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Table 3 Pulmonary Structural and Functional Characteristics of Patients with Lymphangioleiomyomatosis

From: Assessing the outcomes of everolimus on renal angiomyolipoma associated with tuberous sclerosis complex in China: a two years trial

Value

Baseline

(n = 6)

12 months

(n = 6)

24 months

(n = 5)

Change from Baseline

12 months (n = 6)

24 months (n = 5)

FEV1

 Least-square mean — liters

1.59 ± 0.41

1.87 ± 0.47

1.65 ± 0.45

0.28 ± 0.08 (P<0.001)

0.13 ± 0.05 (P = 0.004)

 Percent of predicted value

58.51 ± 11.09

65.32 ± 12.32

59.89 ± 11.58

6.81 ± 2.28 (P<0.001)

3.46 ± 1.57 (P = 0.008)

FVC

 Least-square mean — liters

2.77 ± 0.56

3.20 ± 0.68

2.95 ± 0.66

0.43 ± 0.17 (P = 0.002)

0.27 ± 0.14 (P = 0.013)

Percent of predicted value

79.90 ± 12.68

87.16 ± 12.63

84.36 ± 13.71

7.26 ± 2.16 (P<0.001)

6.39 ± 1.36 (P<0.001)

Total lung capacity

 Least-square mean — liters

5.16 ± 0.51

5.18 ± 0.44

5.14 ± 0.49

0.03 ± 0.12 (P = 0.62)

0.04 ± 0.10 (P = 0.43)

 Percent of predicted value

99.21 ± 7.01

99.70 ± 7.28

97.75 ± 7.24

0.49 ± 1.47 (P = 0.45)

−0.22 ± 0.78 (P = 0.57)

Residual volume

 Least-square mean — liters

2.39 ± 0.10

1.98 ± 0.31

2.11 ± 0.29

−0.41 ± 0.24 (P = 0.009)

−0.31 ± 0.23 (P = 0.036)

 Percent of predicted value

124.56 ± 10.04

104.23 ± 8.85

112.30 ± 5.41

−20.32 ± 1.62 (P<0.001)

−15.79 ± 2.38 (P<0.001)

DLco

 Least-square mean — ml/mmHg/min

12.93 ± 2.42

13.00 ± 2.41

12.59 ± 2.39

0.07 ± 0.46 (P = 0.71)

−0.21 ± 0.55 (P = 0.43)

 Percent of predicted value

53.40 ± 9.29

53.60 ± 8.70

54.27 ± 9.56

0.19 ± 1.39 (P = 0.74)

1.68 ± 2.11 (P = 0.15)

  1. Data are listed for 6 patients with lymphangioleiomyomatosis who completed the 12-month study visit. Data for the 24-month study visit were available for 5 of these 6 patients; 1 patient had withdrawn during the second year. Changes from baseline were not significant unless otherwise indicated. DLco denotes diffusing capacity of the lung for carbon monoxide, FEV1 forced expiratory volume in 1 s, FVC forced vital capacity