PRESENT STATE OF COMBINED LIVER AND KIDNEY-TRANSPLANTATION IN PRIMARYHYPEROXALURIA TYPE-1

Primary hyperoxaluria type I (PH1) is a rare hereditary metabolic diso rder, due to the deficiency of the liver specific peroxisomal enzyme a lanine: glyoxylate aminotransferase. Since it was first attempted in 1 983, combined liver and kidney transplantation has become the only rea l therapeutic option for end-stage renal failure in pyridoxine-unrespo nsive PH1 and should be performed if the diagnosis of PH1 is unquestio nably established. The results - which are usually good - are even bet ter when transplantation is done in an early stage of the disease, bef ore calcium oxalate has dangerously accumulated throughout the body (o xalosis). 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 bee n reported in small children with PH1.