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Table 1 Demographic and clinical characteristics of patients included in the EARCO registry and comparison between augmented and non-augmented PI*ZZ patients

From: Clinical and functional characteristics of individuals with alpha-1 antitrypsin deficiency: EARCO international registry

Variables

All ZZ (n = 629)

ZZ non-augmented (n = 436)

ZZ augmented (n = 190)

p value

Age, years

55.6 (13.2)

54.4 (14.0)

58.4 (10.9)

0.009

Sex, male (%)

324 (51.5)

208 (47.7)

114 (60)

0.005

BMI (kg/m2)

26 (4.8)

26 (4.8)

26.1 (4.8)

0.629

Smokers (%)

11 (1.8)

10 (2.3)

1 (0.5)

0.187

Ex-smokers (%)

356 (56.8)

202 (46.3)

152 (80.9)

< 0.001

Never smokers (%)

260 (41.5)

224 (51.4)

35 (18.6)

< 0.001

Tobacco Exposure (Pack-years)

17.8 (14)

14.2 (12.2)

22.7 (14.8)

< 0.001

Working status

 

 Active

338 (53.8)

263 (60.5)

72 (37.9)

< 0.001

 Retired

235 (37.4)

135 (31)

100 (52.6)

< 0.001

 Unemployed

30 (4.8)

15 (3.4)

15 (7.9)

0.029

 Other

25 (4.0)

22 (5.1)

3 (1.6)

0.069

Age at diagnosis of AATD

44.7 (16.7)

43.1 (17.9)

48.3 (13.0)

0.010

Reason for diagnosis

 Index case (%)

457 (72.8)

302 (69.4)

152 (80.0)

0.006

 Family screening (%)

115 (18.3)

100 (22.9)

15 (7.9)

< 0.001

Clinical characteristics

 Chronic respiratory disease (%)

508 (81.4)

317 (73.5)

188 (98.9)

< 0.001

 COPD (%)

349 (55.9)

180 (41.8)

167 (87.9)

< 0.001

 Emphysema (%)

357 (57.2)

187 (43.4)

169 (88.9)

< 0.001

 Bronchiectasis (%)

137 (22)

87 (20.2)

49 (25.8)

0.120

 Asthma (%)

88 (14.1)

74 (17.2)

14 (7.4)

0.001

 Liver disease (%)

99 (16)

69 (16.1)

30 (16)

0.981

 Charlson index (age corrected)

3.3 (1.9)

3 (1.9)

4 (1.8)

< 0.001

 Cardiovascular disease (%)

163 (26.1)

91 (20.9)

72 (38.5)

< 0.001

 Exacerbations previous year (%)

189 (30.2)

113 (25.9)

75 (39.7)

< 0.001

 History of pneumonia (%)

173 (29.3)

113 (28)

57 (30.8)

0.491

  1. Data are presented a n (% of patients with non-missing data) or mean (Standard deviation)
  2. BMI: Body mass index; COPD: Chronic obstructive pulmonary disease; AATD: Alpha-1 antitrypsin deficiency; cardiovascular disease includes hypertension, ischaemic heart disease, congestive heart failure and peripheral vascular disease