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Table 1 Responses to the question about criteria for the indication of augmentation therapy for alpha-1 antitrypsin deficiency (n, %)

From: Practice and knowledge about diagnosis and treatment of alpha-1 antitrypsin deficiency in Spain and Portugal

 

Spain

Portugal

P value

PUL

IMS

PCP

P value

Total

N = 221

N = 155

N = 100

N = 100

N = 176

N = 376

Severe AATD

190 (86.0)

103 (66.5)

<0.001

96 (96.0)

77 (77.0)

120 (68.2)

<0.001

293 (77.9.0)

Emphysema

146 (66.1)

69 (44.5)

0.001

71 (71.0)

55 (55.0)

89 (50.6)

0.004

215 (57.2)

FEV1

99 (44.0)

54 (34.8)

NS

63 (63.0)

36 (36.0)

54 (30.7)

<0.001

153 (40.7)

 -<=35 %

28 (12.7)

17 (11.0)

NS

20 (20.0)

12 (12.0)

13 (7.4)

0.008

45 (12.0)

 -36–60 %

71 (32.1)

25 (16.1)

0.001

42 (42.0)

18 (18.0)

36 (20.6)

<0.001

96 (25.5)

 -61–80 %

7 (7.7)

16 (10.3)

NS

17 (17.0)

8 (8.0)

8 (4.5)

<0.001

33 (8.7)

Non smoker

99 (44.8)

56 (36.1)

NS

72 (72.0)

33 (33.0)

50 (28.1)

<0.001

155 (41.2)

Liver disease

80 (36.2)

42 (27.1)

NS

33 (33.0)

40 (40.0)

49 (27.1)

NS

122 (32.4)

Age >18 years

65 (29.4)

46 (29.7)

NS

46 (46.0)

23 (23.0)

42 (23.9)

<0.001

111 (29.5)

  1. PUL pulmonologists, IMS internal medicine specialists, PCP primary care physicians, AATD Alpha-1 antitrypsin deficiency, NS nonsignificant