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血友病 A 伴 VIII 因子抑制物患者行腹膜透析成功。

Successful peritoneal dialysis in a hemophilia A patient with factor VIII inhibitor.

出版信息

Perit Dial Int. 2010 Jan-Feb;30(1):114-6. doi: 10.3747/pdi.2009.00011.

Abstract

Chronic kidney disease and need for renal replacement therapy in hemophiliacs are relatively rare occurrences. Successful hemodialysis and peritoneal dialysis applications have been reported in the literature. We report a difficult-to-manage patient with hemophilia A plus factor VIII inhibitor who presented with gastrointestinal bleeding complicated by uremia. We admitted this hemophilia A patient with gastrointestinal bleeding who did not take regular factor infusions. He also had chronic kidney disease due to urinary stone disease. Since uremia might have contributed to bleeding, we chose hemodialysis along with factor VIII supplementation. His factor VIII and factor VIII inhibitor levels were 4% and 5 Bethesda units respectively. In order to bypass the inhibitor, we applied factor VIIA and prothrombin complex concentrate. After cessation of the hemorrhage, we placed a Tenckhoff catheter under prothrombin complex concentrate infusion. We did not observe any perioperative complication. To our knowledge, this is the first report of successful peritoneal dialysis in a hemophilia A patient who had factor VIII inhibitors.

摘要

慢性肾脏疾病和需要肾替代治疗的血友病患者相对较少见。文献中有成功进行血液透析和腹膜透析应用的报道。我们报告了一例血友病 A 合并因子 VIII 抑制剂的难治性患者,该患者患有尿毒症并发胃肠道出血。我们收治了一名未定期输注因子的血友病 A 合并胃肠道出血患者,该患者还患有因尿路结石病引起的慢性肾脏病。由于尿毒症可能导致出血,我们选择了血液透析和补充因子 VIII。他的因子 VIII 和因子 VIII 抑制剂水平分别为 4%和 5 个 Bethesda 单位。为了绕过抑制剂,我们应用了因子 VIIA 和凝血酶原复合物浓缩物。出血停止后,我们在凝血酶原复合物浓缩物输注下放置了 Tenckhoff 导管。我们没有观察到任何围手术期并发症。据我们所知,这是首例报道的血友病 A 患者因子 VIII 抑制剂成功进行腹膜透析的病例。

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