Liver Transplant

Kidney and Liver Transplant for Oxalosis

Primary hyperoxaluria (sometimes referred to as oxalosis) is a genetic disorder that results in an increased production of oxalate by the liver. The large amount of oxalate leads to high concentrations in the urine which, over time, can cause kidney stones and kidney failure. The disorder has two types, PH-I and PH-II (depending on the defective enzyme), both are rare. Kidney transplantation can be an effective treatment for people with this condition. Patients with PH-I who are unresponsive to vitamin B6 (pyridoxine) therapy will require combined liver and kidney transplantation. Long-term success with both transplants is good.

Moving quickly toward transplant is advisable because the longer the patient is on dialysis, the more the oxalate builds up in the body and the longer it takes to remove it (up to five years for patients who have been on dialysis for more than a year).

Patients with primary hyperoxaluria should undergo liver biopsy to confirm the subtype, and PH-I patients whould be tested for responsiveness to pyridoxine therapy. Urine oxalate and plasma oxalate values are also useful.

For questions regarding eligibility for kidney or combined liver/kidney transplantation as a treatment of primary hyperoxaluria, please contact Dawn Milliner, M.D., Division of Nephrology or Scott Nyberg, M.D., Ph.D., Division of Transplantation Surgery.