铁过载的预防:有高质量的证据证明,以恰当剂量定期使用的去铁敏(去铁胺)是一种有效的螯合剂,对于定期输血的β地中海贫血患者,可维持其铁平衡。[36]Brittenham GM, Griffith PM, Nienhuis AW, et al. Efficacy of deferoxamine in preventing complications of iron overload in patients with thalassemia major. N Engl J Med. 1994;331:567-573.http://www.nejm.org/doi/full/10.1056/NEJM199409013310902#t=articlehttp://www.ncbi.nlm.nih.gov/pubmed/8047080?tool=bestpractice.com[37]Fisher SA, Brunskill SJ, Doree C, et al. Desferrioxamine mesylate for managing transfusional iron overload in people with transfusion-dependent thalassaemia. Cochrane Database Syst Rev. 2013;(8):CD004450.http://onlinelibrary.wiley.com/doi/10.1002/14651858.CD004450.pub3/fullhttp://www.ncbi.nlm.nih.gov/pubmed/23963793?tool=bestpractice.com
系统评价或者受试者>200名的随机对照临床试验(RCT)。
铁过载的预防:有高质量的证据证明,以恰当剂量定期使用的去铁敏(去铁胺)是一种有效的螯合剂,对于定期输血的β地中海贫血患者,可维持其铁平衡。[36]Brittenham GM, Griffith PM, Nienhuis AW, et al. Efficacy of deferoxamine in preventing complications of iron overload in patients with thalassemia major. N Engl J Med. 1994;331:567-573.http://www.nejm.org/doi/full/10.1056/NEJM199409013310902#t=articlehttp://www.ncbi.nlm.nih.gov/pubmed/8047080?tool=bestpractice.com[37]Fisher SA, Brunskill SJ, Doree C, et al. Desferrioxamine mesylate for managing transfusional iron overload in people with transfusion-dependent thalassaemia. Cochrane Database Syst Rev. 2013;(8):CD004450.http://onlinelibrary.wiley.com/doi/10.1002/14651858.CD004450.pub3/fullhttp://www.ncbi.nlm.nih.gov/pubmed/23963793?tool=bestpractice.com
铁过载的预防:有高质量的证据证明,地拉罗司是一种有效的螯合剂,在获批的剂量水平上,可维持患者的铁平衡。但是,在铁负荷量很高的患者中,在该剂量水平上并不能实现铁的负平衡,尽管较高的剂量可成功实现这一目标。[38]Cappellini MD, Cohen A, Piga A, et al. A phase 3 study of deferasirox (ICL670), a once-daily oral iron chelator, in patients with beta-thalassemia. Blood. 2006;107:3455-3462.http://www.bloodjournal.org/content/107/9/3455.fullhttp://www.ncbi.nlm.nih.gov/pubmed/16352812?tool=bestpractice.com
系统评价或者受试者>200名的随机对照临床试验(RCT)。
铁过载的预防:有高质量的证据证明,地拉罗司是一种有效的螯合剂,在获批的剂量水平上,可维持患者的铁平衡。但是,在铁负荷量很高的患者中,在该剂量水平上并不能实现铁的负平衡,尽管较高的剂量可成功实现这一目标。[38]Cappellini MD, Cohen A, Piga A, et al. A phase 3 study of deferasirox (ICL670), a once-daily oral iron chelator, in patients with beta-thalassemia. Blood. 2006;107:3455-3462.http://www.bloodjournal.org/content/107/9/3455.fullhttp://www.ncbi.nlm.nih.gov/pubmed/16352812?tool=bestpractice.com
铁过载的预防:有中等质量的证据证明,对于去除定期输血的β地中海贫血患者心脏中的铁而言,与去铁胺相比,去铁酮可能是一种更有效的药物。[43]Pennell DJ, Berdoukas V, Karagiorga M, et al. Randomized controlled trial of deferiprone or deferoxamine in beta-thalassemia major patients with asymptomatic myocardial siderosis. Blood. 2006;107:3738-3744.http://www.bloodjournal.org/content/107/9/3738.longhttp://www.ncbi.nlm.nih.gov/pubmed/16352815?tool=bestpractice.com
系统评价或者受试者>200名的随机对照临床试验(RCT)。
铁过载的预防:有中等质量的证据证明,对于去除定期输血的β地中海贫血患者心脏中的铁而言,与去铁胺相比,去铁酮可能是一种更有效的药物。[43]Pennell DJ, Berdoukas V, Karagiorga M, et al. Randomized controlled trial of deferiprone or deferoxamine in beta-thalassemia major patients with asymptomatic myocardial siderosis. Blood. 2006;107:3738-3744.http://www.bloodjournal.org/content/107/9/3738.longhttp://www.ncbi.nlm.nih.gov/pubmed/16352815?tool=bestpractice.com