Reversal by Liver Transplantation of the Complications of Primary Hyperoxaluria as Well as the Metabolic Defect

Abstract

LIVER transplantation reverses a number of inborn errors of metabolism that result from the genetically determined absence of an enzyme.¹ The earliest enzyme-deficiency disorders to be treated by liver transplantation were those that caused the anatomical destruction of the host liver. The replacement of a grossly normal liver to correct a defect that had caused the destruction of another organ was first performed to treat homozygous familial hypercholesterolemia.² Although homozygous familial hypercholesterolemia is not the result of an enzyme defect, this experience undoubtedly encouraged others to attempt to correct primary hyperoxaluria in the same way.^{3 4 5} Three patients with primary hyperoxaluria . . .