AIM:To describe the optical coherence tomography(OCT)findings of the retinal nerve fiber layer thickness(RNFLT)and choroidal thickness(CT)in beta-thalassemia major.METHODS:A systematic search was conducted on PubMed,C...AIM:To describe the optical coherence tomography(OCT)findings of the retinal nerve fiber layer thickness(RNFLT)and choroidal thickness(CT)in beta-thalassemia major.METHODS:A systematic search was conducted on PubMed,Cochrane,and Embase using a combination of specific key words.The records found were screened in two phases(title/abstract,and full-text screening).All the original observational cross-sectional studies conducted on beta-thalassemia major cases and controls reporting the RNFLT and CT were included.The Meta-analysis was run for comparing the OCT measurements between betathalassemia cases and controls including pooled effect size,confidence intervals(CI),quality assessment,and publication bias.The measurements included were RNFLT(average,and in various quadrants),and CT.RESULTS:A total of 10 studies were included in this Meta-analysis including a total of 684 individuals,362 cases and 322 controls.The RNFLT and CT showed a significant reduction in the values of beta-thalassemia cases as compared to controls.The heterogeneity among the included studies was found to be 92.65%for the average RNFLT and 30.13%for the CT making it obvious to use random effects model for analyzing the RNFLT values while fixed effects model for the CT.The Egger’s test showed significant publication bias among all the parameters except for nasal RNFLT(P=0.507),and CT(P=0.281).The estimated average effect size for the average RNFLT was 1.04(95%CI:0.35 to 1.72,Z=2.961,P=0.003)and for CT was 0.74(95%CI:0.51 to 0.96,Z=6.523,P<0.001).CONCLUSION:This Meta-analysis concludes that the RNFLT and CT are significantly thinner in beta-thalassemia cases in comparison to healthy individuals.Therefore,the RNFLT and CT must be evaluated in routine clinical practice in order to avoid irreversible vision loss particularly in betathalassemia individuals.展开更多
Iron deficiency anemia and iron overload conditions affect more than one billion people worldwide.Iron homeostasis involves the regulation of cells that export iron into the plasma and cells that utilize or store iron...Iron deficiency anemia and iron overload conditions affect more than one billion people worldwide.Iron homeostasis involves the regulation of cells that export iron into the plasma and cells that utilize or store iron.The cellular iron balance in humans is primarily mediated by the hepcidin-ferroportin axis.Ferroportin is the sole cellular iron export protein,and its expression is regulated transcriptionally,post-transcriptionally and posttranslationally.Hepcidin,a hormone produced by liver cells,post-translationally regulates ferroportin expression on iron exporting cells by binding with ferroportin and promoting its internalization by endocytosis and subsequent degradation by lysosomes.Dysregulation of iron homeostasis leading to iron deposition in vital organs is the main cause of death in betathalassemia patients.Beta-thalassemia patients show marked hepcidin suppression,ineffective eiythropoiesis,anemia and iron overload.Beta-thalassemia is common in the Mediterranean region,Southeast Asia and the Indian subcontinent,and the focus of this review is to provide an update on the factors mediating hepcidin related iron dysregulation in beta-thalassemia disease.Understanding this process may pave the way for new treatments to ameliorate iron overloading and improve the long term prognosis of these patients.展开更多
AIM: To detect the prevalence of anti-HAV IgG antibodies in adult multitransfused beta-thalassemic patients. METHODS: We studied 182 adult beta-thalassemic patients and 209 controls matched for age and sex from the sa...AIM: To detect the prevalence of anti-HAV IgG antibodies in adult multitransfused beta-thalassemic patients. METHODS: We studied 182 adult beta-thalassemic patients and 209 controls matched for age and sex from the same geographic area, at the same time. Anti-HAV IgG antibodies, viral markers of hepatitis B virus (HBV) and hepatitis C virus (HCV) infection were evaluated. RESULTS: Anti-HAV IgG antibodies were detected more frequently in thalassemic patients (133/182; 73.1%) than in healthy controls (38/209; 18.2%, P < 0.0005). When we retrospectively evaluated the prevalence of anti-HAV IgG antibodies in 176/182 (96.7%) thalassemic patients, whose medical history was available for the previous ten years, it was found that 83 (47.2%) of them were continuously anti-HAV IgG positive, 16 (9.1%) acquired anti-HAV IgG antibody during the previous ten years, 49 (27.8%) presented anti-HAV positivity intermittently and 28 (15.9%) were anti-HAV negative continuously. CONCLUSION: Multitransfused adult beta-thalassemic patients present higher frequency of anti-HAV IgG antibodies than normal population of the same geographic area. This difference is difficult to explain, but it can be attributed to the higher vulnerability of thalassemics to HAV infection and to passive transfer of anti-HAV antibodies by blood transfusions.展开更多
I. INTRODUCTIONAlthough the PCR (polymerase chain reaction )combined with ASO (allele-specific oligonucleotide) probe hybridization has greatly facilitated the gene diagnosis and the prenatal diagnosis of beta-thalass...I. INTRODUCTIONAlthough the PCR (polymerase chain reaction )combined with ASO (allele-specific oligonucleotide) probe hybridization has greatly facilitated the gene diagnosis and the prenatal diagnosis of beta-thalassemia, the methodology requires the use of radioactive probes, and it is rather time-consuming and still technically complex. To simplify展开更多
