Table 1.

Characteristics of patients with AATD and those receiving AAT augmentation therapy from the Alpha-1 Foundation DNA and Tissue Bank at the University of Florida, USA and the National Alpha-1 Antitrypsin Deficiency Clinic in Beaumont Hospital, Ireland

	Healthy control donors	AATD without augmentation therapy	AATD with augmentation therapy	P value
Number of participants	16	28	19
Age, years (mean±SD)	34.6±7.2	60.6±13.0	55.6±7.1	0.1546
Gender (male/female)	7/9	16/12	8/11	–
AAT genotype (MM/ZZ)	16/0	0/28	0/19	–
Baseline plasma AAT (μM)	25.0±3.0	5.2±1.8	20.0±7.0	<0.0001
Former smokers (%)	0	71.42	63.15	–
Emphysema (%)	0	35.71	63.15	–

Genotyping and phenotyping of patients with AATD are as described in the online supplemental methodology section. Data are expressed as number or mean±SD. Comparisons between patients with AATD and those receiving AAT augmentation therapy was performed using non-parametric Kolmogorov-Smirnov t-test. Plasma from healthy donors and patients was used in experiments of figure 1.

AAT, alpha-1 antitrypsin; AATD, alpha-1 antitrypsin deficiency.