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Table 1 Patient characteristics

From: Health status decline in α-1 antitrypsin deficiency: a feasible outcome for disease modifying therapies?

 

No Obstruction

Obstruction

p

N

Median

IQR

N

Median

IQR

Male n(%)

33 (39%)

235 (64%)

< 0.001

Age

84

42.4

35.5–53.9

370

52.5

46.4–58.5

< 0.001

Index n(%)

39 (46%)

331 (89%)

< 0.001

Never Smoker n(%)

59 (70%)

72 (19%)

< 0.001

Pack Year History

25

6.0

2.0–12.8

296

19.0

10.0–28.0

< 0.001

BMI

84

25.9

23.1–29.9

370

25.1

22.8–27.9

0.043

Baseline

 FEV1% predicted

84

113.8

97.9–123.0

370

49.2

36.5–66.1

< 0.001

 FVC % predicted

84

115.0

102.2–129.0

370

108.3

94.8–124.3

0.025

 FEV1 / FVC Ratio

84

80.6

77.2–86.0

370

37.6

30.3–48.0

< 0.001

 Tlco % predicted

84

92.5

80.8–108.9

368

66.2

52.5–77.1

< 0.001

 Kco % predicted

84

91.1

79.3–100.3

368

63.6

52.1–74.3

< 0.001

 SGRQ Symptoms

84

30.9

11.7–57.1

370

62.5

46.2–78.6

< 0.001

 SGRQ Activity

84

12.2

0–41.6

370

60.4

47.4–79.7

< 0.001

 SGRQ Impacts

84

5.8

0–21.4

370

34.9

21.3–49.9

< 0.001

 SGRQ Total

84

14.0

4.8–35.5

370

48.2

33.9–62.4

< 0.001

Annual Decline

 FEV1% predicted Slope/yr

84

−0.25

−1.11 – 0.47

370

−1.02

−1.99 – − 0.03

< 0.001

 Kco % predicted Slope/yr

84

−0.92

−1.66 – 0.01

364

−1.13

−1.94 – −0.42

0.063

 SGRQ Symptoms Slope/yr

84

0.00

−2.45 – 2.02

370

0.21

−2.29 – 2.15

0.291

 SGRQ Activity Slope/yr

84

0.00

−0.78 – 1.37

370

1.18

−0.47 – 3.62

< 0.001

 SGRQ Impacts Slope/yr

84

0.14

−0.53 – 0.94

370

0.38

−1.07 – 2.18

0.092

 SGRQ Total Slope/yr

84

0.21

−0.76 – 1.06

370

0.66

−0.83 – 2.37

0.025

  1. Demographics of the patient group divided into those with and without airflow obstruction (FEV1/FVC ratio above and below 0.7). Data is expressed as median and interquartile range. The annual % predicted decline in lung function and St George’s Respiratory Questionnaire domains are also shown
  2. BMI Body mass index, FEV1 Forced Expiratory Volume in 1 Second, FVC Forced Vital Capacity, Tlco Diffusing Capacity of the Lung for carbon monoxide, Kco Transfer Coefficient for carbon monoxide, SGRQ St George’s Respiratory Questionnaire