Ishii Hiroki, Miyoshi Chiaki, Hirai Keiji, Morino Junki, Minato Saori, Kaneko Shohei, Yanai Katsunori, Matsuyama Momoko, Kitano Taisuke, Shindo Mitsutoshi, Aomatsu Akinori, Shimoyama Hiroshi, Miyazawa Haruhisa, Ito Kiyonori, Ueda Yuichiro, Kaku Yoshio, Hoshino Taro, Ookawara Susumu, Morishita Yoshiyuki
Division of Nephrology, First Department of Integrated Medicine, Saitama Medical Center, Jichi Medical University, 1-847 Amanuma-cho, Omiya-ku, Saitama, Saitama, 330-8503, Japan.
CEN Case Rep. 2020 Aug;9(3):225-231. doi: 10.1007/s13730-020-00461-1. Epub 2020 Mar 16.
An 88-year-old man with congenital hemophilia A developed end-stage renal disease due to microscopic polyangiitis. He was at risk for catheter-related infection because he was taking immunosuppressive agents for the treatment of polyangiitis. He was also unable to manipulate the peritoneal dialysis device. Therefore, hemodialysis using an arteriovenous fistula was induced for renal replacement therapy. Recombinant coagulation factor VIII (1000 IU) was administered via the venous chamber of the hemodialysis circuit 10 min before the end of each hemodialysis session, and nafamostat mesylate (25 mg/h) was employed as an anticoagulant during hemodialysis. His clotting factor VIII activity level increased to > 50% and activated partial thromboplastin time decreased to 50 s at the end of each hemodialysis session. This method allowed him to achieve hemostasis at the puncture site of the arteriovenous fistula and undergo stable hemodialysis with no complications, including bleeding. This case suggests that hemodialysis using an arteriovenous fistula with coagulation factor replacement and nafamostat mesylate in each hemodialysis session is a therapeutic option for end-stage renal disease in patients of advanced age with hemophilia at high risk of bleeding.
一名88岁的先天性甲型血友病男性因显微镜下多血管炎发展为终末期肾病。由于他正在服用免疫抑制剂治疗多血管炎，存在导管相关感染的风险。他也无法操作腹膜透析装置。因此，诱导采用动静脉内瘘进行血液透析以进行肾脏替代治疗。在每次血液透析结束前10分钟，通过血液透析回路的静脉腔给予重组凝血因子VIII（1000 IU），并在血液透析期间使用甲磺酸萘莫司他（25 mg/h）作为抗凝剂。每次血液透析结束时，他的凝血因子VIII活性水平升至>50%，活化部分凝血活酶时间降至50秒。这种方法使他能够在动静脉内瘘穿刺部位实现止血，并进行稳定的血液透析，且无包括出血在内的并发症。该病例表明，对于有高出血风险的老年血友病患者的终末期肾病，每次血液透析时使用动静脉内瘘并进行凝血因子替代和甲磺酸萘莫司他的血液透析是一种治疗选择。
