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本文引用的文献

1
Episodic replacement of clotting factor concentrates does not prevent bleeding or musculoskeletal damage - the MUSFIH study.凝血因子浓缩物的间歇性替代并不能预防出血或肌肉骨骼损伤——MUSFIH研究。
Haemophilia. 2017 Jul;23(4):538-546. doi: 10.1111/hae.13242. Epub 2017 Jun 2.
2
Low-dose prophylaxis for children with haemophilia in a resource-limited setting in south India-A clinical audit report.印度南部资源有限环境下血友病儿童的低剂量预防——一份临床审计报告
Haemophilia. 2017 Jul;23(4):e382-e384. doi: 10.1111/hae.13272. Epub 2017 May 24.
3
Iranian Low-dose Escalating Prophylaxis Regimen in Children with Severe Hemophilia A and B.伊朗针对重度甲型和乙型血友病儿童的低剂量递增预防方案。
Clin Appl Thromb Hemost. 2018 Apr;24(3):513-518. doi: 10.1177/1076029616685429. Epub 2017 Jan 4.
4
Low dose prophylaxis in Tunisian children with haemophilia.突尼斯血友病儿童的低剂量预防治疗
Haemophilia. 2017 Jan;23(1):77-81. doi: 10.1111/hae.13048. Epub 2016 Dec 11.
5
Individualized prophylaxis for optimizing hemophilia care: can we apply this to both developed and developing nations?优化血友病护理的个体化预防措施:我们能否将其应用于发达国家和发展中国家?
Thromb J. 2016 Oct 4;14(Suppl 1):32. doi: 10.1186/s12959-016-0096-y. eCollection 2016.
6
Haemophilia: a global challenge.
Haemophilia. 1995 Jan;1(1):11-3. doi: 10.1111/j.1365-2516.1995.tb00033.x.
7
A randomized study of very low-dose factor VIII prophylaxis in severe haemophilia - A success story from a resource limited country.一项关于极低剂量凝血因子 VIII 预防治疗重度甲型血友病的随机研究——来自资源有限国家的成功案例
Haemophilia. 2016 May;22(3):342-8. doi: 10.1111/hae.12838. Epub 2016 Mar 14.
8
Emerging and future therapies for hemophilia.血友病的新兴及未来疗法
J Blood Med. 2015 Sep 3;6:245-55. doi: 10.2147/JBM.S42669. eCollection 2015.
9
Benefits of prophylaxis versus on-demand treatment in adolescents and adults with severe haemophilia A: the POTTER study.预防治疗与按需治疗在重度 A 型血友病青少年和成人患者中的获益:POTTER 研究。
Thromb Haemost. 2015 Jul;114(1):35-45. doi: 10.1160/TH14-05-0407. Epub 2015 Apr 9.
10
Intermediate-dose versus high-dose prophylaxis for severe hemophilia: comparing outcome and costs since the 1970s.中剂量与高剂量预防重度血友病:自 20 世纪 70 年代以来的结果和成本比较。
Blood. 2013 Aug 15;122(7):1129-36. doi: 10.1182/blood-2012-12-470898. Epub 2013 Jun 18.

血友病治疗中的低剂量预防

Low Dose Prophylaxis in Hemophilia Care.

作者信息

Sidharthan Neeraj, Sudevan Remya

机构信息

1Department of Haemato Oncology, Amrita Institute of Medical Sciences, Kochi, India.

2Department of Health Sciences Research, Amrita Institute of Medical Sciences, Kochi, India.

出版信息

Indian J Hematol Blood Transfus. 2020 Jan;36(1):16-25. doi: 10.1007/s12288-019-01147-0. Epub 2019 Jun 15.

DOI:10.1007/s12288-019-01147-0
PMID:32158085
原文链接:https://pmc.ncbi.nlm.nih.gov/articles/PMC7042432/
Abstract

Hemophilia is an inherited bleeding disorder which causes impaired blood clotting. The severity of disease depends on the type of Hemophilia, level of clotting factor concentrate (CFC), phenotypic heterogeneity and the development of inhibitors. The currently accepted standard of care of this disease is prophylaxis therapy (PT) with CFC. Prophylaxis therapy for Hemophilia is given in developed countries for the last few decades. On the contrary, episodic therapy (ET) is still the mode of treatment in middle and low income countries. ET is documented to have several potential risks such as increased bleeding rate, disability due to haemarthrosis, poor quality of life and increased chances of mortality. Several studies conducted in developed countries have confirmed the clinical efficacy of PT in Hemophilia treatment. Currently, there exist several challenges for implementation of this effective treatment in resource poor nations. Low dose prophylaxis (LDP) has been developed as a solution to minimize these challenges and to provide better care for subjects with Hemophilia from low resource countries. The impact of LDP was evaluated by several recent studies and the reported clinical outcomes seem to suggest an optimistic future for this line of therapy. Several themes related to Hemophilia care like inhibitor development, tolerance, pharmacokinetics of CFCs and cost-benefit analysis of different prophylaxis regimens are currently understood poorly. These distinct elements are highly relevant to assess the actual benefits of LDP regimen in a global scale.

摘要

血友病是一种遗传性出血性疾病,会导致血液凝固功能受损。疾病的严重程度取决于血友病的类型、凝血因子浓缩物(CFC)水平、表型异质性以及抑制剂的产生情况。目前公认的这种疾病的治疗标准是使用CFC进行预防性治疗(PT)。在过去几十年里,发达国家一直在对血友病进行预防性治疗。相反,在中低收入国家,按需治疗(ET)仍然是主要的治疗方式。据记载,按需治疗存在一些潜在风险,如出血率增加、因关节积血导致残疾、生活质量差以及死亡几率增加。发达国家进行的多项研究证实了预防性治疗在血友病治疗中的临床疗效。目前,在资源匮乏的国家实施这种有效治疗存在诸多挑战。低剂量预防性治疗(LDP)已被开发出来,以尽量减少这些挑战,并为来自资源匮乏国家的血友病患者提供更好的治疗。最近的几项研究评估了低剂量预防性治疗的效果,报告的临床结果似乎表明这种治疗方法前景乐观。目前,人们对与血友病治疗相关的几个主题,如抑制剂的产生、耐受性、CFC的药代动力学以及不同预防性治疗方案的成本效益分析了解甚少。这些不同的因素对于在全球范围内评估低剂量预防性治疗方案的实际益处高度相关。