A Case of Primary Hyperoxaluria with Renal Allograft Dysfunction

Korean Journal of Nephrology 2011;30(6):676-681.

Eun Joo Song, M.D.1, Jang Hee Cho, M.D.1, Young Deuk Yoon, M.D.1, Ja Yong Park, M.D.1, Ji Young Choi, M.D.1, Se Hee Yoon, M.D.1, Yon Su Kim, M.D.2, Chan Duck Kim, M.D.1, Yong-Lim Kim, M.D.1 and Sun-Hee Park, M.D.1

Department of Internal Medicine¹
Kyungpook National University, Daegu, Korea; Department of Internal Medicine²
Seoul National University Hospital, Seoul, Korea

증례 : 이식신의 기능상실이 동반된 일차성 과옥살산뇨증 1예

송은주1 , 조장희1 , 윤영득1 , 박자용1 , 최지영1, 윤세희1 , 김연수2 , 김찬덕1 , 김용림1 , 박선희1

경북대학교 의학전문대학원 내과학교실1 , 서울대학교 의과대학 내과학교실2

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 On account of its high rate of graft loss associated with primary hyperoxaluria, isolated kidney transplantation has been replaced by combined liver/kidney transplantation.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.

Key Words: Oxalate, Primary hyperoxaluria, Kidney transplantation