Longitudinal follow-up of patients with α1-protease inhibitor deficiency before and during therapy with IV α1-protease inhibitor

M Wencker, B Fuhrmann, N Konietzko, N Banik - Chest, 2001 - Elsevier
Background: The efficacy of IV augmentation therapywith human α 1-protease inhibitor (α 1-
Pi) inpatients with severe α 1-Pi deficiency is still underdebate. Study objectives: To evaluate
the progressionof emphysema in patients with α 1-Pi deficiency before and during a period
in which they received treatment withα 1-Pi. Design: Multicenter, retrospective cohort study.
Settings: Outpatientclinics of 26 university clinics and pulmonary hospitals. Patients: Ninety-
six patients with severeα 1-Pi deficiency receiving weekly augmentation therapywith human …