Liver Transplantation in Alpha-1 Antitrypsin Deficiency.

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.