去铁酮联合去铁胺治疗重症地中海贫血患儿疗效观察
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摘要
目的探讨去铁酮联合去铁胺治疗重症地中海贫血患儿的临床疗效。方法选择2013年7月到2017年12月期间中山市博爱医院儿科收治的60例重型地中海贫血患儿为研究对象,根据随机数表法将患儿分为观察组与对照组,每组30例,对照组予单独应用去铁胺治疗,观察组则联合应用去铁酮和去铁胺治疗,疗程12个月。比较两组患儿治疗后的临床疗效以及治疗前后不同时间患儿血清铁蛋白(SF)水平的变化。结果观察组患儿的治疗总反应率为83.33%,明显高于对照组的63.33%,差异有统计学意义(P<0.05);观察组患儿治疗后6个月、12个月血清SF水平分别为(2 260.7±204.8)μg/L、(1 986.6±215.8)μg/L,均明显低于对照组的(2 398.3±172.3)μg/L、(2 127.4±233.9)μg/L,差异均有统计学意义(P<0.05)。结论去铁酮联合去铁胺治疗重症地中海贫血患儿的去铁效果优于单用去铁胺,其可以尽快降低患儿的铁负荷,提高治疗反应率,且无明显不良反应。
Objective To investigate the clinical efficacy of deferiprone combined with desferrioxamine in the treatment of children with severe thalassemia. Methods A total of 60 cases of children with severe thalassemia, who admitted to Department of Pediatrics of Zhongshan Humanity Hospital from July 2013 to December 2017, were selected and divided into the observation group and the control group according to random number table method, with 30 cases in each group. The control group was treated with desferrioxamine alone, while the observation group was treated with deferiprone combined with desferrioxamine for 12 months. The curative effect after treatment and the changes of serum ferritin(SF) levels in children before and after treatment at different time in the two groups were compared. Results The total response rate of the observation group was 83.33%, which was significantly higher than 63.33% of the control group(P<0.05); the serum SF of the observation group was(2 260.7±204.8) μg/L and(1 986.6±215.8) μg/L at 6 and 12 months after treatment, respectively, which were significantly lower than corresponding(2 398.3±172.3) μg/L and(2 127.4±233.9) μg/L of the control group(P<0.05). Conclusion The effect of deferiprone combined with desferrioxamine in the treatment of children with severe thalassemia is better than that of desferrioxamine alone. It can reduce the iron load of children as soon as possible and improve the response rate of treatment without obvious adverse reactions.
Objective To investigate the clinical efficacy of deferiprone combined with desferrioxamine in the treatment of children with severe thalassemia. Methods A total of 60 cases of children with severe thalassemia, who admitted to Department of Pediatrics of Zhongshan Humanity Hospital from July 2013 to December 2017, were selected and divided into the observation group and the control group according to random number table method, with 30 cases in each group. The control group was treated with desferrioxamine alone, while the observation group was treated with deferiprone combined with desferrioxamine for 12 months. The curative effect after treatment and the changes of serum ferritin(SF) levels in children before and after treatment at different time in the two groups were compared. Results The total response rate of the observation group was 83.33%, which was significantly higher than 63.33% of the control group(P<0.05); the serum SF of the observation group was(2 260.7±204.8) μg/L and(1 986.6±215.8) μg/L at 6 and 12 months after treatment, respectively, which were significantly lower than corresponding(2 398.3±172.3) μg/L and(2 127.4±233.9) μg/L of the control group(P<0.05). Conclusion The effect of deferiprone combined with desferrioxamine in the treatment of children with severe thalassemia is better than that of desferrioxamine alone. It can reduce the iron load of children as soon as possible and improve the response rate of treatment without obvious adverse reactions.
引文
[1]谭建强,潘莉珍,陆碧玉,等.柳州市中重度地中海贫血患儿出生原因调查[J].中国妇幼保健, 2017, 32(1):135-138.
[2] RAVANGARD R, MIRZAEI Z, KESHAVARZ K, et al. Blood transfusion versus hydroxyurea in beta-thalassemia in Iran:a cost-effectiveness study[J]. Hematology, 2018, 23(7):417-422.
[3]黄春生,陈国华.去铁胺治疗重型β-地中海贫血患儿的疗效及不良反应分析[J].中国妇幼健康研究, 2016, 27(6):726-728.
[4] WAHIDIYAT PA, YOSIA M, SARI TT. Comparison of deferiprone to deferasirox and deferoxamine to cardiac and hepatic T2 MRI in thalassemia patients:evidence-based case report[J]. Acta Med Indones, 2018, 50(2):168-176.
[5]中华医学会儿科学分会血液学组,《中华儿科杂志》编辑委员会.重型β地中海贫血的诊断和治疗指南[J].中华儿科杂志, 2010, 48(3):186-189.
[6]许燕,夏苏建,张思恒,等.铁螯合剂治疗重型β-地中海贫血疗效的Meta分析[J].中国药学杂志, 2013, 48(21):1875-1880.
[7] SHAW J, CHAKRABORTY A, NAG A, et al. Intracellular iron overload leading to DNA damage of lymphocytes and immune dysfunction in thalassemia major patients[J]. Eur J Haematol, 2017, 99(5):399-408.
[8]简咏芬,周少雄,潘永红,等.网织红细胞参数用于鉴别诊断地中海贫血与缺铁性贫血的价值[J].医学检验与临床, 2016, 27(2):64-65, 86.
