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Abstract：
BACKGROUND: Haemophilia A (HA) is an X-linked recessive bleeding disorder caused by lack or deficiency of coagulation factor VIII.
OBJECTIVE: The aim of this study is to determine the incidence and treatment-related risk factors of inhibitor development after intensive FVIII replacement for major orthopaedic surgery in previous treated persons with HA.
METHODS: A total of 151 HA who underwent 221 major orthopaedic surgical procedures after intensive FVIII treatment were reviewed. The results of inhibitor tests were collected. Potential clinical risk factors for inhibitor development were analyzed.
RESULTS: 111 people were diagnosed with severe HA. Thirty-seven persons (24.5%) had history of previous intensive FVIII treatment for surgical procedure. They received a mean perioperative cumulative FVIII of 498 iu/kg within first week after surgery. Seven cases (4.6%) developed an inhibitor post-operatively in our study. Surgical procedure for pseudotumor and the group of persons who experienced postoperative complications had the higher incidence of inhibitor development (9.5%, 13.3% respectively). Only previous history for intensive FVIII exposure was considered as a significant predictor for postoperative inhibitor development after multivariate logistic regression analysis (OR: 29.5, P = 0.002).
CONCLUSIONS: The incidence of inhibitor development in previously treated persons with HA undergoing major orthopaedic surgery was 4.6% and the history of previous intensive FVIII treatment for surgery was associated with higher risk of inhibitor development.
OBJECTIVE: The aim of this study is to determine the incidence and treatment-related risk factors of inhibitor development after intensive FVIII replacement for major orthopaedic surgery in previous treated persons with HA.
METHODS: A total of 151 HA who underwent 221 major orthopaedic surgical procedures after intensive FVIII treatment were reviewed. The results of inhibitor tests were collected. Potential clinical risk factors for inhibitor development were analyzed.
RESULTS: 111 people were diagnosed with severe HA. Thirty-seven persons (24.5%) had history of previous intensive FVIII treatment for surgical procedure. They received a mean perioperative cumulative FVIII of 498 iu/kg within first week after surgery. Seven cases (4.6%) developed an inhibitor post-operatively in our study. Surgical procedure for pseudotumor and the group of persons who experienced postoperative complications had the higher incidence of inhibitor development (9.5%, 13.3% respectively). Only previous history for intensive FVIII exposure was considered as a significant predictor for postoperative inhibitor development after multivariate logistic regression analysis (OR: 29.5, P = 0.002).
CONCLUSIONS: The incidence of inhibitor development in previously treated persons with HA undergoing major orthopaedic surgery was 4.6% and the history of previous intensive FVIII treatment for surgery was associated with higher risk of inhibitor development.
摘要：
背景：血友病A（HA）是一种由凝血因子VIII缺乏或缺乏引起的X连锁隐性出血性疾病。
目的：本研究的目的是确定在先前接受过HA治疗的患者中，在大的骨科手术中强化FVIII替代治疗后抑制剂发展的发生率和治疗相关的危险因素。
方法：对在强化FVIII治疗后接受221次大型骨科手术的151例HA进行了回顾。收集抑制剂试验的结果。分析抑制剂开发的潜在临床危险因素。
结果：111人被诊断为重度HA。37人（24.5％）有先前进行过FVIII强化手术治疗的病史。他们在手术后第一周内接受了平均围手术期累积FVIII为498iu/kg。在我们的研究中，有7例（4.6％）在手术后产生了抑制剂。假瘤的外科手术和经历术后并发症的人群具有更高的抑制剂发展发生率(9.5%,分别为13.3%）。在多变量逻辑回归分析后，只有以前的强化FVIII暴露史被认为是术后抑制剂发展的重要预测因子(OR:29.5,P=0.002)。
结论：在接受过重大骨科手术的先前接受过治疗的HA患者中，抑制剂发展的发生率为4.6％，并且先前的手术强化FVIII治疗史与抑制剂发展的风险更高相关。
目的：本研究的目的是确定在先前接受过HA治疗的患者中，在大的骨科手术中强化FVIII替代治疗后抑制剂发展的发生率和治疗相关的危险因素。
方法：对在强化FVIII治疗后接受221次大型骨科手术的151例HA进行了回顾。收集抑制剂试验的结果。分析抑制剂开发的潜在临床危险因素。
结果：111人被诊断为重度HA。37人（24.5％）有先前进行过FVIII强化手术治疗的病史。他们在手术后第一周内接受了平均围手术期累积FVIII为498iu/kg。在我们的研究中，有7例（4.6％）在手术后产生了抑制剂。假瘤的外科手术和经历术后并发症的人群具有更高的抑制剂发展发生率(9.5%,分别为13.3%）。在多变量逻辑回归分析后，只有以前的强化FVIII暴露史被认为是术后抑制剂发展的重要预测因子(OR:29.5,P=0.002)。
结论：在接受过重大骨科手术的先前接受过治疗的HA患者中，抑制剂发展的发生率为4.6％，并且先前的手术强化FVIII治疗史与抑制剂发展的风险更高相关。
