Myocardial siderosis is known as the major cause of death in thalassemia major(TM) patients since it can lead to iron overload cardiomyopathy.Although this condition can be prevented if timely effective intensive chel...Myocardial siderosis is known as the major cause of death in thalassemia major(TM) patients since it can lead to iron overload cardiomyopathy.Although this condition can be prevented if timely effective intensive chelation is given to patients,the mortality rate of iron overload cardiomyopathy still remains high due to late detection of this condition.Various direct and indirect methods of iron assessment,including serum ferritin level,echocardiogram,non-transferrin-bound iron,cardiac magnetic resonance T2*,heart rate variability,and liver biopsy and myocardial biopsy,have been proposed for early detection of cardiac iron overload in TM patients.However,controversial evidence and limitations of their use in clinical practice exist.In this review article,all of these iron assessment methods that have been proposed or used to directly or indirectly determine the cardiac iron status in TM reported from both basic and clinical studies are comprehensively summarized and presented.Since there has been growing evidence in the past decades that cardiac magnetic resonance imaging as well as cardiac autonomic status known as the heart rate variability can provide early detection of cardiac involvement in TM patients,these two methods are also presented and discussed.The existing controversy regarding the assessment of cardiac involvement in thalassemia is also discussed.展开更多
Objective:To evaluate the iron—chelating properties and free—radical scavenging activities of1-(N-acetyl-6-aminohexyl)-3-hydroxy-2-methyIpyridin—4-one(CM1) treatment in chronic iron-loaded β-thalassemic(BKO) mice....Objective:To evaluate the iron—chelating properties and free—radical scavenging activities of1-(N-acetyl-6-aminohexyl)-3-hydroxy-2-methyIpyridin—4-one(CM1) treatment in chronic iron-loaded β-thalassemic(BKO) mice.Methods:The BKO mice were fed with a ferrocene-rich diet and were orally administered with CM1|50 mg/(kg·day)| for 6 months.Blood levels of non-transferrin hound iron,labile plasma iron.ferritin(Ft) and malondialdehyde were determined.Results:The BKO mice were fed with an iron diet for 8 months which resulted in iron overload.Interestingly,the mice showed a decrease in the non—transferrin bound iron,labile plasma iron and malondialdehyde levels,but not the Ft levels after continuous CM1 treatment.Conclusions:CM1 could be an effective oral iron chelator that can reduce iron overload and lipid peroxidation in chronic iron overload β—thalassemic mice.展开更多
目的探讨非贫血人群血清铁蛋白(serum ferritin,SF)和转铁蛋白饱和度(transferrin saturation,TSAT)与全因死亡和心血管死亡的相关性。方法选取1999—2000年和2001—2002年国家健康和营养检查调查研究(national health and nutrition ex...目的探讨非贫血人群血清铁蛋白(serum ferritin,SF)和转铁蛋白饱和度(transferrin saturation,TSAT)与全因死亡和心血管死亡的相关性。方法选取1999—2000年和2001—2002年国家健康和营养检查调查研究(national health and nutrition examination survey,NHANES)的非贫血人群7167例,于2006年12月31日前对死亡终点进行随访,采用阈值效应分析和多因素cox回归模型分析SF和TAST与全因死亡和心血管死亡风险的相关性。结果7167例患者中男3533例、女3634例,年龄18~85岁,平均(46.1±20.0)岁,BMI平均(27.9±6.2)kg/m2。平均随访(5.1±1.2)年,共随访11623人/年,其中全因死亡452例、心血管死亡117例。SF与全因死亡和心血管死亡呈非线性相关,SF的截断值为200 ng/ml;当SF<200 ng/ml时,SF每增加100 ng/ml,全因死亡风险增加25%(HR=1.252,95%CI:1.068~1.486,P=0.008),心血管死亡风险增加37%(HR=1.370,95%CI:1.076~1.900,P=0.036)。TSAT与全因死亡率呈L型非线性相关,截断值为30%,当TSAT<30%时,TSAT每增加10%,全因死亡风险降低21%(HR=0.791,95%CI:0.681~0.914,P=0.001);TSAT与心血管死亡风险呈线性负相关(HR=0.803,95%CI:0.660~0.963,P=0.014)。结论非贫血人群的SF与全因死亡和心血管死亡呈非线性相关、截断值为200 ng/ml,TSAT与全因死亡呈L型相关、截断值为30%,且TSAT与心血管死亡呈负相关。建议将非贫血人群的SF与TSAT控制在合适范围,以降低死亡风险,并改善预后。展开更多
基金Supported by Thailand Research Fund grants RTA5580006 and BRG5480003
文摘Myocardial siderosis is known as the major cause of death in thalassemia major(TM) patients since it can lead to iron overload cardiomyopathy.Although this condition can be prevented if timely effective intensive chelation is given to patients,the mortality rate of iron overload cardiomyopathy still remains high due to late detection of this condition.Various direct and indirect methods of iron assessment,including serum ferritin level,echocardiogram,non-transferrin-bound iron,cardiac magnetic resonance T2*,heart rate variability,and liver biopsy and myocardial biopsy,have been proposed for early detection of cardiac iron overload in TM patients.However,controversial evidence and limitations of their use in clinical practice exist.In this review article,all of these iron assessment methods that have been proposed or used to directly or indirectly determine the cardiac iron status in TM reported from both basic and clinical studies are comprehensively summarized and presented.Since there has been growing evidence in the past decades that cardiac magnetic resonance imaging as well as cardiac autonomic status known as the heart rate variability can provide early detection of cardiac involvement in TM patients,these two methods are also presented and discussed.The existing controversy regarding the assessment of cardiac involvement in thalassemia is also discussed.
基金Supported by the Royal Golden Jubilee PhD Program.Thailand Research Fund(Grant No.PHD/0333/2551)
文摘Objective:To evaluate the iron—chelating properties and free—radical scavenging activities of1-(N-acetyl-6-aminohexyl)-3-hydroxy-2-methyIpyridin—4-one(CM1) treatment in chronic iron-loaded β-thalassemic(BKO) mice.Methods:The BKO mice were fed with a ferrocene-rich diet and were orally administered with CM1|50 mg/(kg·day)| for 6 months.Blood levels of non-transferrin hound iron,labile plasma iron.ferritin(Ft) and malondialdehyde were determined.Results:The BKO mice were fed with an iron diet for 8 months which resulted in iron overload.Interestingly,the mice showed a decrease in the non—transferrin bound iron,labile plasma iron and malondialdehyde levels,but not the Ft levels after continuous CM1 treatment.Conclusions:CM1 could be an effective oral iron chelator that can reduce iron overload and lipid peroxidation in chronic iron overload β—thalassemic mice.
