Alanine-glyoxalate aminotransferase deficiency occurs in patients with primary hyperoxaluria type 1. Increased hepatic oxalate production leads to high urine concentrations of glycolate and oxalate. Calcium oxalate nephrolithiasis and nephrocalcinosis occur, and renal function progressively declines until patients develop end-stage renal disease. Renal transplantation alone is inadequate therapy because the primary enzyme deficiency remains. We report what we believe to be the second-youngest recipient to undergo successful sequential liver and kidney transplantation from a single living-related donor for treatment of primary hyperoxaluria type 1. We also discuss the changes in this patient's serum oxalate levels after transplantation.
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