Table 1 Characteristics of people with CF who received a CT-based body composition analysis at baseline and after initiation of ETI therapy.

From: Elexacaftor/tezacaftor/ivacaftor influences body composition in adults with cystic fibrosis: a fully automated CT-based analysis

n = 66

Baseline (T0)

ETI (T1)

p value

Age, years

35 (27.75; 42.5)

  

Female sex, n (%)

31 (47)

  

FEV1 (% predicted)

37 (28; 49.5)

47.5 (36.25; 63)

 < 0.001

FEV1 [L]

1.35 (0.97; 1.9)

1.70 (1.22; 2.27)

 < 0.001

BMI, kg/m2

19.5 (18; 21.95)

21.8 (20; 23.75)

 < 0.001

 Underweight, n (%)

20 (30)

5 (8)

0.003

 Normal, n (%)

40 (61)

52 (79)

 

 Overweight, n (%)

5 (8)

9 (14)

 

Body weight, kg

58 (52, 64)

62 (57, 72)

 < 0.001

Body height, m

1.73 (1.65; 1.77)

1.72 (1.64, 1.77)

0.500

Body surface area, m2

1.68 (1.58; 1.79)

1.71 (1.61; 1.89)

 < 0.001

Sweat chloride, mmol/L*

102 (92.5; 110)

46 (39; 63)

 < 0.001

CFTR genotype, n (%)

 Homozygous dF508

38 (58)

  

 Heterozygous dF508

25 (38)

  

 Other**

3 (5)

  

Prior CFTR modulator therapy, n (%)

 Mono-/dual-combination***

33 (50)

  

 None

33 (50)

  

Chest CT scan

 Before/after ETI, days

52 (0;209.25)

529 (357.75; 648.25)

 

Indication for CT scan, n (%)

  

0.999

 Pulmonary exacerbation

10 (15)

9 (14)

 

 Hemoptysis

3 (5)

4 (6)

 

 Other (elective CT scan)****

53 (80)

53 (80)

 
  1. Values are median (first quartile, third quartile) or number of patients (%). P-value was determined using Wilcoxon signed rank test or Pearson Chi-squared test. * p < 0.05 ** p < 0.01 *** p < 0.001.CF, cystic fibrosis; FEV1, forced expiratory volume in 1 s; CFTR, cystic fibrosis transmembrane conductance regulator. *paired data available for 64 (pulmonary function, BMI, body weight) and 35 (sweat chloride) participants, respectively. **other: R553X/I336K, G542X/3849 + 10KbC- > T, R553X/3121-2A > G. ***mono-/dual-combination CFTR modulators: ivacaftor, lumacaftor/ivacaftor, tezacaftor/ivacaftor. ****reasons for elective CT scans: cancer staging, transplantation listing/cancer screening, monitoring of nontuberculous mycobacteria-/fungal-related lesions and allergic bronchopulmonary aspergillosis, fatigue of unknown reason, evaluation of pulmonary infiltrates, abnormal laboratory results, non-acute decline of pulmonary function or surveillance.