Decline in FEV1 in patients with PiZ alpha-1-antitrypsin deficiency: the Australian experience.

Objective: Alpha-1-antitrypsin (alpha1antitrypsin) deficiency is a rare hereditary disorder which characteristically presents with emphysema at an early age. The aim of the present study was to determine whether the rate of decline of lung function in alpha1antitrypsin-deficient subjects in Australia was similar to that found elsewhere.

Methodology: Patients registered with the Australian Alpha-1-Antitrypsin Replacement Program were studied.