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甲型血友病的每周两次预防性治疗。

Twice weekly prophylactic therapy in haemophilia A.

作者信息

Aronstam A, Kirk P J, McHardy J, Culver-James J W, McLellan D S, Turk P, Rainsford S G, Slattery M

出版信息

J Clin Pathol. 1977 Jan;30(1):65-7. doi: 10.1136/jcp.30.1.65.

DOI:10.1136/jcp.30.1.65
PMID:320230
原文链接:https://pmc.ncbi.nlm.nih.gov/articles/PMC476643/
Abstract

Factor VIII-containing materials were administered to four severely affected haemophiliacs twice weekly in doses calculated to raise the factor VIII level to either 15% or 30% of average normal. The pooled results from those patients with statistically similar baseline bleeding frequencies showed a significant reduction in bleeding frequency on both doses in the first 48 hours. The 30% dose produced a more significant reduction than the 15% dose in the first 24 hours, but there was no significant difference between the two doses in the second 24 hours. It appears that to reduce the bleeding frequency of severely affected haemophiliacs by 60% would require a two-and-a-half-fold increase in therapeutic materials. A 90% reduction would need nine times the amount of material currently in use.

摘要

给四名重症血友病患者每周两次注射含凝血因子VIII的材料,剂量按能将凝血因子VIII水平提高到平均正常值的15%或30%来计算。那些基线出血频率在统计学上相似的患者的汇总结果显示,在最初48小时内,两种剂量的出血频率均显著降低。在最初24小时内,30%剂量组的出血频率降低幅度比15%剂量组更显著,但在第二个24小时内,两组剂量之间无显著差异。似乎要将重症血友病患者的出血频率降低60%,治疗材料需要增加2.5倍。要将出血频率降低90%,所需材料量将是目前使用量的9倍。

相似文献

1
Twice weekly prophylactic therapy in haemophilia A.甲型血友病的每周两次预防性治疗。
J Clin Pathol. 1977 Jan;30(1):65-7. doi: 10.1136/jcp.30.1.65.
2
Prophylaxis in haemophilia: a double-blind controlled trial.血友病的预防:一项双盲对照试验。
Br J Haematol. 1976 May;33(1):81-90. doi: 10.1111/j.1365-2141.1976.tb00973.x.
3
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4
Role of enhanced half-life factor VIII and IX in the treatment of haemophilia.增强半衰期因子 VIII 和 IX 在血友病治疗中的作用。
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Long-term high dose factor VIII treatment of 3 haemophiliacs with factor VIII inhibitor.对3名患有因子VIII抑制剂的血友病患者进行长期高剂量因子VIII治疗。
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Elucidation of Factor VIII Activity Pharmacokinetics: A Pooled Population Analysis in Patients With Hemophilia A Treated With Moroctocog Alfa.凝血因子VIII活性药代动力学的阐明:对接受莫罗凝血因子α治疗的甲型血友病患者的汇总群体分析。
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Break-through bleeding in relation to pharmacokinetics of Factor VIII in paediatric patients with severe haemophilia A.突破性出血与儿科重度 A 型血友病患者因子 VIII 药代动力学的关系。
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引用本文的文献

1
Clotting factor concentrates for preventing bleeding and bleeding-related complications in previously treated individuals with haemophilia A or B.用于预防既往接受过治疗的 A 型或 B 型血友病患者出血和出血相关并发症的凝血因子浓缩物。
Cochrane Database Syst Rev. 2021 Aug 18;8(8):CD014201. doi: 10.1002/14651858.CD014201.
2
Achieving the unimaginable: Health equity in haemophilia.实现难以想象的目标:血友病中的健康公平。
Haemophilia. 2020 Jan;26(1):17-24. doi: 10.1111/hae.13862. Epub 2019 Nov 13.
3
Review of antihemophilic factor injection for the routine prophylaxis of bleeding episodes and risk of joint damage in severe hemophilia A.抗血友病因子注射剂用于重度甲型血友病出血发作的常规预防及关节损伤风险的综述。
Vasc Health Risk Manag. 2010 Mar 3;6:59-68. doi: 10.2147/vhrm.s5217.
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Primary prophylaxis in children with haemophilia.血友病患儿的初级预防。
Blood Transfus. 2008 Sep;6 Suppl 2(Suppl 2):s4-11. doi: 10.2450/2008.0030-08.
5
Hemophilia treatment in historical perspective: a review of medical and social developments.血友病治疗的历史回顾:医学与社会发展综述
Ann Hematol. 1991 Feb;62(1):5-15. doi: 10.1007/BF01714977.
6
Transfusion requirements of adolescents with severe haemophilia A.重度甲型血友病青少年的输血需求。
J Clin Pathol. 1979 Sep;32(9):927-30. doi: 10.1136/jcp.32.9.927.
7
[The role of self-infusion treatment for the patient with haemophilia (author's transl)].
Blut. 1978 Feb;36(2):63-71. doi: 10.1007/BF00996832.

本文引用的文献

1
Prophylactic therapy in classical hemophilia: a preliminary report.经典血友病的预防性治疗:初步报告。
Can Med Assoc J. 1967 Sep 9;97(11):559-61.
2
Our experience in Sweden with prophylaxis on haemophilia.
Bibl Haematol. 1970;34:111-24.
3
A trial of prophylactic replacement therapy in haemophilia and Christmas disease.血友病和克里斯马斯病预防性替代疗法的试验。
J Clin Pathol. 1973 Apr;26(4):243-7. doi: 10.1136/jcp.26.4.243.
4
A three-year study of adolescent boys suffering from haemophilia and allied disorders.一项针对患有血友病及相关疾病的青春期男孩的为期三年的研究。
Br J Haematol. 1973 May;24(5):539-51. doi: 10.1111/j.1365-2141.1973.tb01680.x.
5
Prophylaxis in haemophilia: a double-blind controlled trial.血友病的预防:一项双盲对照试验。
Br J Haematol. 1976 May;33(1):81-90. doi: 10.1111/j.1365-2141.1976.tb00973.x.