Nephrol Dial Transplant 1995;10 Suppl 8:33-7

The European Primary Hyperoxaluria Type 1 Transplant Registry report on the results of combined liver/kidney transplantation for primary hyperoxaluria 1984-1994. European PH1 Transplantation Study Group

Jamieson NV

Department of Surgery, Addenbrook's Hospital, Cambridge, UK

Abstract
This paper reports on a total of 61 patients receiving 64 liver grafts for
primary hyperoxaluria type 1 (PH1) at 25 centres throughout Europe between 1984
and 1994 recorded in a central registry. Fifty-seven patients received
simultaneous kidney grafts and one patient a delayed kidney graft, two patients
received isolated liver grafts and a further three patients underwent
retransplantation of the liver, a final patient receiving a liver alone dying
intra-operatively before a planned kidney graft was performed. Results of such
liver grafts are excellent, reversing the metabolic defect of PH1. Thirteen
grafts have been lost and 10 patients have died, with actuarial graft and
patient survival rates of 88% at 1 year and patient survival of 80% at 5 years.
Early combined liver and kidney grafting appears to represent the optimal
treatment for patients with PH1, avoiding the adverse effects of a prolonged
period of dialysis and the development of the complications of systemic
oxalosis. The registry will continue to collect and collate data on patients
undergoing hepatic transplantation for PH1