TABLE 1

Demographics and clinical characteristics in α1-antitrypsin deficiency patients and controls

VariableControlLow riskIntermediateHigh riskp-value
Subjects1820348
Age years11 (3–16)11 (4–14)10 (2–18)13 (6–16)0.25
Male/female11 (61%)/7 (39%)11 (55%)/9 (45%)21 (62%)/13 (38%)6 (75%)/2 (25%)0.97
AAT mg·dL−1#140 (118–212)117 (87–167)83 (52–139)23 (19–36)<0.0001
BMI kg·m−220 (14–22)19 (13–27)19 (14–26)23 (16–25)0.52
FEV1 % pred96 (88–113)92 (71–115)106 (86–145)101 (86–121)0.60
FVC % pred100 (90–108)96 (73–156)101 (79–144)105 (80–117)0.94
FEV1/FVC % pred+92 (78–114)88 (84–116)94 (84–116)84.0 (79–109)0.29
FEF25–75 % pred§82 (65–111)82 (65–128)103 (70–141)79 (66–110)0.07
DLCO % pred103 (95–104)119 (99–130)102 (101–103)0.70
AST U·L−1ƒ28 (21–37)26 (19–38)28 (19–45)26 (18–42)0.60
ALT U·L−1ƒ20 (10–40)18 (11–28)20 (14–41)24 (15–34)0.09
GGT U·L−1##15 (12–18)16 (10–24)15 (11–24)18 (16–23)0.03
  • Data are presented as median (range), unless otherwise stated. Bold indicates statistical significance (p<0.05) after application of the Kruskal–Wallis test. Comparison of proportions was performed using the Chi-squared test. AAT: α1-antitrypsin; BMI: body mass index; FEV1: forced expiratory volume in 1 s; FVC: forced vital capacity; FEF25–75: forced expiratory flow at 25–75% of FVC; DLCO: diffusing capacity of the lung for carbon monoxide; AST: aspartate aminotransferase; ALT: alanine aminotransferase; GGT: γ-glutamyl-transferase. #: normal range 90–200 mg·dL−1; : normal ≥80% predicted; +: normal >80% predicted; §: normal ≥65% predicted; ƒ: normal range 1–37 U·L−1; ##: normal range 1–55 U·L−1.