Successful renal transplantation in primary hyperoxaluria.
A successful live related renal transplant in a 29-year-old male patient with Type 1 primary hyperoxaluria, who remains well 32 months postoperatively, is described. The plasma oxalate and exchangeable oxalate pool before transplantation were 160 mumol/1 and 4429 mumol respectively. Since the transplant these have been greatly reduced although they remain elevated above the normal by a factor of 2. Pyridoxine therapy and the avoidance of oxalate-rich foods have been effective in maintaining these reduced levels and the 24-hr urinary oxalate excretion has also been maintained close to normal levels on this regime. After review of the previously reported transplants in patients with well documented primary hyperoxaluria and from the experience with this patient, the following guidelines for successful renal transplantation in primary hyperoxaluria are suggested: transplants should only be carried out in those who have shown a response to adequate pyrodoxine therapy; frequent haemodialysis pre-operatively and during periods of oliguria postoperatively is necessary; oxalate-rich foods should be avoided and a high fluid intake should be maintained after transplantation. If these guidelines are followed there is no contra-indicatin to live related renal transplants in primary hyperoxaluric patients.
D000328:Adult; D006801:Humans; D016030:Kidney Transplantation; D008297:Male; D008659:Metabolic Diseases / Q000178:diet therapy / Q000188:drug therapy / Q000601:surgery; D010070:Oxalates / Q000378:metabolism / Q000652:urine*; D011736:Pyridoxine / Q000627:therapeutic use
D010070:Oxalates; D011736:Pyridoxine