Transplantation Outcomes in Primary Hyperoxaluria

Abstract

Optimal transplantation strategies are uncertain in primary hyperoxaluria (PH) due to potential for recurrent oxalosis. Outcomes of different transplantation approaches were compared using life -table methods to

Combined liver and kidney transplantation in children

“…Early reports from the USA suggested equally good outcomes with isolated KT and CLKT [30]. However, more recent data from Europe and USA indicate significantly better outcomes after CLKT [27,29]. In the European database, only one of 38 PH1 children (3 %) transplanted after 2000 received isolated KT.…”

“…Of the 26 PH1 patients in the IPHR, 67 % were alive 7 years after CLKT. Among patients who were transplanted since 2000, 84 % of the 29 first kidney grafts (24 CLKT) were functioning in 2010 [27]. In the large European registry, the kidney graft survival rate in PH1 patients was 82, 79, and 76 % at 1, 3, and 5 years, respectively, for CLKT.…”

“…Better survival following CLKT for PH1 has been attributed to early diagnosis, short time on dialysis, and good condition of the patient (less complications of oxalosis) before the operation. In two recent registry reports, kidney graft survival rates in PH1 patients were 79 and 84 %, respectively, at 3 years [23,27].…”

“…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]. CLKT is the method of choice in patients with severe forms of PH1 but not in those who clearly benefit from vitamin B6 therapy and/or have favorable mutations, such as the homozygous c,508G>A mutation [28].…”

Crystalline Bone Marrow in Systemic Oxalosis

“…2 For some patients, treatment with pyridoxine to reduce urine oxalate levels may be beneficial, though liver and kidney transplantation is often required. 3 Both kidney transplants had taken place overseas, and the patient's initial renal failure and first transplant failure were attributed to obstructive nephropathy from renal calculi. The second transplant loss was from rejection related to a reduction of immunosuppression, though biopsy revealed deposition of calcium oxalate crystals as well.…”