Present state of combined liver and kidney transplantation in primary hyperoxaluria type 1

Primary hyperoxaluria type I (PH1) is a rare hereditary metabolic disorder, due to the deficiency of the liver specific peroxisomal enzyme alanine: glyoxylate aminotransferase. Since it was first attempted in 1983, combined liver and kidney transplantation has become the only real therapeutic option for end-stage renal failure in pyridoxine-unresponsive PH1 and should be performed if the diagnosis of PH1 is unquestionably established. The results - which are usually good - are even better when transplantation is done in an early stage of the disease, before calcium oxalate has dangerously accumulated throughout the body (oxalosis). Several pre- and postoperative measures can reduce the risk of oxalate crystal deposition in the kidney graft in case of oxalosis. A favourable outcome of combined liver-kidney transplantation has been reported in small children with PH1.