Transplantation
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Multicenter Study
Long-term Outcomes of Kidney Transplantation in Fabry Disease.
Fabry disease (FD) is a rare X-linked lysosomal storage disorder caused by mutations in the α-galactosidase A gene that obliterate or markedly reduce α-galactosidase A activity. This results in the systemic accumulation of its glycosphingolipid substrates in body fluids and organs, including the kidney. Fabry nephropathy can lead to end-stage renal disease requiring kidney transplantation. Little is known about its long-term outcomes and the overall patient survival after kidney transplantation. ⋯ We conclude that kidney transplantation has an excellent long-term outcome in FD.