Preemptive Liver Transplantation from a Living Related Donor for Primary Hyperoxaluria Type I

To the Editor: Primary hyperoxaluria type I is an autosomal recessive disorder characterized by a deficiency of liver-specific peroxisomal alanine–glyoxylate aminotransferase (EC 2.6.1.44). The disease leads to increased urinary excretion of oxalate, causing urolithiasis, nephrocalcinosis, progressive renal insufficiency, and eventually, oxalosis — that is, the accumulation of insoluble oxalate throughout the body. The interval between the onset of symptoms and the development of end-stage renal failure varies. Although optimal conservative therapy can delay end-stage renal failure, it usually cannot prevent it. Kidney transplantation to correct end-stage renal failure is frequently followed by recurrence of the disease in the transplanted kidney. . . .