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Table 1 Demographic data of the study population and the results of lung function tests.

From: Survival in severe alpha-1-antitrypsin deficiency (PiZZ)

  All
n (%)
R cases
(n = 574)
NR cases
(n = 431)
S cases
(n = 334)
Men (%) 48 52 44 49
Smoking status     
Smokers, no. (%) 737 (55) 423 (74) 177 (41) 137 (41)
Never-smokers no. (%) 602 (45) 151 (26) 254 (59) 197 (59)
Respiratory symptoms at inclusion, no. (%) 839 (63) 574 166 (38) 99 (30)
Deaths, no. (%) 315 (24) 212 (37) 79 (18) 24 (7)
Lung transplant (no.) 56 51 3 2
Liver transplant (no.) 2 - 2 -
Mean (range) age at diagnosis (years) 41 (0-88) 51 (0.1-88) 40 (0.01-84) 24 (0-77)
Mean (range) age at inclusion (years) 47 (18-89) 54 (18-89) 45 (18-87) 35 (18-78)
Mean (range) follow-up time (years) 9 (0.02-17) 8 (0.02-17) 10 (0.02-17) 11 (0.07-17)
Mean age (range) at death 67 (20-95) 67 (30-95) 69 (20-90) 61 (37-85)
Mean (SD) FEV1 (% predicted) 72 (33) 50 (29)* 88 (25) 89 (23)
Mean (SD) FVC (% predicted) 84 (23) 75 (25)* 89 (18) 93 (16)
Mean (SD) FEV1/FVC ratio (%) 63 (23) 48 (22)* 75 (17) 76 (15)
  1. R: respiratory. NR: non-respiratory. S: screened.
  2. *p < 0.001 vs. NR cases and S cases.