pearson product-moment correlation coefficients, t-test, and One-way ANOVA to ascertain a significant between the group differences on attributional factors and levels of anxiety symptoms. The results show that Adolescent samples with higher levels of anxiety revealed statistically significant relationship among three negative attributional dimensions （overall composite F = 4.5, p 〈 0.05; negative composite F = 4.99, p 〈 0.01; negative-internality F = 4.99 p 〈 0.01; negative-stability F = 3.42, p 〈 0.05 and negative-globality F = 3.77, p 〈 0.05）. In addition, significant age- group differences were found for the total negative-globality （t = 2.05, p 〈 0.05） and negative- globality （t = -2.22, p 〈 0.05）. These data are consistent with the reformulated learned helplessness model of depression. In finding, the individuals who attribute negative life events to internal, stable, and global causes will be more vulnerable to anxiety than those who make external, unstable, and specific attributions. Most interestingly, those adolescents more than 17 years evidence more negative-globality attfibutional style than group less than 16 years, and female adolescents may influence this pattern. These results suggest that targeting Adolescents with Transfusion-dependent thalassemia may be important for improving aspect of coping on psychological adjustment to their chronic illness.
文摘Objective To determine whether cyclosporine A(CsA)plus andronges was as effective as the current standard immunosuppressive therapy(IST)for transfusion-dependent nonsevere aplastic anemia(TD-NSAA).
文摘Survival in patients with transfusion-dependent thalassemias(TDT)has increased,and complications such as hepatocellular carcinoma(HCC)are emerging.Risk factors include viral infection,mainly hepatitis C virus(HCV),iron overload,the presence of cirrhosis,and immune dysregulation.Median survival after HCC occurrence has been estimated at 12 months,while data regarding the incidence of HCC in this population are minimal.Implementing effective hepatitis B virus(HBV)/HCV antiviral treatment and universal HBV vaccination programs is expected to decrease the risk for hepatocarcinogenesis substantially.Significant hemosiderosis and hepatic fibrosis are common in patients with TDT despite chelation therapy and have been correlated with HCC development.Thus,iron overload should be monitored with liver iron concentration and ferritin levels,and effective chelation therapy should be applied.In addition,all TDT patients,particularly those with cirrhosis,should be under surveillance every six months with abdominal ultrasound±alpha-fetoprotein levels,as this combination seems to provide better sensitivity for early HCC detection.
