title: A 20-year experience of combined liver/kidney transplantation for primary hyperoxaluria (PH1): the European PH1 transplant registry experience 1984-2004.
abstract: Primary hyperoxaluria (PH1) is a condition caused by a hepatic-based enzyme defect which can lead to renal failure due to oxalate stone disease, obstructive uropathy and nephrocalcinosis. It has been shown that the underlying metabolic defect can be corrected by liver transplantation and in most cases (renal failure having already occurred) is accompanied by a kidney graft. This paper describes the current results of 127 liver transplants performed in 117 patients over a 20-year period from 1984 to 2004 in 35 European centres. The mean age at onset of symptoms was 5.6 +/- 7.8 years and the mean age at which a diagnosis was made was 8.8 +/- 9.5 years. The diagnosis was confirmed by liver biopsy proven decreased AGT activity in 68% of cases, hyperoxaluria in 74%, hyperglycolicaciduria in 37% and hyperoxalaemia in 50%. Patients were transplanted at a mean age of 16.5 +/- 11.4 years following a period of dialysis of 3.2 +/- 3.2 years (range 0-14.4 years). 1-, 5- and 10-year patient survival values were 86, 80 and 69%, respectively, and liver graft survival rates of 80, 72 and 60% at the same time intervals. There have been 27 deaths and 10 liver retransplants have been carried out. Patient outcomes are improved when prolonged periods on dialysis and the complications of systemic oxalosis have not occurred.
authors.affiliation: Department of Surgery, Addenbrookes Hospital, Cambridge, UK. nvj1000@cam.ac.uk
authors.forename: Neville V
authors.initials: NV
authors.lastname: Jamieson
mesh_terms: D002648:Child; D002675:Child, Preschool; D003404:Creatinine / Q000652:urine; D005060:Europe; D006085:Graft Survival; D006801:Humans; D006960:Hyperoxaluria, Primary / Q000097:blood / Q000601:surgery* / Q000628:therapy; D016030:Kidney Transplantation; D016031:Liver Transplantation; D010070:Oxalates / Q000097:blood; D011184:Postoperative Period; D012042:Registries; D006435:Renal Dialysis; D016019:Survival Analysis; D013997:Time Factors
chemical_list: D010070:Oxalates; D003404:Creatinine