Guidelines on the use of prophylactic factor replacement for children and adults with Haemophilia A and B
Guidelines on the use of prophylactic factor replacement for children and adults with Haemophilia A and B
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DOI：
10.1111/bjh.16704
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发表时间：
2020-05-10
影响因子：
6.5
通讯作者：
Biss, Tina
中科院分区：
文献类型：
--
作者：
Rayment, Rachel;Chalmers, Elizabeth;Biss, Tina
A型或B型血友病（PWH）患者的凝血因子替代治疗可以在出血时进行[按需（OD）治疗]，也可以定期进行以预防出血（预防性治疗）。英国血友病中心医生组织（UKHCDO）于2010年制定了儿童和成人重度血友病A（SHA）预防性治疗指南，总结了男孩预防性治疗的高水平循证研究，并就预防性治疗在成人SHA中的作用提出了建议。1本指南建立在前者的基础上，接受了SHA儿童预防获益的明确证据。它解决了儿童和成人血友病A和B预防的最佳使用，并在适当的情况下给出了基于证据的建议。该指南将对医疗保健专业人员、实验室科学家、患者和负责资助服务的人具有价值。
Coagulation factor replacement in people with haemophilia (PWH) A or B may be given either in response to a bleed [on-demand (OD) therapy] or regularly to prevent bleeding (prophylactic therapy). Guidelines for prophylactic treatment of children and adults with severe haemophilia A (SHA) were produced by the United Kingdom Haemophilia Centre Doctors’ Organisation (UKHCDO) in 2010, summarising the high-level, evidence-based studies of prophylaxis in boys and advising on the role of prophylaxis in adults with SHA. 1 This guideline builds on the former, accepting the clear evidence of benefit of prophylaxis in children with SHA. It addresses the optimum use of prophylaxis in children and adults with haemophilia A and B and gives evidence-based recommendations where appropriate. The guidance will be of value to healthcare professionals, laboratory scientists, patients and those with a responsibility for funding services.