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摘要: 一例青年男性, 自幼诊断重型A型血友病,反复关节出血、泌尿道出血等,预防治疗后年出血次数有所减少,因右膝关节出血加重、严重活动障碍就诊。因合并HIV感染等多种感染,经多学科协作团队(MDT)专家共同讨论, 考虑存在右膝关节置换手术指征,多学科支持下采用新型锆铌合金假体及术中导航技术完成膝关节置换,围手术期给予物理康复治疗,获得满意关节功能。同时血友病、HIV感染及抗病毒药物造成患者的骨质疏松,给予严密监测及治疗。多学科保驾年轻患者的晚期血友病性关节炎的手术治疗,并严密监测治疗骨质疏松。对该例患者的诊治体现了多学科协作在疑难罕见病诊疗过程中的重要意义。Abstract: A young male diagnosed with severe hemophilia A since childhood, was presented with recurrent joint and urinary bleeding. Annualized bleed rates dropped below five with low dose prophylactic medication.Bleeding in the right knee joint recently aggravated. Due to coexisting HIV infection and advanced hemophilic arthritis, the patient was managed by a multi-disciplinary team(MDT).Total knee arthroplasty was performed by an experienced surgeon using modern prosthesis design and intraoperative navigation technologies.Physical and rehabilitation therapy was provided during the postoperative period, and joint function improved. The MDT managed the young patient with HIV infection and advanced hemophilic arthritis. The patient was diagnosed with osteoporosis thought to have been caused by hemophilia, HIV infection and antiviral drugs; and he received treatment. The treatment of this patient reflects the importance of multidisciplinary cooperation in the management of difficult and rare diseases.
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Key words:
- hemophilia /
- arthritis /
- HIV /
- osteoporosis
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表 1 围手术期及术后凝血因子用药方案
Table 1. Perioperative and postoperative coagulation factor medication regimen
围手术期 FⅧ用量 术日(术前1 h) 50 IU/kg 手术日(返室后)及术后D1~2 40 IU/kg q12h 术后D3~6 30 IU/kg q12h 术后D7~14 20~30 IU/kg qd 康复期 20~25 IU/kg tid(康复日) -
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