학술논문
Outcomes of Kidney Transplantation in Fabry Disease: A Meta-Analysis.
Document Type
Academic Journal
Author
Suarez MLG; Division of Nephrology and Hypertension, Department of Medicine, Mayo Clinic, Rochester, MN 55905, USA.; Division of Nephrology, Department of Internal Medicine, University of Mississippi Medical Center, Jackson, MS 39216, USA.; Thongprayoon C; Division of Nephrology and Hypertension, Department of Medicine, Mayo Clinic, Rochester, MN 55905, USA.; Hansrivijit P; Department of Internal Medicine, UPMC Pinnacle, Harrisburg, PA 17105, USA.; Medaura J; Division of Nephrology, Department of Internal Medicine, University of Mississippi Medical Center, Jackson, MS 39216, USA.; Vaitla P; Division of Nephrology, Department of Internal Medicine, University of Mississippi Medical Center, Jackson, MS 39216, USA.; Mao MA; Division of Nephrology and Hypertension, Department of Medicine, Mayo Clinic, Jacksonville, FL 32224, USA.; Bathini T; Department of Internal Medicine, University of Arizona, Tucson, AZ 85721, USA.; Boonpheng B; Department of Medicine, David Geffen School of Medicine, University of California, Los Angeles, CA 90095, USA.; Kanduri SR; Division of Nephrology, Ochsner Medical Center, New Orleans, LA 70121, USA.; Kovvuru K; Division of Nephrology, Ochsner Medical Center, New Orleans, LA 70121, USA.; Basu A; Emory Transplant Center and Department of Medicine, Renal Division, Emory University School of Medicine, Atlanta, GA 30322, USA.; Cheungpasitporn W; Division of Nephrology and Hypertension, Department of Medicine, Mayo Clinic, Rochester, MN 55905, USA.
Source
Publisher: MDPI AG Country of Publication: Switzerland NLM ID: 101636232 Publication Model: Electronic Cited Medium: Print ISSN: 2079-9721 (Print) Linking ISSN: 20799721 NLM ISO Abbreviation: Diseases Subsets: PubMed not MEDLINE
Subject
Language
English
ISSN
2079-9721
Abstract
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 therapy involves 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: A comprehensive literature review was conducted utilizing MEDLINE, EMBASE, and Cochrane Database, from inception through to 28 February 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: In total, 11 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. In the 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 are no significant differences in the risks of all-cause graft failure ( p = 0.10) or mortality (0.48) among recipients with vs. without FD.
Conclusions: Despite possible FD recurrence after transplantation of 11.1%, allograft and patient survival are comparable among kidney transplant recipients with vs. without FD.
Methods: A comprehensive literature review was conducted utilizing MEDLINE, EMBASE, and Cochrane Database, from inception through to 28 February 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: In total, 11 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. In the 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 are no significant differences in the risks of all-cause graft failure ( p = 0.10) or mortality (0.48) among recipients with vs. without FD.
Conclusions: Despite possible FD recurrence after transplantation of 11.1%, allograft and patient survival are comparable among kidney transplant recipients with vs. without FD.