Suppr超能文献

血友病的预防性治疗方面。

Aspects of prophylactic treatment of hemophilia.

作者信息

Ljung Rolf

机构信息

Department of Clinical Sciences Lund-Paediatrics, Lund University, Lund, Sweden ; Department of Paediatrics and Malmö Centre for Thrombosis and Haemostasis, Skåne University Hospital, Malmö, Sweden.

出版信息

Thromb J. 2016 Oct 4;14(Suppl 1):30. doi: 10.1186/s12959-016-0103-3. eCollection 2016.

Abstract

Retrospective and prospective studies unambiguously show that prophylactic treatment of severe hemophilia A or B should be started as primary prophylaxis at 1-2 years' of age and ideally before the first joint bleed. The dose and dose frequency should be individually tailored depending on the goal of treatment, venous access and the bleeding phenotype. The circumstances during the first exposures of factor VIII concentrates in hemophilia A may have an impact on the risk of developing inhibitors. Enhanced half-life products, in particular in hemophilia B, will facilitate treatment in patients with difficult venous access but also in achieving a higher trough level. Evidence accumulate that prophylactic treatment is beneficial also in adults and in patients with inhibitors.

摘要

回顾性和前瞻性研究明确表明,重度甲型或乙型血友病的预防性治疗应在1至2岁时作为初级预防开始,理想情况是在首次关节出血之前。剂量和给药频率应根据治疗目标、静脉通路和出血表型进行个体化调整。甲型血友病患者首次接触凝血因子VIII浓缩物时的情况可能会对产生抑制剂的风险产生影响。半衰期延长的产品,特别是在乙型血友病中,将有助于静脉通路困难的患者进行治疗,也有助于达到更高的谷浓度。越来越多的证据表明,预防性治疗对成人和有抑制剂的患者也有益。

相似文献

1
Aspects of prophylactic treatment of hemophilia.
Thromb J. 2016 Oct 4;14(Suppl 1):30. doi: 10.1186/s12959-016-0103-3. eCollection 2016.
2
The current status of prophylactic replacement therapy in children and adults with haemophilia.
Br J Haematol. 2015 Jun;169(6):777-86. doi: 10.1111/bjh.13365. Epub 2015 Mar 29.
3
Clotting factor concentrates given to prevent bleeding and bleeding-related complications in people with hemophilia A or B.
Cochrane Database Syst Rev. 2006 Apr 19(2):CD003429. doi: 10.1002/14651858.CD003429.pub3.
4
Clotting factor concentrates given to prevent bleeding and bleeding-related complications in people with hemophilia A or B.
Cochrane Database Syst Rev. 2005 Apr 18(2):CD003429. doi: 10.1002/14651858.CD003429.pub2.
5
Primary prophylaxis in haemophilia care: Guideline update 2016.
Blood Cells Mol Dis. 2017 Sep;67:81-85. doi: 10.1016/j.bcmd.2017.02.004. Epub 2017 Feb 17.
6
Clotting factor concentrates given to prevent bleeding and bleeding-related complications in people with hemophilia A or B.
Cochrane Database Syst Rev. 2011 Sep 7(9):CD003429. doi: 10.1002/14651858.CD003429.pub4.
7
Prophylactic versus on-demand treatments for hemophilia: advantages and drawbacks.
Expert Rev Hematol. 2018 Jul;11(7):567-576. doi: 10.1080/17474086.2018.1486704. Epub 2018 Jun 19.
8
Prophylaxis in children with hemophilia: evidence-based achievements, old and new challenges.
Semin Thromb Hemost. 2012 Feb;38(1):79-94. doi: 10.1055/s-0031-1300954. Epub 2012 Feb 7.
9
When should prophylactic treatment in patients with haemophilia A and B start?--The German experience.
Haemophilia. 1998 Jul;4(4):413-7. doi: 10.1046/j.1365-2516.1998.440413.x.

引用本文的文献

3
Management of haemophilia A with inhibitors: A regional cross-talk.
Haemophilia. 2022 Nov;28(6):950-961. doi: 10.1111/hae.14638. Epub 2022 Jul 22.
5
Novel blood coagulation molecules: Skeletal muscle myosin and cardiac myosin.
J Thromb Haemost. 2021 Jan;19(1):7-19. doi: 10.1111/jth.15097. Epub 2020 Oct 25.
8
Extended Half-Life Coagulation Factors: A New Era in the Management of Hemophilia Patients.
Turk J Haematol. 2019 Aug 2;36(3):141-154. doi: 10.4274/tjh.galenos.2019.2018.0393. Epub 2019 May 15.
10
Extended Half-Life Factor VIII and Factor IX Preparations.
Transfus Med Hemother. 2018 Apr;45(2):86-91. doi: 10.1159/000488060. Epub 2018 Mar 21.

本文引用的文献

1
Extended half-life clotting factor concentrates: results from published clinical trials.
Haemophilia. 2016 Jul;22 Suppl 5:25-30. doi: 10.1111/hae.13028.
2
Spanish Consensus Guidelines on prophylaxis with bypassing agents in patients with haemophilia and inhibitors.
Thromb Haemost. 2016 May 2;115(5):872-95. doi: 10.1160/TH15-07-0568. Epub 2016 Feb 4.
3
Long-acting recombinant coagulation factor IX albumin fusion protein (rIX-FP) in hemophilia B: results of a phase 3 trial.
Blood. 2016 Apr 7;127(14):1761-9. doi: 10.1182/blood-2015-09-669234. Epub 2016 Jan 11.
4
The current status of prophylactic replacement therapy in children and adults with haemophilia.
Br J Haematol. 2015 Jun;169(6):777-86. doi: 10.1111/bjh.13365. Epub 2015 Mar 29.
5
Recombinant long-acting glycoPEGylated factor IX in hemophilia B: a multinational randomized phase 3 trial.
Blood. 2014 Dec 18;124(26):3880-6. doi: 10.1182/blood-2014-05-573055. Epub 2014 Sep 26.
7
Definitions in hemophilia: communication from the SSC of the ISTH.
J Thromb Haemost. 2014 Nov;12(11):1935-9. doi: 10.1111/jth.12672. Epub 2014 Sep 3.
8
The European standards of Haemophilia Centres.
Blood Transfus. 2014 Apr;12 Suppl 3(Suppl 3):s525-30. doi: 10.2450/2014.0056-14s.
9
Similar bleeding phenotype in young children with haemophilia A or B: a cohort study.
Haemophilia. 2014 Nov;20(6):747-55. doi: 10.1111/hae.12470. Epub 2014 Jun 3.
10
Novel products for haemostasis - current status.
Haemophilia. 2014 May;20 Suppl 4:23-8. doi: 10.1111/hae.12428.

文献AI研究员

20分钟写一篇综述,助力文献阅读效率提升50倍。

立即体验

用中文搜PubMed

大模型驱动的PubMed中文搜索引擎

马上搜索

文档翻译

学术文献翻译模型,支持多种主流文档格式。

立即体验