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Case Reports
. 2018 Jan;18(1):253-257.
doi: 10.1111/ajt.14418. Epub 2017 Aug 14.

Combined Liver-Kidney Transplantation for Primary Hyperoxaluria Type 2: A Case Report

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Free PMC article
Case Reports

Combined Liver-Kidney Transplantation for Primary Hyperoxaluria Type 2: A Case Report

T Dhondup et al. Am J Transplant. .
Free PMC article

Abstract

Combined liver/kidney transplant is the preferred transplant option for most patients with primary hyperoxaluria type 1 (PH1) since orthotopic liver transplantation replaces the deficient liver-specific AGT enzyme, thus restoring normal metabolic oxalate production. However, primary hyperoxaluria type 2 (PH2) is caused by deficient glyoxylate reductase/hydroxypyruvate reductase (GRHPR), and this enzyme is widely distributed throughout the body. Though the relative abundance and activity of GRHPR in various tissues is not clear, some evidence suggests that the majority of enzyme activity may indeed reside within the liver. Thus the effectiveness of liver transplantation in correcting this metabolic disorder has not been demonstrated. Here we report a case of 44-year-old man with PH2, frequent stone events, and end-stage renal disease; he received a combined liver/kidney transplant. Although requiring confirmation in additional cases, the normalization of plasma oxalate, urine oxalate, and urine glycerate levels observed in this patient within a month of the transplant that remain reduced at the most recent follow-up at 13 months suggests that correction of the GRHPR deficiency in PH2 can be achieved by liver transplantation.

Keywords: clinical research/practice; kidney failure/injury; kidney transplantation/nephrology; liver biology; liver transplantation/hepatology.

Conflict of interest statement

Disclosure

The authors of this manuscript have no conflicts of interest to disclose as described by the American Journal of Transplantation.

Figures

Figure 1
Figure 1
Biopsy of kidney- alone transplant. Calcium oxalate crystals (arrows) are apparent in H&E section (Panel A) and are birefringent under polarizing light (Panel B).
Figure 2
Figure 2
Change in plasma oxalate level pre and post combined Liver-Kidney transplant (Normal plasma oxalate level <1.8 µmol/L).

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