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) deficienty (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