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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.