title: 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.
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.
authors.affiliation: Department of Surgery, Addenbrook's Hospital, Cambridge, UK.
authors.forename: N V
authors.initials: NV
authors.lastname: Jamieson
mesh_terms: D000293:Adolescent; D000328:Adult; D002648:Child; D002675:Child, Preschool; D003404:Creatinine / Q000097:blood; D005060:Europe; D005260:Female; D006084:Graft Rejection; D006801:Humans; D006960:Hyperoxaluria, Primary / Q000097:blood / Q000601:surgery*; D007223:Infant; D016030:Kidney Transplantation; D016031:Liver Transplantation; D008297:Male; D010070:Oxalates / Q000097:blood; D012042:Registries; D012086:Reoperation / Q000706:statistics & numerical data; D016019:Survival Analysis; D016896:Treatment Outcome
chemical_list: D010070:Oxalates; D003404:Creatinine