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Table 2 Effects of sirolimus on rates of change in pulmonary function and arterial blood gas

From: Functional improvements in patients with lymphangioleiomyomatosis after sirolimus: an observational study

Variable

Change per month (Paired)

Before sirolimus

After sirolimus

P value

Pulmonary Function

(N = 18)

 

 FEV1 (mL)

−31.12 ± 30.78

16.11 ± 36.00

0.002

 FEV1/pred (%)

−0.95 ± 0.82

0.30 ± 0.89

< 0.001

 FVC/pred (%)

−1.13 ± 0.93

0.71 ± 1.36

0.001

 FEV1/FVC (%)

−0.82 ± 1.36

0.78 ± 2.16

0.019

 RV/pred (%)

2.50 ± 9.01

0.16 ± 8.30

0.497

 TLC/pred (%)

1.02 ± 4.49

1.15 ± 6.20

0.953

 RV/TLC (%)

0.78 ± 2.38

0.41 ± 2.58

0.688

 DLCO/pred (%)

−0.72 ± 1.34

1.10 ± 2.28

0.043

Arterial Blood Gas

(N = 17)

 

 PaO2 (mmHg)

−0.55 ± 0.60

0.30 ± 1.19

0.018

 PaCO2 (mmHg)

0.005 ± 0.327

0.058 ± 0.194

0.170

 P(A-a)O2 (mmHg)

0.31 ± 0.92

−0.75 ± 1.28

0.037

  1. Abbreviations: FEV 1 forced expiratory volume in 1 s, FVC forced vital capacity, RV residual volume, TLC total lung capacity, DL CO diffusing capacity for carbon monoxide, P a O 2 partial pressure of oxygen in arterial blood, P a CO 2 partial pressure of carbon dioxide in arterial blood, P (A-a) O 2 alveolar-arterial oxygen gradient
  2. Data are presented as mean ± SD
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Orphanet Journal of Rare Diseases

ISSN: 1750-1172