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多次输血的地中海贫血患者中去铁胺治疗依从性的自我报告水平及影响因素

Self-reported level of and factors influencing the compliance to desferrioxamine therapy in multitransfused thalassaemias.

作者信息

Lee Way Seah, Toh Tsun How, Chai Pei Fan, Soo Thian Lian

机构信息

Department of Paediatrics, Faculty of Medicine, University of Malaya, Kuala Lumpur, Malaysia.

出版信息

J Paediatr Child Health. 2011 Aug;47(8):535-40. doi: 10.1111/j.1440-1754.2011.02017.x. Epub 2011 Mar 10.

Abstract

AIM

To analyse the self-reported degree of and factors influencing the compliance to desferrioxamine (DFO) therapy in children with transfusion-dependent thalassaemia major in Malaysia.

METHODS

A cross-sectional study was conducted on all children with thalassaemia major on DFO attending Likas Hospital, Sabah, in September 2008. Patients or carer-providers were interviewed to report on the degree of compliance as either highly (administering >90% of DFO), moderately (51-90%), poorly (1%-50%) or non-compliant (0%) to DFO in the preceding month. The latest serum ferritin levels were noted.

RESULTS

The median (range) age at first blood transfusion of these 139 (73 males, median (range) age at interview: 9.0 years (2-16 years)) patients were 1.0 year (2 months to 10 years). The median (range) duration of regular DFO therapy was 2 years (2 months to 10 years). Forty-three (31%) of the patients reported themselves to be highly compliant, 70 (50%) moderately and 26 (19%) poorly or non-compliant. Multivariate analysis showed that a lower family income negatively affected the degree of compliance, while family support positively affected the degree of compliance to DFO. No correlation existed between self-reported degree of compliance and latest serum ferritin level (6444 ± µmol/L; P = 0.186).

CONCLUSION

The self-reported compliance to DFO therapy was moderate in this cohort of patients with thalassaemia major in Malaysia. The serum ferritin level was high, possibly because of the relatively short duration of DFO therapy and compliance. Oral iron chelator should be considered to improve the compliance to iron chelation.

摘要

目的

分析马来西亚重型输血依赖型地中海贫血患儿去铁胺(DFO)治疗的自我报告依从程度及影响因素。

方法

2008年9月,对沙巴州利卡斯医院所有接受DFO治疗的重型地中海贫血患儿进行了一项横断面研究。对患者或护理提供者进行访谈,以报告前一个月对DFO的依从程度,分为高度依从(服用>90%的DFO)、中度依从(51%-90%)、低度依从(1%-50%)或不依从(0%)。记录最新的血清铁蛋白水平。

结果

这139名患者(73名男性,访谈时年龄中位数(范围):9.0岁(2-16岁))首次输血时的年龄中位数(范围)为1.0岁(2个月至10岁)。定期DFO治疗的中位数(范围)持续时间为2年(2个月至10年)。43名(31%)患者报告自己高度依从,70名(50%)中度依从,26名(19%)低度依从或不依从。多变量分析显示,家庭收入较低对依从程度有负面影响,而家庭支持对DFO的依从程度有积极影响。自我报告的依从程度与最新血清铁蛋白水平(6444±µmol/L;P = 0.186)之间无相关性。

结论

在马来西亚的这组重型地中海贫血患者中,自我报告的DFO治疗依从性为中度。血清铁蛋白水平较高,可能是由于DFO治疗和依从性的持续时间相对较短。应考虑使用口服铁螯合剂来提高铁螯合的依从性。

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