Liver Transplantation in Alpha-1 Antitrypsin Deficiency.

Virginia C Clark

Journal: Clinics in liver disease 2018;21(2):355-365

PMID: 28364818

Full Text on Elsevier Science View on PubMed

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.

Address: Division of Gastroenterology, Hepatology, and Nutrition, University of Florida, 1600 Southwest Archer Road, Room M440, Gainesville, FL 32601, USA. Electronic address: [email protected].

DOI: 10.1016/j.cld.2016.12.008