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Fabry Disease Transplant Considerations — ESENeph MCQ

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ModerateTransplantationFabry Disease Transplant ConsiderationsESENeph

A 40-year-old man with Fabry disease is being evaluated for kidney transplant. He has ESKD from Fabry nephropathy. His alpha-galactosidase A activity is <1%. He is on enzyme replacement therapy. What specific transplant consideration applies to Fabry disease?

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Correct answer: C — Transplanted kidneys do not develop recurrent Fabry nephropathy (the donor kidney has normal alpha-galactosidase A); ERT should continue post-transplant for systemic disease management (cardiac, neurological)

The transplanted kidney from a non-Fabry donor has normal alpha-galactosidase A enzyme activity and does not develop Fabry-related deposits. Therefore, recurrent Fabry nephropathy in the allograft does not occur. However, ERT (or Migalastat if amenable mutation) must continue post-transplant to manage systemic Fabry disease (cardiac involvement, neuropathy, cerebrovascular disease). Transplant outcomes in Fabry disease are comparable to matched controls. Living related donors should be screened for Fabry carrier status.

Reference: KDIGO 2020 – Transplant Candidate Evaluation; ERA-EDTA 2022 – Fabry Guideline