Watts R W, Morgan S H, Mansell M A, Purkiss P
Division of Inherited Metabolic Diseases, MRC Clinical Research Centre, Harrow, United Kingdom.
Ann Acad Med Singap. 1987 Apr;16(2):337-9.
The increased production and excretion of oxalate in primary hyperoxaluria causes urolithiasis, nephrocalcinosis with renal failure, and systemic oxalosis. Systemic oxalosis occurs late in the course of the disease when there is both oxalate retention and increased oxalate synthesis. The uraemia can be controlled by conventional haemodialysis or peritoneal dialysis but treatment cannot usually keep up with accelerated rate of oxalate production, and dialysed patients develop systemic oxalosis. Most attempts to treat primary hyperoxaluria by renal transplantation have been unsuccessful because of rapid recurrence of nephrocalcinosis with uraemia and systemic oxalosis. Dynamic studies of overall oxalate metabolism in vivo have shown that the renal retention factor becomes a major determinant of oxalosis when the GFR decreases to less than 25 ml min-1 1.73 m-2. We conclude provisionally that vigorous haemodialysis should be begun and transplantation arranged when the GFR reaches this level. Such early transplantation with vigorous perioperative haemodialysis and a large perioperative diuresis of water gives good immediate graft function and oxalate mobilisation from the miscible oxalate pool. The longer term outlook is then influenced more by the factors which determine the success of renal transplantation in non-hyperoxaluric patients.
原发性高草酸尿症中草酸盐生成和排泄增加会导致尿路结石、伴有肾衰竭的肾钙质沉着症以及全身性草酸中毒。全身性草酸中毒在疾病后期出现，此时既存在草酸盐潴留又有草酸盐合成增加。尿毒症可通过传统的血液透析或腹膜透析得到控制，但治疗通常无法跟上草酸盐生成加速的速度，接受透析的患者会发生全身性草酸中毒。大多数通过肾移植治疗原发性高草酸尿症的尝试都未成功，原因是肾钙质沉着症伴尿毒症和全身性草酸中毒迅速复发。对体内整体草酸代谢的动态研究表明，当肾小球滤过率（GFR）降至低于25 ml·min⁻¹·1.73 m⁻²时，肾脏潴留因素成为草酸中毒的主要决定因素。我们初步得出结论，当GFR达到此水平时，应开始积极的血液透析并安排移植。这种早期移植结合积极的围手术期血液透析和大量围手术期水利尿，可使移植肾立即具有良好功能，并促进可混溶草酸盐池中的草酸盐动员。长期预后随后更多地受到决定非高草酸尿症患者肾移植成功的因素影响。
