Ladefoged S D, Jørgensen H E
Ugeskr Laeger. 1989 Jul 17;151(29):1856-9.
Primary hyperoxaluria is a recessive hereditary disturbance of glyoxylate metabolism caused by deficiency of the liver enzyme, alanine glyoxylate transaminase. The main symptoms are recurrent renal stones, nephrocalcinosis and renal failure. In the advanced state, the disease is frequently complicated by osseous disease, vascular insufficiency and cardiac arrhytmias caused by deposits of calcium oxalate in the tissue. The prognosis is poor. No specific medical treatment exists. Dialysis is not effective and the results of renal transplantation is poor. Combined liver and renal transplantation correct the metabolic defect and the excretion of oxalate is normalised. Combined transplantation must be regarded as the optimal treatment of renal failure caused by PHO. The transplantation should be undertaken preferably before the creatinine clearance falls below 10-20 ml/min in order to avoid tissue deposits of calcium oxalate and excessive urinary excretion of oxalate during immediate post-transplantation period.
原发性高草酸尿症是一种隐性遗传性乙醛酸代谢紊乱疾病，由肝脏酶丙氨酸乙醛酸转氨酶缺乏引起。主要症状为复发性肾结石、肾钙质沉着症和肾衰竭。在疾病晚期，常因草酸钙在组织中沉积而并发骨病、血管功能不全和心律失常。预后较差。目前尚无特效药物治疗。透析无效，肾移植效果不佳。肝肾联合移植可纠正代谢缺陷，使草酸盐排泄正常化。肝肾联合移植应被视为原发性高草酸尿症所致肾衰竭的最佳治疗方法。移植最好在肌酐清除率降至10 - 20 ml/min以下之前进行，以避免移植后即刻出现草酸钙组织沉积和草酸盐尿排泄过多。
