Iron is essential for all organisms including microbial,cancer and human cells. More than a quarter of the human population is affected by abnormalities of iron metabolism, mainly from iron deficiency and iron overloa...Iron is essential for all organisms including microbial,cancer and human cells. More than a quarter of the human population is affected by abnormalities of iron metabolism, mainly from iron deficiency and iron overload. Iron also plays an important role in free radical pathology and oxidative damage which is observed in almost all major diseases, cancer and ageing. New developments include the complete treatment of iron overload and reduction of morbidity and mortality in thalassaemia using deferiprone and selected deferiprone/deferoxamine combinations and also the use of the maltol iron complex in the treatment of iron deficiency anaemia. There is also a prospect of using deferiprone as a universal antioxidant in non iron overloaded diseases such as neurodegenerative, cardiovascular, renal, infectious diseases and cancer. New regulatory molecules of iron metabolism such as endogenous and dietary chelating molecules, hepcidin, mitochondrial ferritin and their role in health and disease is under evaluation. Similarly, new mechanisms of iron deposition, removal, distribution and toxicity have been identified using new techniques such as magnetic resonance imaging increasing our understanding of iron metabolic processes and the targeted treatment of related diseases. The uniform distribution of iron in iron overload between organs and within each organ is no longer valid. Several other controversies such as the toxicity impact of non transferrin bound iron vs injected iron, the excess levels of iron in tissues causing toxicity and the role of chelation on iron absorption need further investigation. Commercial interests of pharmaceutical companies and connections to leading journals are playing a crucial role in shaping worldwide medical opinion on drug sales and use but also patients' therapeutic outcome and safety. Major controversies include the selection criteria and risk/benefit assessment in the use of deferasirox in thalassaemia and more so in idiopathic haemochromatosis, thalassaemia intermedia and ex-thalass展开更多
本研究旨在探讨骨髓增生异常综合征(MDS)患者袪铁治疗后红细胞生成素(EPO)、血红蛋白(Hb)、重组EPO(rEPO)的变化及EPO与血清铁蛋白(SF)的相关性。检测172例MDS患者及30例健康对照者SF、EPO浓度、血清铁(SI)、总铁结合力(TIBC)、C反应蛋...本研究旨在探讨骨髓增生异常综合征(MDS)患者袪铁治疗后红细胞生成素(EPO)、血红蛋白(Hb)、重组EPO(rEPO)的变化及EPO与血清铁蛋白(SF)的相关性。检测172例MDS患者及30例健康对照者SF、EPO浓度、血清铁(SI)、总铁结合力(TIBC)、C反应蛋白(CRP)、Hb;根据CRP值对SF进行调整。对34例低危组、SF>1 000 mg/L的患者给予袪铁胺治疗,比较治疗前后SF、EPO、SI、TIBC、Hb的变化。对58例低危组、EPO<1 000 U/L的患者给予rEPO治疗,比较铁过载组与非铁过载组袪铁治疗前后EPO抵抗的发生率。结果表明:非铁过载组EPO浓度高于正常对照组(997.44±473.48 vs 467.27±238.49)(P<0.05);铁过载组EPO浓度高于无铁过载组及正常对照组(3257.59±697.19 vs 997.44±473.48;3257.59±697.19 vs 467.27±238.49)(P均<0.05);铁过载组EPO抵抗发生率高于非铁过载组(18/35 vs 2/23)(P=0.001),铁过载组MDS患者EPO与SF呈显著正相关(r=0.310)(P=0.036)。袪铁治疗后SF、SI、TIBC及EPO浓度均较治疗前明显下降(3942.38±641.82 vs 2266.35±367.31;48.61±10.65 vs 28.52±12.61;59.84±12.62 vs 33.76±15.43;3808.01±750.22 vs 1954.78±473.18)(P均<0.05),Hb升高(35±18 vs 57±21)(P=0.046),部分EPO抵抗患者恢复疗效。结论:袪铁治疗能增强贫血MDS患者对EPO反应,缓解EPO抵抗,降低EPO病理性升高,提升Hb水平,减少输血依赖。展开更多
Adherence to thalassemia treatment including chelation drugs is influenced by numerous factors. This study aims to explore beliefs about iron chelation therapy and adherence to this medication in Jordanian children wi...Adherence to thalassemia treatment including chelation drugs is influenced by numerous factors. This study aims to explore beliefs about iron chelation therapy and adherence to this medication in Jordanian children with thalassemia major. In this descriptive cross-sectional study, seventy three patients were selected conveniently to complete the study instruments. Participants reported high adherence to their chelation therapy (88.1%). The majority (87.6%) expressed necessity for chelation therapy. However, 42.18% of the participants expressed their concerns about the therapy in which 57.9% of them showed concerns about dependency on medicine and 46.4% of them revealed concerns about the long-term effect of medicine. Overall, about 12.9% of the participants recorded correct and complete answers about thalassemia and its treatment. Knowledge about thalassemia and concerns about chelation were not significantly associated with adherence to the chelation therapy (r = 0.32, p = 0.19;r = 0.29, p = 0.25, respectively). However, there was a significant positive relationship between beliefs about the necessity of the therapy and adherence to it (r = 0.38, p = 0.03). In conclusion, Jordanian children showed proper adherence to their chelation therapy. Health care professionals should discuss patients’ concerns about chelation therapy which might increase patients’ adherence to their therapy.展开更多
