Would you refer a patient for kidney only or kidney and liver transplantation if they develop advanced chronic kidney disease secondary to primary hyperoxaluria type 2?
Now that the data suggesting a benefit for nedosiran for PH2 is very disappointing, I think we have to say simultaneous liver and kidney. I have this one experience. My PH2 patient had kidney only because I was thinking that nedosiran would be effective. Ultimately, the kidney failed after about 5 years, and though it wasn't solely the hyperoxaluria, that WAS the final nail in that kidney's function, with oxalate nephropathy eventually demonstrated; though earlier biopsies were negative. Now the patient is doing well after liver and his second kidney transplant.
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We have reasonable experience with kidney and liver transplant in primary hyperoxaluria type 2, and we do believe that it corrects the hyperoxaluria. Thus, in our center, we typically evaluate PH2 patients with kidney failure for potential liver transplantation, especially since the siRNA treatments do not seem to be effective for PH2.
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