Renal Failure and Transplantation in Primary Hyperoxaluria
Primary hyperoxaluria is a rare inherited metabolic disease in which striking oxalate overproduction is associated with recurrent calcium oxalate urolithiasis leading to end-stage renal failure before the end of the second or third decade. Because conventional methods of renal substitution such as regular dialysis or renal transplantation do not alter the underlying metabolic defect the longterm results of such treatments are far worse in patients with primary hyperoxaluria than in patients whose renal failure is due to other more common causes such as chronic glomerulonephritis or pyelonephritis. This chapter reviews recent developments in the management of patients with primary hyperoxaluria as they approach terminal renal failure, together with the timing and management of renal transplantation and the option of combined synchronous hepatic and renal transplantation.
KeywordsDopamine Creatinine Dehydration Cyclosporin Oxalate
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