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.

Combined liver and kidney transplantation in children

“…The 5-and 10-year patient survival rates were 80 and 69 %, respectively, in a European cohort of 117 PH1 patients transplanted before 2005 [25]. In this cohort, 13 renal grafts failed, of which seven were lost due to oxalate deposition.…”

“…For over two decades the predominant strategy for management of PH1 in Europe has been to directly pursue CLKT for patients with kidney failure [25,26]. According to a recent report of the International Primary Hyperoxaluria Registry (IPHR), the use of CLKT as the primary therapy has also increased in non-European centers such that, since 2000, 24 of the 29 transplanted PH1 patients underwent CLKT [27].…”

Liver transplantation in oxalosis prior to advanced chronic kidney disease

“…This "cure" assumes that kidney failure will occur, and will recur, without that treatment. The predominant strategy for management of PH in Europe, for the past two decades, has been to directly pursue combined K/LTx for patients with renal failure [6,7]. The underlying medical milieu is one of relatively uncommon LD (living donor) isolated kidney transplantation (KTx), but a cooperative relationship of physicians with specialized PH transplant centers in Europe.…”

“…The European consortium provided a life table analysis for K/LTx patients with PH [6,7]. This registry reflects selected patients, but it additionally contains data on the importance of the condition of patients transplanted to survival that are not available in other datasets.…”

“…International Registry (Mayo) [16] e Data from the European Oxalate Transplant Registry [6] f UNOS: overall data [13] g UNOS: metabolic diseases [13] h UNOS: pediatric data [13] i Data from the European Transplant Registry [15] j UNOS: all data [14] k UNOS: metabolic diseases [14] The opportunity to look at specialized centers was provided from a clinic in Hamburg, Germany [17], whose experience with 13 patients was reported. Seven patients underwent K/LTx; all were alive at reporting, with one having required a repeat KTx for "chronic allograft nephropathy".…”