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 . . .