Rosenblatt Gregory S, Jenkins Randall D, Barry John M
Department of Surgery, Division of Urology and Renal Transplantation, Oregon Health and Science University, Portland, Oregon 97239-3098, USA.
Urology. 2006 Aug;68(2):427.e7-8. doi: 10.1016/j.urology.2006.02.035.
Alanine-glyoxalate aminotransferase deficiency occurs in patients with primary hyperoxaluria type 1. Increased hepatic oxalate production leads to high urine concentrations of glycolate and oxalate. Calcium oxalate nephrolithiasis and nephrocalcinosis occur, and renal function progressively declines until patients develop end-stage renal disease. Renal transplantation alone is inadequate therapy because the primary enzyme deficiency remains. We report what we believe to be the second-youngest recipient to undergo successful sequential liver and kidney transplantation from a single living-related donor for treatment of primary hyperoxaluria type 1. We also discuss the changes in this patient's serum oxalate levels after transplantation.
丙氨酸-乙醛酸转氨酶缺乏症发生在1型原发性高草酸尿症患者中。肝脏草酸盐生成增加导致尿液中乙醇酸盐和草酸盐浓度升高。草酸钙肾结石和肾钙质沉着症出现，肾功能逐渐下降，直至患者发展为终末期肾病。仅进行肾移植治疗并不充分，因为原发性酶缺乏依然存在。我们报告了我们认为是第二年轻的接受来自单一活体亲属供体的序贯肝移植和肾移植以治疗1型原发性高草酸尿症并获得成功的受者。我们还讨论了该患者移植后血清草酸盐水平的变化。
