Wong P N, Tong G M W, Lo K Y, Mak S K, Law E L K, Wong A K M
Renal Unit, Department of Medicine and Geriatrics, Kwong Wah Hospital, 25 Waterloo Road, Hong Kong.
Hong Kong Med J. 2002 Jun;8(3):202-6.
We report on a middle-aged man with end-stage renal failure apparently secondary to recurrent renal stones. He developed systemic oxalosis soon after commencing dialysis. The diagnosis of primary hyperoxaluria type 1 was supported by the finding of high dialysate glycolate excretion. The patient subsequently received an isolated cadaveric renal transplant, but the outcome was a rapid recurrence of oxalosis and early graft failure. Although isolated liver or renal transplantation in addition to various adjuvant measures may be considered in the early stage, combined liver-kidney transplantation remains the only definitive therapy for a patient with end-stage renal failure and systemic oxalosis due to hyperoxaluria type 1. This case illustrates the possible late presentation of primary hyperoxaluria type 1 and the poor outcome with isolated renal transplantation after the development of systemic oxalosis. One should thus have a high index of suspicion in patients with recurrent renal stones of this rare, but nevertheless important, entity.
我们报告了一名中年男性，患有终末期肾衰竭，明显继发于复发性肾结石。他在开始透析后不久就出现了全身性草酸中毒。高透析液乙醇酸盐排泄这一发现支持了1型原发性高草酸尿症的诊断。该患者随后接受了孤立的尸体肾移植，但结果是草酸中毒迅速复发且移植肾早期失功。虽然在早期可考虑孤立的肝移植或肾移植以及各种辅助措施，但肝肾联合移植仍然是1型高草酸尿症所致终末期肾衰竭和全身性草酸中毒患者的唯一确定性治疗方法。本病例说明了1型原发性高草酸尿症可能出现的晚期表现，以及全身性草酸中毒发生后孤立肾移植的不良预后。因此，对于这种罕见但很重要的复发性肾结石患者，应保持高度怀疑。
