Abstract
Patients with some hereditary nephropathies—including autosomal dominant polycystic kidney disease (ADPKD), Fabry disease and Alport syndrome—can progress to end-stage renal disease (ESRD) and are candidates for kidney transplantation. When considering whether a potential living donor is appropriate for a particular patient, clinicians should be aware of the increased risk of adverse outcomes for the donor and the recipient. Renal transplantation from a living related donor is not contraindicated in most nephropathies that have an autosomal recessive mode of inheritance (for example, autosomal recessive polycystic kidney disease and cystinosis). Renal transplant recipients with ADPKD, however, should only receive a kidney from a related donor if the disease has been excluded in the donor by imaging and/or genetic testing. Potential living related donors for patients with Alport syndrome should be evaluated carefully for the presence of microhematuria and microalbuminuria before a decision is made to perform transplantation, and mothers or heterozygous sisters of affected male recipients with X-linked Alport syndrome should be informed about the possible long-term increased risk of renal dysfunction associated with donation. Most patients with atypical hemolytic uremic syndrome should not receive a kidney transplant from a living donor because there is a high risk of disease recurrence and graft loss.
Key Points
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Living donor kidney transplantation is often presented as the best option for patients awaiting renal transplantation, but patients whose renal failure is the result of an inherited disease might not be suitable candidates for living related transplantation
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The possible occurrence of the same disease in the related living donor should be excluded before living donor transplantation is performed in a patient with a hereditary nephropathy
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For living donor transplantation to be suitable for a particular patient, the risk of graft loss should not be higher than it would be if the patient received a graft from a deceased donor
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Individuals with autosomal dominant polycystic kidney disease requiring renal transplantation should only receive a kidney from a relative if the disease has been excluded in that relative; if imaging data is equivocal, the potential living related donor should undergo molecular testing
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Female heterozygotes for X-linked Alport syndrome who are considering donating a kidney should be informed about the long-term increased risk of renal dysfunction associated with kidney donation
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Living donor kidney transplantation is contraindicated in patients with atypical hemolytic uremic syndrome because of the high risk of recurrence and the associated high risk of graft loss
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Niaudet, P. Living donor kidney transplantation in patients with hereditary nephropathies. Nat Rev Nephrol 6, 736–743 (2010). https://doi.org/10.1038/nrneph.2010.122
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DOI: https://doi.org/10.1038/nrneph.2010.122
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