Pirson Y, Christophe J L, Goffin E
Service de Néphrologie, Cliniques Universitaires St Luc, Brussels, Belgium.
Nephrol Dial Transplant. 1996;11 Suppl 6:24-8. doi: 10.1093/ndt/11.supp6.24.
We review our own experience as well as pertinent literature on the outcome of renal replacement therapy (RRT) in autosomal dominant polycystic kidney disease (ADPKD). Due to the virtual absence of data on peritoneal dialysis in ADPKD, we deal only with haemodialysis (HD) and renal transplantation (TP). Special attention is paid to the renal and extrarenal complications of ADPKD. On HD, 5 year survival is 10-15% greater in ADPKD than in non-ADPKD patients, probably because of a lower cardiac mortality of ADPKD patients. After TP, patient as well as graft survival rates of ADPKD patients are similar to those of non-ADPKD patients. On HD, the prevalence of renal pain, gross haematuria and renal infection is significantly greater in ADPKD (36, 36 and 16% respectively) than in non-ADPKD patients (2, 16 and 2% respectively), but these complications are rarely severe. Other than preparation for TP, nephrectomy is required in only 4% of ADPKD patients on HD. With a policy of selective removal of problematic kidneys before TP, complications due to native polycystic kidneys do not frequently occur after TP, leading to post-TP nephrectomy in only 7% of ADPKD patients. There is a mild excess of stroke among ADPKD patients undergoing RRT, the contribution of intracranial aneurysm rupture not being clearly defined. Symptoms related to hepatic cysts are rare and to cardiac valvular abnormalities very rare. In conclusion, RRT is at least as successful in ADPKD as in non-ADPKD patients. Renal complications are frequent but rarely severe. Extrarenal complications are not frequent.
我们回顾了自身经验以及关于常染色体显性多囊肾病（ADPKD）患者肾脏替代治疗（RRT）结果的相关文献。由于几乎没有关于ADPKD患者腹膜透析的数据，我们仅探讨血液透析（HD）和肾移植（TP）。我们特别关注ADPKD的肾脏及肾外并发症。在血液透析方面，ADPKD患者的5年生存率比非ADPKD患者高10 - 15%，这可能是因为ADPKD患者的心脏死亡率较低。肾移植后，ADPKD患者的患者及移植物生存率与非ADPKD患者相似。在血液透析时，ADPKD患者肾区疼痛、肉眼血尿和肾脏感染的发生率（分别为36%、36%和16%）显著高于非ADPKD患者（分别为2%、16%和2%），但这些并发症很少严重。除了为肾移植做准备外，接受血液透析的ADPKD患者中仅4%需要进行肾切除术。采取在肾移植前选择性切除有问题肾脏的策略后，肾移植后因多囊肾引起的并发症并不常见，仅7%的ADPKD患者在肾移植后需要进行肾切除术。接受RRT的ADPKD患者中风发生率略高，颅内动脉瘤破裂的作用尚不明确。与肝囊肿相关的症状很少见，与心脏瓣膜异常相关的症状则极为罕见。总之，RRT在ADPKD患者中的成功率至少与非ADPKD患者相同。肾脏并发症常见但很少严重。肾外并发症不常见。
