Adult liver transplantation for metabolic liver disease

Clin Liver Dis. 1998 Feb;2(1):187-210. doi: 10.1016/s1089-3261(05)70371-8.


Liver replacement provides an effective method of replacing a failing liver, and corrects the underlying defect in many metabolic conditions. Results of liver transplantation for metabolic diseases have been encouraging, with the exception of hereditary hemochromatosis, in which infectious and for which cardiac complications appear to increase posttransplant mortality. An improved understanding of the underlying genetic and molecular defect will lead to advances in medical therapy and perhaps will decrease the need for liver replacement. The prospects of gene therapy are being pursued for many metabolic disorders, however until this research leads to direct clinical application, liver transplantation remains the only effective option for many patients with metabolic liver disease.

Publication types

  • Review

MeSH terms

  • Amyloidosis / complications
  • Amyloidosis / metabolism
  • Copper / toxicity
  • Hemochromatosis / complications
  • Hemochromatosis / metabolism
  • Hepatolenticular Degeneration / complications
  • Hepatolenticular Degeneration / metabolism
  • Humans
  • Hyperoxaluria, Primary / complications
  • Hyperoxaluria, Primary / metabolism
  • Liver Diseases / metabolism
  • Liver Diseases / surgery*
  • Liver Transplantation*
  • Ornithine Carbamoyltransferase Deficiency Disease / complications
  • Ornithine Carbamoyltransferase Deficiency Disease / metabolism
  • alpha 1-Antitrypsin Deficiency / complications
  • alpha 1-Antitrypsin Deficiency / metabolism


  • Copper