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Kidney Week

Abstract: PO2536

Outcomes of Kidney Transplantation in Fabry Disease: A Meta-Analysis

Session Information

Category: Transplantation

  • 1902 Transplantation: Clinical

Authors

  • Gonzalez Suarez, Maria Lourdes, University of Mississippi Medical Center, Jackson, Mississippi, United States
  • Thongprayoon, Charat, Mayo Clinic, Rochester, Minnesota, United States
  • Mao, Michael A., Mayo Clinic, Jacksonville, Florida, United States
  • Medaura, Juan Antonio, University of Mississippi Medical Center, Jackson, Mississippi, United States
  • Cheungpasitporn, Wisit, University of Mississippi Medical Center, Jackson, Mississippi, United States
Background

Fabry disease (FD) is a rare X-linked lysosomal storage disorder with progressive systemic deposition of globotriaosylceramide, leading to life-threatening cardiac, central nervous system, and kidney disease. Current therapies involve symptomatic medical management, enzyme replacement therapy (ERT), dialysis, kidney transplantation, and more recently gene therapy. The aim of this systematic review was to assess outcomes of kidney transplantation among patients with FD.

Methods

Comprehensive literature review was conducted utilizing MEDLINE, EMBASE and Cochrane Database, from inception through February 28, 2020 to identify studies that evaluate outcomes of kidney transplantation including patient and allograft survival among kidney transplant patients with FD. Effect estimates from each study were extracted and combined using the random-effects, generic inverse variance method of DerSimonian and Laird.

Results

Eleven studies including 424 kidney transplant recipients with FD were enrolled. The post-transplant median follow-up time ranged from 3 to 11.5 years. Overall, the pooled estimated rates of all-cause graft failure, graft failure before death, and allograft rejection were 32.5% (95%CI: 23.9%-42.5%), 14.5% (95%CI: 8.4%-23.7%), and 20.2% (95%CI: 15.4%-25.9%), respectively. A sensitivity analysis limited only to the recent studies (year 2001 or newer when ERT became available), the pooled estimated rates of all-cause graft failure, graft failure before death, and allograft rejection were 28.1% (95%CI: 20.5%-37.3%), 11.7% (95%CI: 8.4%-16.0%), and 20.2% (95%CI: 15.5%-26.0%), respectively. The pooled estimated rate of biopsy proven FD recurrence was 11.1% (95%CI: 3.6%-29.4%), respectively. There was no significant difference in the risk of all-cause graft failure (P = 0.10) nor mortality (0.48) among recipients with vs. without FD.

Conclusion

Despite possible FD recurrence after transplantation of 11.1%, allograft and patient survival are similar among kidney transplant recipients with vs. without FD.