Saturday, December 18, 2010

Genetic Nephropathies and Kidney Transplantation

A recent article in Nature Review Nephrology reviews the diseases we always get worried if a living donor can be used.
This review outlines the does and don't of hereditary nephropathies and donor evaluation of kidney transplantation. A table in the articles nicely summarizes the disease entity and if the living related donation would be appropriate.
In Finnish type Congenital Nephrotic syndrome, NPHS2 FSGS, NPHS3 FSGS, Pierson Syndrome, Schimke's immunoosseous dystrophy, nephronophthisis, cystinosis and ARPKD and alport syndrome:- it is ok to use living related donation in transplantation.  In Primary Hyperoxaluria and Atypical HUS, one has to be careful in selecting the donor from a living relative.
In general AR type of diseases, the donor can be a relative and most of the time its not a problem. Autosomal Dominant diseases is always a concern. We come across this most in ADPKD and the donor evaluation in that case is so strict and needs careful screening if its a relative.  Atypical HUS should not receive a kidney transplantation from a living donor because it is a high risk for disease recurrence and graft loss.


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