文摘Iron is essential for all organisms including microbial,cancer and human cells. More than a quarter of the human population is affected by abnormalities of iron metabolism, mainly from iron deficiency and iron overload. Iron also plays an important role in free radical pathology and oxidative damage which is observed in almost all major diseases, cancer and ageing. New developments include the complete treatment of iron overload and reduction of morbidity and mortality in thalassaemia using deferiprone and selected deferiprone/deferoxamine combinations and also the use of the maltol iron complex in the treatment of iron deficiency anaemia. There is also a prospect of using deferiprone as a universal antioxidant in non iron overloaded diseases such as neurodegenerative, cardiovascular, renal, infectious diseases and cancer. New regulatory molecules of iron metabolism such as endogenous and dietary chelating molecules, hepcidin, mitochondrial ferritin and their role in health and disease is under evaluation. Similarly, new mechanisms of iron deposition, removal, distribution and toxicity have been identified using new techniques such as magnetic resonance imaging increasing our understanding of iron metabolic processes and the targeted treatment of related diseases. The uniform distribution of iron in iron overload between organs and within each organ is no longer valid. Several other controversies such as the toxicity impact of non transferrin bound iron vs injected iron, the excess levels of iron in tissues causing toxicity and the role of chelation on iron absorption need further investigation. Commercial interests of pharmaceutical companies and connections to leading journals are playing a crucial role in shaping worldwide medical opinion on drug sales and use but also patients' therapeutic outcome and safety. Major controversies include the selection criteria and risk/benefit assessment in the use of deferasirox in thalassaemia and more so in idiopathic haemochromatosis, thalassaemia intermedia and ex-thalass
文摘本研究旨在探讨骨髓增生异常综合征(MDS)患者袪铁治疗后红细胞生成素(EPO)、血红蛋白(Hb)、重组EPO(rEPO)的变化及EPO与血清铁蛋白(SF)的相关性。检测172例MDS患者及30例健康对照者SF、EPO浓度、血清铁(SI)、总铁结合力(TIBC)、C反应蛋白(CRP)、Hb;根据CRP值对SF进行调整。对34例低危组、SF>1 000 mg/L的患者给予袪铁胺治疗,比较治疗前后SF、EPO、SI、TIBC、Hb的变化。对58例低危组、EPO<1 000 U/L的患者给予rEPO治疗,比较铁过载组与非铁过载组袪铁治疗前后EPO抵抗的发生率。结果表明:非铁过载组EPO浓度高于正常对照组(997.44±473.48 vs 467.27±238.49)(P<0.05);铁过载组EPO浓度高于无铁过载组及正常对照组(3257.59±697.19 vs 997.44±473.48;3257.59±697.19 vs 467.27±238.49)(P均<0.05);铁过载组EPO抵抗发生率高于非铁过载组(18/35 vs 2/23)(P=0.001),铁过载组MDS患者EPO与SF呈显著正相关(r=0.310)(P=0.036)。袪铁治疗后SF、SI、TIBC及EPO浓度均较治疗前明显下降(3942.38±641.82 vs 2266.35±367.31;48.61±10.65 vs 28.52±12.61;59.84±12.62 vs 33.76±15.43;3808.01±750.22 vs 1954.78±473.18)(P均<0.05),Hb升高(35±18 vs 57±21)(P=0.046),部分EPO抵抗患者恢复疗效。结论:袪铁治疗能增强贫血MDS患者对EPO反应,缓解EPO抵抗,降低EPO病理性升高,提升Hb水平,减少输血依赖。
文摘Adherence to thalassemia treatment including chelation drugs is influenced by numerous factors. This study aims to explore beliefs about iron chelation therapy and adherence to this medication in Jordanian children with thalassemia major. In this descriptive cross-sectional study, seventy three patients were selected conveniently to complete the study instruments. Participants reported high adherence to their chelation therapy (88.1%). The majority (87.6%) expressed necessity for chelation therapy. However, 42.18% of the participants expressed their concerns about the therapy in which 57.9% of them showed concerns about dependency on medicine and 46.4% of them revealed concerns about the long-term effect of medicine. Overall, about 12.9% of the participants recorded correct and complete answers about thalassemia and its treatment. Knowledge about thalassemia and concerns about chelation were not significantly associated with adherence to the chelation therapy (r = 0.32, p = 0.19;r = 0.29, p = 0.25, respectively). However, there was a significant positive relationship between beliefs about the necessity of the therapy and adherence to it (r = 0.38, p = 0.03). In conclusion, Jordanian children showed proper adherence to their chelation therapy. Health care professionals should discuss patients’ concerns about chelation therapy which might increase patients’ adherence to their therapy.