HbE is the most common hemoglobin variant in the world.In compound heterozygotes,the interaction of HbE with othermutations in the β-globin gene results in HbE/β-thalasse-mia disease,which shows great variation in s...HbE is the most common hemoglobin variant in the world.In compound heterozygotes,the interaction of HbE with othermutations in the β-globin gene results in HbE/β-thalasse-mia disease,which shows great variation in severity,includingcases that require blood transfusions just like the β-thalasse-mia major patients.Many hematologic disease could developmyelofibrosis.However,HbE/β-thalassemia with myelofibro-展开更多
文摘AIM:To describe the optical coherence tomography(OCT)findings of the retinal nerve fiber layer thickness(RNFLT)and choroidal thickness(CT)in beta-thalassemia major.METHODS:A systematic search was conducted on PubMed,Cochrane,and Embase using a combination of specific key words.The records found were screened in two phases(title/abstract,and full-text screening).All the original observational cross-sectional studies conducted on beta-thalassemia major cases and controls reporting the RNFLT and CT were included.The Meta-analysis was run for comparing the OCT measurements between betathalassemia cases and controls including pooled effect size,confidence intervals(CI),quality assessment,and publication bias.The measurements included were RNFLT(average,and in various quadrants),and CT.RESULTS:A total of 10 studies were included in this Meta-analysis including a total of 684 individuals,362 cases and 322 controls.The RNFLT and CT showed a significant reduction in the values of beta-thalassemia cases as compared to controls.The heterogeneity among the included studies was found to be 92.65%for the average RNFLT and 30.13%for the CT making it obvious to use random effects model for analyzing the RNFLT values while fixed effects model for the CT.The Egger’s test showed significant publication bias among all the parameters except for nasal RNFLT(P=0.507),and CT(P=0.281).The estimated average effect size for the average RNFLT was 1.04(95%CI:0.35 to 1.72,Z=2.961,P=0.003)and for CT was 0.74(95%CI:0.51 to 0.96,Z=6.523,P<0.001).CONCLUSION:This Meta-analysis concludes that the RNFLT and CT are significantly thinner in beta-thalassemia cases in comparison to healthy individuals.Therefore,the RNFLT and CT must be evaluated in routine clinical practice in order to avoid irreversible vision loss particularly in betathalassemia individuals.
基金supported by the Thailand Research Fund(BRG5780004, IRG5780009 and IRN58W0002)by a Research Chair Grant from the National Science and Technology Development Agency(NSTDA)supported by a Thai Royal Golden Jubilee Ph.D.Research Scholarship(PHD/0101/2553)
文摘Iron deficiency anemia and iron overload conditions affect more than one billion people worldwide.Iron homeostasis involves the regulation of cells that export iron into the plasma and cells that utilize or store iron.The cellular iron balance in humans is primarily mediated by the hepcidin-ferroportin axis.Ferroportin is the sole cellular iron export protein,and its expression is regulated transcriptionally,post-transcriptionally and posttranslationally.Hepcidin,a hormone produced by liver cells,post-translationally regulates ferroportin expression on iron exporting cells by binding with ferroportin and promoting its internalization by endocytosis and subsequent degradation by lysosomes.Dysregulation of iron homeostasis leading to iron deposition in vital organs is the main cause of death in betathalassemia patients.Beta-thalassemia patients show marked hepcidin suppression,ineffective eiythropoiesis,anemia and iron overload.Beta-thalassemia is common in the Mediterranean region,Southeast Asia and the Indian subcontinent,and the focus of this review is to provide an update on the factors mediating hepcidin related iron dysregulation in beta-thalassemia disease.Understanding this process may pave the way for new treatments to ameliorate iron overloading and improve the long term prognosis of these patients.
文摘AIM: To detect the prevalence of anti-HAV IgG antibodies in adult multitransfused beta-thalassemic patients. METHODS: We studied 182 adult beta-thalassemic patients and 209 controls matched for age and sex from the same geographic area, at the same time. Anti-HAV IgG antibodies, viral markers of hepatitis B virus (HBV) and hepatitis C virus (HCV) infection were evaluated. RESULTS: Anti-HAV IgG antibodies were detected more frequently in thalassemic patients (133/182; 73.1%) than in healthy controls (38/209; 18.2%, P < 0.0005). When we retrospectively evaluated the prevalence of anti-HAV IgG antibodies in 176/182 (96.7%) thalassemic patients, whose medical history was available for the previous ten years, it was found that 83 (47.2%) of them were continuously anti-HAV IgG positive, 16 (9.1%) acquired anti-HAV IgG antibody during the previous ten years, 49 (27.8%) presented anti-HAV positivity intermittently and 28 (15.9%) were anti-HAV negative continuously. CONCLUSION: Multitransfused adult beta-thalassemic patients present higher frequency of anti-HAV IgG antibodies than normal population of the same geographic area. This difference is difficult to explain, but it can be attributed to the higher vulnerability of thalassemics to HAV infection and to passive transfer of anti-HAV antibodies by blood transfusions.
基金Project supported by the National 863 Research Grant.
文摘I. INTRODUCTIONAlthough the PCR (polymerase chain reaction )combined with ASO (allele-specific oligonucleotide) probe hybridization has greatly facilitated the gene diagnosis and the prenatal diagnosis of beta-thalassemia, the methodology requires the use of radioactive probes, and it is rather time-consuming and still technically complex. To simplify
文摘HbE is the most common hemoglobin variant in the world.In compound heterozygotes,the interaction of HbE with othermutations in the β-globin gene results in HbE/β-thalasse-mia disease,which shows great variation in severity,includingcases that require blood transfusions just like the β-thalasse-mia major patients.Many hematologic disease could developmyelofibrosis.However,HbE/β-thalassemia with myelofibro-
