口服deferiprone患有地中海贫血的铁螯合。

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罗伯茨DJ, Brunskill SJ, Doree C,威廉姆斯年代,海德CJ霍华德·J

口服deferiprone患有地中海贫血的铁螯合。

科克伦数据库系统启2007年7月18日;(3):CD004839。

PubMed ID
17636775 (在PubMed
]
文摘

背景:地中海贫血主要是一种遗传疾病的特点是减少产生血红蛋白的能力。造成贫血的管理是通过红细胞输血。重复输血导致过度积累在体内的铁(铁过载),去除是通过铁螯合疗法。常用的铁螯合剂,deferiprone,发现药物有效。然而,重要的问题存在的疗效和安全性deferiprone相比另一个铁螯beplayapp合剂,desferrioxamine。目的:总结试验数据deferiprone和比较的临床疗效和安全性的临床疗效和安全性deferiprone与desferrioxamine地中海贫血。搜索策略:我们搜查了该集团的血红蛋白病试验注册、MEDLINE、EMBASE,生物提取,ZETOC、当前对照试验和相关出版物的书目。我们联系了厂家deferiprone desferrioxamine。最近搜索:2006年6月。选择标准:随机对照试验比较deferiprone和另一个铁螯合剂; or comparing two schedules of deferiprone, in people with transfusion-dependent thalassaemia. DATA COLLECTION AND ANALYSIS: Two authors independently assessed trial quality and extracted data. Missing data were requested from the original investigators. MAIN RESULTS: Ten trials involving 398 people (range 10 to 144 people) were included. Nine trials compared deferiprone with desferrioxamine or a combination of deferiprone and desferrioxamine and one compared different schedules of deferiprone. There was little consistency between outcomes and little information to fully assess the methodological quality of most of the included trials. No trial reported long-term outcomes (mortality and end organ damage). There was no consistent effect on reduction of iron overload between all treatment comparisons, with the exception of urinary iron excretion in comparisons of deferiprone with desferrioxamine. An increase in iron excretion levels favoured deferiprone in one trial and desferrioxamine in three trials, even though measurement of urinary iron excretion underestimates total iron excretion by desferrioxamine.Adverse events were recorded in trials comparing deferiprone with desferrioxamine. There was evidence of adverse events in all treatment groups. Adverse events in one trial were significantly more likely with deferiprone than desferrioxamine, relative risk 2.24 (95% confidence interval 1.19 to 4.23). AUTHORS' CONCLUSIONS: We found no reason to change current treatment recommendations, namely deferiprone is indicated for treating iron overload in people with thalassaemia major when desferrioxamine is contraindicated or inadequate. However, there is an urgent need for adequately-powered, high quality trials comparing the overall clinical efficacy and long-term outcome of deferiprone with desferrioxamine.

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