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在心脏手术期间和之后,乙型血友病患者采用连续输注因子替代治疗:更好的选择?

Continuous infusion factor replacement in haemophilia B during and after cardiac surgery: the better choice?

机构信息

Internal Medicine, MedStar Georgetown Washington Hospital Center, Washington, DC, USA.

Internal Medicine, MedStar Georgetown Washington Hospital Center, Washington, DC, USA

出版信息

BMJ Case Rep. 2020 Nov 30;13(11):e235859. doi: 10.1136/bcr-2020-235859.

Abstract

A 57-year-old man with mild haemophilia B was admitted for coronary artery bypass graft surgery. His factor IX (FIX) activity was 15% on admission. Our goal was to maintain his FIX activity at 80%-100% for post-op days (PODs) 0-3, and at 60%-80% for PODs 4-14. Preoperatively, the patient was given recombinant FIX (rFIX) bolus using the formula:Dosage needed=%(desired FIX level-current level of FIX)×weight (kg)×1.3.This increased his activity to 100%. One IU of rFIX increased FIX activity by 0.8%; the half-life of rFIX is 18-24 hours. The rFIX infusion was started intraoperatively and continued after surgery to maintain target FIX activity. He was discharged on POD 9 on rFIX bolus dosing of 5000 IU every 12 hours for an additional 5 days. Using continuous factor infusion, we managed to decrease the amount rFIX used by >60% while maintaining steady state FIX activity level.

摘要

一位 57 岁轻度乙型血友病男性患者因冠状动脉旁路移植术入院。他入院时的因子 IX(FIX)活性为 15%。我们的目标是在术后第 0-3 天将他的 FIX 活性维持在 80%-100%,在术后第 4-14 天维持在 60%-80%。术前,根据公式给予患者重组 FIX(rFIX)推注:所需剂量=[(目标 FIX 水平-当前 FIX 水平)×体重(kg)×1.3]%。这将他的活性提高到了 100%。1IU 的 rFIX 可提高 FIX 活性 0.8%;rFIX 的半衰期为 18-24 小时。rFIX 输注在手术期间开始,并在手术后继续以维持目标 FIX 活性。他在术后第 9 天出院,rFIX 推注剂量为 5000IU,每 12 小时一次,持续 5 天。通过持续因子输注,我们成功地将 rFIX 的使用量减少了 60%以上,同时保持了稳定的 FIX 活性水平。

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本文引用的文献

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Recent advances in developing specific therapies for haemophilia.血友病特异性治疗方法的最新进展。
Br J Haematol. 2018 Apr;181(2):161-172. doi: 10.1111/bjh.15084. Epub 2018 Jan 23.
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Guidelines for the management of hemophilia.血友病管理指南。
Haemophilia. 2013 Jan;19(1):e1-47. doi: 10.1111/j.1365-2516.2012.02909.x. Epub 2012 Jul 6.
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Do patients with haemophilia undergoing cardiac surgery have good surgical outcomes?接受心脏手术的血友病患者手术结果良好吗?
Interact Cardiovasc Thorac Surg. 2011 Sep;13(3):320-31. doi: 10.1510/icvts.2011.272401. Epub 2011 Jun 28.

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