Renal transplantation in primary hyperoxaluria

Type I primary hyperoxaluria is a rare autosomal recessive disorder of gloxylate matabolism that ultimately leads to chronic renal insufficiency.¹While numerous forms of therapy have been tried, none has been shown to halt the inexorable progression of the renal lesion. We report a patient with renal failure secondary to primary hyperoxaluria. The patient received a renal transplant, and we review the literature on transplantation in this metabolic disorder. Patient Summary Patient 1. —A 30-year-old white man was transferred to the Peter Bent Brigham Hospital on March 30,1971, to be evaluated for long-term hemodialysis and kidney transplantation. When two years old, he first passed a renal stone. Renal colic recurred when the patient was 12 years old and in the ensuing years he passed numerous stones. When he was 18 years old, the patient had a blood urea nitrogen (BUN) concentration of 50 mg/100 ml and bilateral nephrolithiasis. In