法布里病患者肾移植的良好结局。
Excellent outcome of renal transplantation in patients with Fabry's disease.
作者信息
Ojo A, Meier-Kriesche H U, Friedman G, Hanson J, Cibrik D, Leichtman A, Kaplan B
机构信息
University of Michigan Health System, Department of Internal Medicine, Ann Arbor 48109-0364, USA.
出版信息
Transplantation. 2000 Jun 15;69(11):2337-9. doi: 10.1097/00007890-200006150-00020.
INTRODUCTION
Fabry's disease is an X-linked error of glycosphingolipid metabolism. Clinical manifestations of the disease are secondary to accumulation of glycosphingolipids in various tissues. Renal failure and vascular complications are common. There are conflicting reports regarding the outcomes of patients with Fabry's disease after renal transplantation.
METHODS
We reviewed the United States Renal Data System Registry database from 1988 and 1998, and found 93 patients with Fabry's disease who had received a renal transplant. Case-matched patients were identified to serve as controls.
RESULTS
Patients with Fabry's disease demonstrated equivalent 5-year patient and graft survival, compared with controls (83% and 75%, respectively, for those with Fabry's disease vs. 82% and 67% for controls).
CONCLUSION
Despite their high risk for cardiovascular complications, patients with Fabry's disease have excellent outcomes after renal transplantation.
引言
法布里病是一种X连锁糖鞘脂代谢紊乱疾病。该疾病的临床表现是由于糖鞘脂在各种组织中蓄积所致。肾衰竭和血管并发症很常见。关于肾移植后法布里病患者的预后存在相互矛盾的报道。
方法
我们回顾了1988年至1998年美国肾脏数据系统登记数据库，发现93例接受肾移植的法布里病患者。确定病例匹配的患者作为对照。
结果
与对照组相比，法布里病患者的5年患者生存率和移植物生存率相当（法布里病患者分别为83%和75%，对照组为82%和67%）。
结论
尽管法布里病患者有发生心血管并发症的高风险，但肾移植后预后良好。
Zotero 插件