Résumé
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 On account of its high rate of graft loss associated with primary hyperoxaluria, isolated kidney transplantation has been replaced by combined liver/kidney transplantation.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.
Sujets)
Humains , Glyoxylates , Hyperoxalurie primaire , Rein , Défaillance rénale chronique , Transplantation rénale , Néphrocalcinose , Caractéristiques de la population , Insuffisance rénale , Transplantation homologue , Transplants , UrolithiaseRésumé
BACKGROUND/AIMS: The Korean Network for Organ Sharing (KONOS) was established in 2000, and the KONOS criteria for expanded-criteria donors (ECD) have since been applied to kidney allocation from deceased donors. The outcome of cadaveric kidney transplantation from ECD according to KONOS criteria has not been investigated. METHODS: Seventy-seven cadaveric kidney transplants from January 2003 to December 2009 were recruited retrospectively. Factors that influence the glomerular filtration rate (GFR) of graft kidneys up to 36 months after transplantation were evaluated. Postoperative renal function and allograft and patient survival in the ECD group (n = 28) were compared with those in the standard-criteria donor (SCD) group (n = 49). RESULTS: The GFR of graft kidneys was different according to donor GFR, age, hypertension history, and cause of brain death. In the ECD group, GFR was lower than that in the SCD group by KONOS criteria. No differences in allograft or patient survival were observed until 3 years after kidney transplantation. CONCLUSIONS: Cadaveric kidney transplantation using the ECD by KONOS criteria is acceptable in term of graft and 3-year patient survival, although the GFR was lower in the ECD than in the SCD group.