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Table 4 Lung function, blood analysis, health related quality of life and physical activity of PI*ZZ patients according to their impairment in FEV1(%), KCO(%) or both

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

Variables

Impaired PFTs (n = 248)

Impaired KCO(%) (n = 69)

Impaired FEV1(%) (n = 37)

Normal PFTs (n = 95)

p value

Lung function

 FVC, %

84.7 (22.3)

111.8 (19.9)

82.6 (21.3)

103.9 (13.4)

< 0.001acdf

 FEV1, %

44.9 (16.8)

101.2 (17.8)

54.6 (20.1)

101.8 (12.3)

< 0.001acdf

 FEV1/FVC, %

0.42 (0.12)

0.72 (0.11)

0.51 (0.13)

0.80 (0.08)

< 0.001abcdf

 KCO, %

53.2 (13.9)

65.5 (10.4)

97.4 (14.4)

96.7 (12.1)

< 0.001abcde

Quality of life

 CAT

16.7 (8.5)

8.6 (7.1)

12.9 (8.9)

5.9 (6.7)

< 0.001acf

 BODEx

3.1 (1.9)

0.4 (0.7)

1.9 (1.9)

0.2 (0.4)

< 0.001abcdf

 EQ-5D

0.74 (0.24)

0.9 (0.14)

0.81 (0.18)

0.93 (0.10)

< 0.001acf

 VAS

53.6 (24.1)

73.1 (17.0)

61.6 (24.1)

76.4 (26.6)

< 0.001acf

Exercise/activity

 Active days in last week

2.7 (2.4)

2.7 (2.4)

2.7 (2.3)

2.6 (2.2)

0.994

 Active days usual week

2.8 (2.4)

3.0 (2.3)

3.1 (2.4)

2.7 (2.1)

0.822

 Minutes walked per day

56.9 (67.7)

60.2 (49.2)

77.3 (76.4)

64.6 (59.6)

0.034

Blood analysis

 Haemoglobin

15.4 (1.6)

14.7 (1.4)

15.1 (1.9)

14.1 (2.6)

< 0.001acf

 White blood cells

7.5 (2.1)

6.2 (1.7)

6.8 (1.7)

6.1 (1.8)

< 0.001ac

 Neutrophils

4.8 (2.5)

4.1 (4.7)

5.2 (7.3)

3.5 (1.3)

< 0.001ac

 Eosinophils

0.23 (0.35)

0.18 (0.22)

0.27 (0.54)

0.24 (0.59)

0.175

 Platelets

239.2 (75.7)

216.8 (65.0)

235.9 (56.0)

235.8 (66.8)

0.134

 AAT (mg/dL)*

24.4 (7.6)

22.1 (9.2)

27.1 (10)

23.3 (7.5)

0.012d

  AST (UI/L)

29.3 (14.3)

36.3 (34.3)

31.3 (13.8)

27.5 (13.9)

0.159

 ALT (UI/L)

29.4 (18.6)

30.3 (16.7)

30.7 (15.2)

29.3 (19.5)

0.561

 GGT (UI/L)

52.7 (52.1)

42.2 (49.7)

42.5 (39.3)

30.3 (25.2)

< 0.001f

 FIB-4

1.7 (3)

2.4 (4.2)

1.4 (0.7)

1.1 (0.7)

< 0.001ef

  1. Impairment is considered when values are < 80% of reference
  2. Values are mean (Standard deviation) unless otherwise specified
  3. FVC: Forced vital capacity; FEV1: Forced expiratory volume in 1 s; KCO: Carbon monoxide transfer coefficient; CAT: COPD assessment test; BODEx: Body mass index, obstruction, dyspnoea, exacerbations index; EQ-5D: Euro QoL 5 dimensions questionnaire; VAS: Visual analogue scale; AST: Aspartate aminotransferase; ALT: Alanine aminotransferase; GGT: Gamma-glutamyl transferase; FIB-4: Fibrosis 4
  4. *AAT levels were obtained at diagnosis, before initiating augmentation therapy
  5. Bonferroni ap < 0.05 for comparison between the groups “Impaired PFTs and Impaired KCO(%)”. bp < 0.05 for comparison between the groups “Impaired PFTs and impaired FEV1(%)”. cp < 0.05 for comparison between the groups “Impaired PFTs and normal PFTs”. dp < 0.05 for comparison between the groups “Impaired KCO(%) and impaired FEV1(%)”. ep < 0.05 for comparison between the groups “Impaired KCO(%) and normal PFTs”. fp < 0.05 for comparison between the groups “Impaired FEV1(%) and normal PFTs”