Liver Transplantation in Alpha-1 Antitrypsin Deficiency

Virginia C Clark

doi:10.1016/j.cld.2016.12.008

Liver Transplantation in Alpha-1 Antitrypsin Deficiency

Clin Liver Dis. 2017 May;21(2):355-365. doi: 10.1016/j.cld.2016.12.008. Epub 2017 Feb 13.

Author

Virginia C Clark¹

Affiliation

¹ Division of Gastroenterology, Hepatology, and Nutrition, University of Florida, 1600 Southwest Archer Road, Room M440, Gainesville, FL 32601, USA. Electronic address: Virginia.Clark@medicine.ufl.edu.

PMID: 28364818
DOI: 10.1016/j.cld.2016.12.008

Abstract

Alpha-1 antitrypsin (AAT) deficiency is a common inherited metabolic disorder caused by a point mutation in the SERPIN1A gene. A small portion of homozygous PI*ZZ individuals develop severe liver disease that requires liver transplantation. Posttransplant survival is excellent. The largest burden of advanced liver disease lies within the adult population rather than children. Evaluation of lung function in adults before transplant is essential because of the underlying risk for chronic obstructive pulmonary disease. Post-liver transplantation lung function should also be monitored for decline. Although uncommon, cases of simultaneous lung and liver transplant for AAT deficiency have been reported.

Keywords: Alpha-1 antitrypsin; Chronic obstructive pulmonary disease; Liver transplantation.

Publication types

Review

MeSH terms

Disease Progression
Humans
Liver Neoplasms / etiology
Liver Neoplasms / prevention & control*
Liver Transplantation / methods*
alpha 1-Antitrypsin Deficiency / complications
alpha 1-Antitrypsin Deficiency / surgery*

Supplementary concepts

alpha-1-Antitrypsin Deficiency, Autosomal Recessive