Intravenous alpha-1-antitrypsin replacement therapy: case report after one year of treatment.

Alpha-1-antitrypsin deficiency is an autosomally inherited disease in which individuals homozygous for the disorder are prone to develop severe emphysema. We report the case of a 43 yr old man with severe deficiency and advanced emphysema and the first experience in Ireland with intravenous alpha-1-antitrypsin replacement. After 1 yr of replacement therapy we report our experience with dosage and frequency of infusion as well as pulmonary function test data before and during treatment.