Bajo M A, del Peso G, Jiménez V, Aguilera A, Villar A, Jiménez C, Selgas R
Servicio de Nefrología, Hospital Universitario La Paz, Madrid, Spain.
Adv Perit Dial. 2000;16:170-3.
Chronic renal failure is an unusual complication of hereditary clotting disorders (HCDs), but this situation could change in the near future. The modality of dialysis for end-stage renal disease (ESRD) in patients with an HCD is a difficult choice. Hemodialysis (HD) may be considered, but intensive treatment with coagulation factors is required for vascular access execution and for each HD procedure. Peritoneal dialysis (PD) has been infrequently proposed. However, PD requires coagulation replacement therapy only during peritoneal catheter placement. The aim of this paper is to describe our experience of three patients with ESRD and HCD, successfully treated with chronic PD in the medium term. Case 1 was a 58-year-old man with moderate hemophilia A, type 2 diabetes mellitus, and hepatitis C virus (HCV) infection. His ESRD was secondary to glomerulonephritis. A double-cuff peritoneal catheter was surgically placed with pre-emptive factor VIII administration. He began treatment with continuous ambulatory peritoneal dialysis (CAPD). An inguinal hernia was repaired without complications. After eleven months of follow-up, no hemorrhage episodes have been observed and clinical outcome is optimal. Case 2 was a 46-year-old man with severe hemophilia A, type 2 diabetes mellitus, and HCV and human immunodeficiency virus (HIV) infections. He developed a diabetic nephropathy that required renal replacement therapy. A permanent silicone catheter was inserted in the left internal jugular vein, and the patient started HD treatment. Later on, PD therapy was proposed. A peritoneal catheter was implanted with simultaneous factor VIII infusion. Minimal bleeding was observed at the subcutaneous tunnel over the following 48 hours. The patient started PD treatment without complications, and two months later, remaining asymptomatic, transferred to another center. Case 3 was a 41-year-old woman diagnosed with von Willebrand disease type 2A, HCV infection, and polycystic kidney disease, who presented with ESRD. An internal arteriovenous fistula was performed under coagulation factor cover. During a fistulography, and despite coagulation factor substitutive treatment, the patient showed an important hematoma. Afterwards, PD was considered. A peritoneal catheter was implanted under coagulation factor cover. The postoperative course was uncomplicated, and the patient started CAPD treatment. During follow up, she suffered two hemoperitoneum episodes that were resolved with cold dialysate. After nine months, she uneventfully continued on PD. In conclusion, PD is the therapy of choice for patients with hereditary clotting disorders and ESRD requiring dialysis. Peritoneal dialysis therapy avoids many of the complications related to HD therapy.
慢性肾衰竭是遗传性凝血障碍（HCDs）的一种罕见并发症，但这种情况在不久的将来可能会改变。对于患有HCD的终末期肾病（ESRD）患者，透析方式是一个艰难的选择。可以考虑血液透析（HD），但在建立血管通路以及每次HD治疗过程中都需要强化使用凝血因子。腹膜透析（PD）很少被推荐。然而，PD仅在放置腹膜导管期间需要凝血替代治疗。本文的目的是描述我们对3例ESRD合并HCD患者进行中期慢性PD成功治疗的经验。病例1是一名58岁男性，患有中度甲型血友病、2型糖尿病和丙型肝炎病毒（HCV）感染。他的ESRD继发于肾小球肾炎。通过术前给予凝血因子VIII，手术置入了双套囊腹膜导管。他开始接受持续性非卧床腹膜透析（CAPD）治疗。腹股沟疝得以修复，无并发症发生。经过11个月的随访，未观察到出血事件，临床结局良好。病例2是一名46岁男性，患有严重甲型血友病、2型糖尿病以及HCV和人类免疫缺陷病毒（HIV）感染。他患上了糖尿病肾病，需要肾脏替代治疗。在左颈内静脉插入了永久性硅胶导管，患者开始HD治疗。后来，建议进行PD治疗。在输注凝血因子VIII的同时植入了腹膜导管。在随后的48小时内，皮下隧道处观察到少量出血。患者开始PD治疗，无并发症发生，两个月后，无症状地转至另一中心。病例3是一名41岁女性，被诊断为2A型血管性血友病、HCV感染和多囊肾病，出现了ESRD。在凝血因子覆盖下进行了动静脉内瘘手术。在一次瘘管造影检查中，尽管进行了凝血因子替代治疗，患者仍出现了严重血肿。此后，考虑进行PD治疗。在凝血因子覆盖下植入了腹膜导管。术后过程顺利，患者开始CAPD治疗。在随访期间，她发生了两次腹腔积血事件，通过冷透析液得以解决。9个月后，她顺利继续接受PD治疗。总之，对于患有遗传性凝血障碍和需要透析的ESRD患者，PD是首选治疗方法。腹膜透析治疗避免了许多与HD治疗相关的并发症。