[9]陈娟娟,陈光福,高红英.不同铁螯合剂治疗重型β地中海贫血铁过载的临床研究[J].中国小儿血液与肿瘤杂志, 2017, 22(4):185-189,222.
[10] ALLALI S, DE MONTALEMBERT M, BROUSSE V, et al. Management of iron overload in hemoglobinopathies[J]. Transfus Clin Biol,2017, 24(3):223-226.
[11]张旸.输血和去铁治疗对儿童重型地中海贫血心功能的影响[J].包头医学院学报, 2018, 34(1):26-28.
[12]吴坤芳,蔡玉玲.地中海贫血检测结果分析[J].海南医学, 2013, 24(17):2555-2556.
[13]任伟,姚洁,陈金美,等.规律去铁胺治疗输血依赖性铁过载的临床分析[J].四川医学, 2016, 37(11):1261-1263.
[14]许燕,夏苏建,张思恒,等.铁螯合剂治疗重型β-地中海贫血疗效的Meta分析[J].中国药学杂志, 2013, 48(21):1875-1880.
[15]陈爱华,赵国阳,庄琴,等.去铁胺对输血相关性铁过载患者骨密度的影响[J].中国骨质疏松杂志, 2018, 24(3):311-314.
[16] TOTADRI S, BANSAL D, TREHAN A, et al. Hepatic and cardiac iron-load in children on long-term chelation with deferiprone for thalassemia major[J]. Indian Pediatr, 2018, 55(7):573-575.
[17]张进峰,黄海波.去铁酮治疗高量输血的重型β-地中海贫血患儿临床观察[J].北方药学, 2016, 13(9):196.
[18] ALYMARA V, BOURANTAS D, CHAIDOS A, et al. Effectiveness and safety of combined iron-chelation therapy with deferoxamine and deferiprone[J]. Hematol J, 2004, 5(6):475-479.
[2] RAVANGARD R, MIRZAEI Z, KESHAVARZ K, et al. Blood transfusion versus hydroxyurea in beta-thalassemia in Iran:a cost-effectiveness study[J]. Hematology, 2018, 23(7):417-422.
[3]黄春生,陈国华.去铁胺治疗重型β-地中海贫血患儿的疗效及不良反应分析[J].中国妇幼健康研究, 2016, 27(6):726-728.
[4] WAHIDIYAT PA, YOSIA M, SARI TT. Comparison of deferiprone to deferasirox and deferoxamine to cardiac and hepatic T2 MRI in thalassemia patients:evidence-based case report[J]. Acta Med Indones, 2018, 50(2):168-176.
[5]中华医学会儿科学分会血液学组,《中华儿科杂志》编辑委员会.重型β地中海贫血的诊断和治疗指南[J].中华儿科杂志, 2010, 48(3):186-189.
[6]许燕,夏苏建,张思恒,等.铁螯合剂治疗重型β-地中海贫血疗效的Meta分析[J].中国药学杂志, 2013, 48(21):1875-1880.
[7] SHAW J, CHAKRABORTY A, NAG A, et al. Intracellular iron overload leading to DNA damage of lymphocytes and immune dysfunction in thalassemia major patients[J]. Eur J Haematol, 2017, 99(5):399-408.
[8]简咏芬,周少雄,潘永红,等.网织红细胞参数用于鉴别诊断地中海贫血与缺铁性贫血的价值[J].医学检验与临床, 2016, 27(2):64-65, 86.
[9]陈娟娟,陈光福,高红英.不同铁螯合剂治疗重型β地中海贫血铁过载的临床研究[J].中国小儿血液与肿瘤杂志, 2017, 22(4):185-189,222.
[10] ALLALI S, DE MONTALEMBERT M, BROUSSE V, et al. Management of iron overload in hemoglobinopathies[J]. Transfus Clin Biol,2017, 24(3):223-226.
[11]张旸.输血和去铁治疗对儿童重型地中海贫血心功能的影响[J].包头医学院学报, 2018, 34(1):26-28.
[12]吴坤芳,蔡玉玲.地中海贫血检测结果分析[J].海南医学, 2013, 24(17):2555-2556.
[13]任伟,姚洁,陈金美,等.规律去铁胺治疗输血依赖性铁过载的临床分析[J].四川医学, 2016, 37(11):1261-1263.
[14]许燕,夏苏建,张思恒,等.铁螯合剂治疗重型β-地中海贫血疗效的Meta分析[J].中国药学杂志, 2013, 48(21):1875-1880.
[15]陈爱华,赵国阳,庄琴,等.去铁胺对输血相关性铁过载患者骨密度的影响[J].中国骨质疏松杂志, 2018, 24(3):311-314.
[16] TOTADRI S, BANSAL D, TREHAN A, et al. Hepatic and cardiac iron-load in children on long-term chelation with deferiprone for thalassemia major[J]. Indian Pediatr, 2018, 55(7):573-575.
[17]张进峰,黄海波.去铁酮治疗高量输血的重型β-地中海贫血患儿临床观察[J].北方药学, 2016, 13(9):196.
[18] ALYMARA V, BOURANTAS D, CHAIDOS A, et al. Effectiveness and safety of combined iron-chelation therapy with deferoxamine and deferiprone[J]. Hematol J, 2004, 5(6):475-479.