Long-term Outcomes of Kidney Transplantation in Fabry Disease

摘要

Background Fabry disease (FD) is a rare X-linked lysosomal storage disorder caused by mutations in the -galactosidase A gene that obliterate or markedly reduce -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.