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Table 6 Relationship between dependent variables (FEV1, FVC, number of lung lobes, chest CT score, BSI, FACED and e-FACED) and selected characteristics. Coefficients and 95% confidence intervals (CI) calculated with univariate and multivariable linear regression models

From: Exacerbations and Pseudomonas aeruginosa colonization are associated with altered lung structure and function in primary ciliary dyskinesia

Crude slopes(95% CI)

Independent

variables

FEV1(%) Predicted value

FVC (%) Predicted value

Number of lung lobes

Chest CT score

BSI

FACED

e- FACED

Gender(males vs females)

+  8.9 (− 5.3, +  23.2)

+  0.1 (− 13.2, +  13.3)

- 0.6 (−  1.4, +  0.2)

- 4.6 (−  10.3, +  1.0)

- 1.6 (− 4.0, +  0.7)

- 0.6 (−  1.2, +  0.05)

- 0.8 (−  1.9, +  0.3)

Age(10 years)

- 5.4 (− 9.2, −  1.6)

- 5.1 (− 8.6, −  1.6)

+  0.4 (+  0.2, +  0.6)

+  2.9 (+  1.5, +  4.3)

+  0.9 (+  0.3, +  1.5)

+  0.2 (+  0.03, +  0.4)

+  0.4 (+  0.1, +  0.7)

BMI(Kg/m2)

- 1.3 (− 2.9, +  0.3)

- 1.2 (−  2.7, +  0.4)

+  0.2 (+  0.1, +  0.2)

+  1.0 (+  0.4, +  1.5)

+  0.3 (+  0.1, +  0.6)

+  0.1 (+  0.03, +  0.2)

+  0.2 (+  0.1, +  0.3)

PA colonization Yes (# 15) vs No (# 43)

- 4.6 (−  19.9, +  10.7)

- 2.0 (−  16.0, +  12.0)

+  1.0 (+  0.1, +  1.9)

+  7.9 (+  1.8, +  14.0)

+  4.2 (+  1.9, +  6.5)

+  1.3 (+  0.7, +  1.9)

+  1.7 (+  0.6, +  2.8)

Exacerbations

≥ 2 (# 36) vs

<  2 (# 22)

- 15.4 (−  30.3, −  0.5)

- 8,2 (−  22.6, +  6.1)

+  1.4 (+  0.7, +  2.6)

+  9.6 (+  4.2, +  15.0)

+  4.4 (+  2.3, +  6.6)

+  0.9 (+  0.2, +  1.5)

+  1.8 (+  0.7, +  2.8)

                                                                                                                 Slopes adjusted for gender, age, BMI, PA colonization, and exacerbations(95% CI)

Independent variables

FEV1(%) Predicted value

FVC (%)Predicted value

Number of lung lobes

Chest CT score

BSI

FACED

e- FACED

Gender(males vs females)

+  10.4 (−  3.5, +  24.3)

+  1.0 (−  12.4, +  14.4)

- 0.7 (−  1.3, +  0.1)

- 4.9 (−  9.7, −  0.1)

- 0.9 (−  2.8, +  1.0)

- 0.4 (−  0.9, +  0.1)

- 0.5 (−  1.5, +  0.4)

Age(10 years)

- 4.9 (−  9.7, −  0.2)

- 4.9 (−  9.4, −  0.4)

+  0.2 (0.00, +  0.4)

+  1.9 (+  0.2, +  3.6)

+  0.4 (−  0.3, +  1.1)

+  0.05 (−  0.1, +  0.2)

+  0.1 (−  0.2, +  0.4)

BMI(Kg/m2)

- 0.1 (−  2.0, +  1.7)

+  0.03 (−  1.8, +  1.9)

+  0.1 (+  0.01, + 0.2)

+  0.4 (−  0.3, +  1.0)

+  0.1 (−  0.1, +  0.4)

+  0.1 (−  0.01, +  0.1)

+  0.1 (−  0.03, +  0.2)

PA colonization Yes (# 15) vs No (# 43)

+  0.5 (−  14.1, +  15.1)

- 0.7 (−  14.6, +  13.3)

+  0.4 (−  0.4, +  1.1)

+  3.7 (−  1.7, +  9.1)

+  2.8 (+  0.6, +  4.9)

+  1.0 (+  0.4, +  1.6)

+  1.1 (0.0, +  2.1)

Exacerbations ≥ 2 (# 36) vs <  2 (# 22)

- 10.4 (−  25.2, +  4.4)

- 4.7 (−  19.0, +  9.7)

+  0.7 (+  0.1, +  1.4)

+  5.2 (+  0.1, +  10.3)

+  2.9 (+  0.8, +  5.0)

+  0.4 (−  0.2, +  0.4)

+  1.1 (+ 0.1, + 2.1)