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Table 2 Lung function, blood analysis, health related quality of life and physical activity of patients with PI*ZZ genotype and comparison between augmented and non-augmented subjects

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

Lung function

 

 FVC, %

91.8 (23.7)

96.1 (21.3)

81.8 (26.1)

< 0.001

 FEV1, %

66.9 (30.7)

76.3 (29.4)

45.5 (21.7)

< 0.001

 FEV1/FVC, %

0.54 (0.2)

0.59 (0.2)

0.44 (0.1)

< 0.001

 KCO, %

68 (23.2)

75.1 (22.4)

55.8 (19.1)

< 0.001

Quality of life

 CAT

13.2 (9.3)

12.5 (9.7)

14.8 (8.4)

0.001

 BODEx

2 (2.0)

1.4 (1.8)

3.3 (2.0)

 < 0.001

 EQ-5D

0.82 (0.22)

0.85 (0.20)

0.73 (0.25)

 < 0.001

 VAS

59.3 (28.2)

62.9 (28.2)

49.3 (25.7)

< 0.001

Exercise/activity

 

 Active days in last week

2.7 (2.4)

2.7 (2.3)

2.6 (2.4)

0.717

 Active days usual week

2.8 (2.4)

2.9 (2.4)

2.7 (2.4)

0.440

 Minutes walked per day

58.8 (63.9)

59 (64.2)

57.7 (63.0)

0.924

Blood analysis

 Haemoglobin

14.8 (2.4)

14.5 (2.6)

15.3 (1.7)

0.001

 White blood cells

7.0 (2.2)

6.7 (2.0)

7.6 (2.4)

< 0.001

 Neutrophils

4.5 (3.2)

4.2 (2.5)

5.1 (4.2)

0.002

 Eosinophils

0.25 (0.60)

0.22 (0.41)

0.30 (0.85)

0.121

 Platelets

237.8 (73.4)

238.7 (70.9)

237.0 (77.5)

0.571

 AAT (mg/dL)*

23.8 (8.3)

23.8 (8.7)

23.8 (7.5)

0.571

 AST (UI/L)

29.9 (17.4)

30.8 (16.7)

27.8 (13.1)

0.202

 ALT (UI/L)

29.7 (17.9)

30.1 (17.5)

28.7 (19.0)

0.474

 GGT (UI/L)

43.6 (44.1)

41.5 (45.6)

47.8 (40.8)

0.001

 FIB-4

1.6 (2.5)

1.6 (2.1)

1.8 (3.3)

0.103

  1. Values are mean (Standard deviation) unless otherwise specified
  2. 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
  3. *AAT levels were obtained at diagnosis, before initiating augmentation therapy