Schürmann G, Schärer K, Wingen A M, Otto G, Herfarth C
Department of Surgery, University of Heidelberg, West Germany.
Nephrol Dial Transplant. 1990;5(9):825-7. doi: 10.1093/ndt/5.9.825.
Infantile oxalosis is the most severe form of primary hyperoxaluria type 1, an inborn metabolic disorder caused by a deficiency of the hepatic enzyme alanine: glyoxylate aminotransferase (AGT). Renal insufficiency occurs due to excessive production and renal deposits of oxalate. This report concerns a 22-month-old girl with severe type 1 primary hyperoxaluria and chronic renal failure. Liver transplantation was performed successfully as treatment of AGT deficiency. Endogenous creatinine clearance remained stable at about 10 ml/min per 1.73 m2 at 23 months after transplantation. It is suggested that liver transplantation offers potential cure of an otherwise fatal disease. However, it remains questionable if the procedure influences kidney function in the presence of advanced renal disease.
婴儿草酸血症是1型原发性高草酸尿症最严重的形式，这是一种由肝脏酶丙氨酸：乙醛酸转氨酶（AGT）缺乏引起的先天性代谢紊乱。由于草酸盐的过度产生和肾脏沉积，导致肾功能不全。本报告涉及一名患有严重1型原发性高草酸尿症和慢性肾衰竭的22个月大女孩。成功进行了肝移植以治疗AGT缺乏症。移植后23个月，内源性肌酐清除率稳定在每1.73平方米约10毫升/分钟。提示肝移植为这种原本致命的疾病提供了潜在的治愈方法。然而，在存在晚期肾脏疾病的情况下，该手术是否会影响肾功能仍存在疑问。
