A Case of Primary Hyperoxaluria with Renal Allograft Dysfunction / 대한신장학회지

ABSTRACT

Primary hyperoxaluria is a rare disorder of glyoxylate metabolism in which hepatic enzyme deficiencies result in overproduction of oxalate. The resulting elevation of urinary oxalate excretion leads to recurrent urolithiasis and progressive nephrocalcinosis. End-stage renal disease frequently occurs and is accompanied by systemic oxalate deposition along with its harmful effects. With the rarity and various clinical heterogeneity of the disease, the high proportion of patients in whom diagnosis is made after advanced renal failure have developed it. On account of its high rate of graft loss associated with primary hyperoxaluria, isolated kidney transplantation has been replaced by combined liver/kidney transplantation. In this report, we describe a case of primary hyperoxaluria with kidney graft failure who had a history of recurrent renal stones.