Long term effect on lung function of alpha 1-protease inhibitor substitution therapy in COPD patients with Pi ZZ phenotype.

Eight patients suffering from alpha 1-protease inhibitor (alpha 1-PI) deficiency (Pi ZZ phenotype) and chronic obstructive lung disease received substitution therapy (60 mg.kg-1 weekly) for a period of up to 30 months. Intrathoracic gas volume, airway resistance and arterial oxygen tension were followed once every three months. There was no trend for these indices to deteriorate or improve over the period of observation.