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Assessment of the response to triple therapy in paediatric patients with cystic fibrosis using spirometry, sweat testing and chest computed tomography Cover

Assessment of the response to triple therapy in paediatric patients with cystic fibrosis using spirometry, sweat testing and chest computed tomography

Pneumologia
Volume 74 (2025): Issue 1 (January 2025)
By: Andreea-Valentina Stănică,  Dana-Maria Moşescu,  Simona Moşescu,  Anda Mincu and  Alexandru Ulmeanu  
Open Access
|Feb 2026

Figures & Tables

Figure 1.

Bhalla score values assessed before and after 1 year of therapy.
Bhalla score values assessed before and after 1 year of therapy.

Figure 2.

Comparative values of the parameters included in the Bhalla score.
Comparative values of the parameters included in the Bhalla score.

Figure 3.

Effectiveness of ETI treatment demonstrated by the Bhalla score parameters. ETI, elexacaftor–tezacaftor–ivacaftor.
Effectiveness of ETI treatment demonstrated by the Bhalla score parameters. ETI, elexacaftor–tezacaftor–ivacaftor.

Figure 4.

Case 1 – Axial chest CT images of a 16-year-old female patient with severe cystic fibrosis lung disease before and after initiation of triple therapy. (A) Axial CT image prior to initiation of triple therapy showing cylindrical bronchiectasis, bronchial wall thickening, mucus plugging, tree-in-bud opacities, and areas of consolidation. (B) Corresponding axial CT image after 1 year of triple therapy demonstrating persistent bronchiectasis with reduced bronchial wall thickening and mucus plugging, and resolution of previously seen consolidations. (C) Pre-treatment image demon-strating cylindrical and varicose bronchiectasis, bronchial wall thickening, and a tree-in-bud pattern consistent with distal mucus plugging. (D) Corresponding post-treatment image demonstrating persistent bronchiectasis with a significant reduction in intraluminal mucus content, decreased bronchial wall thickening, and only focal residual tree-in-bud changes. (E) Pre-treatment image demonstrating cystic (saccular) bronchiectasis, peripheral consolidations, and tree-in-bud opacities. (F) Corresponding post-treatment image showing persistent bronchiectasis with improved bronchial patency, resolution of consolidations, more homogeneous lung aeration, and only minimal residual tree-in-bud opacities.
Case 1 – Axial chest CT images of a 16-year-old female patient with severe cystic fibrosis lung disease before and after initiation of triple therapy. (A) Axial CT image prior to initiation of triple therapy showing cylindrical bronchiectasis, bronchial wall thickening, mucus plugging, tree-in-bud opacities, and areas of consolidation. (B) Corresponding axial CT image after 1 year of triple therapy demonstrating persistent bronchiectasis with reduced bronchial wall thickening and mucus plugging, and resolution of previously seen consolidations. (C) Pre-treatment image demon-strating cylindrical and varicose bronchiectasis, bronchial wall thickening, and a tree-in-bud pattern consistent with distal mucus plugging. (D) Corresponding post-treatment image demonstrating persistent bronchiectasis with a significant reduction in intraluminal mucus content, decreased bronchial wall thickening, and only focal residual tree-in-bud changes. (E) Pre-treatment image demonstrating cystic (saccular) bronchiectasis, peripheral consolidations, and tree-in-bud opacities. (F) Corresponding post-treatment image showing persistent bronchiectasis with improved bronchial patency, resolution of consolidations, more homogeneous lung aeration, and only minimal residual tree-in-bud opacities.

Figure 5.

Case 2 – CT image obtained before the initiation of triple therapy (A) in a 16-year-old female patient with moderate cystic fibrosis, showing a marked reduction in mucus plugging and improvement of bronchiectasis after 1 year of treatment (B). CT, computed tomography.
Case 2 – CT image obtained before the initiation of triple therapy (A) in a 16-year-old female patient with moderate cystic fibrosis, showing a marked reduction in mucus plugging and improvement of bronchiectasis after 1 year of treatment (B). CT, computed tomography.

Figure 6.

Case 3 – A 16-year-old male patient with mild-to-moderate cystic fibrosis (A). Resolution of mucus plugging and improvement of bronchiectasis are observed after 1 year of ETI therapy (B). ETI, elexacaftor–tezacaftor–ivacaftor.
Case 3 – A 16-year-old male patient with mild-to-moderate cystic fibrosis (A). Resolution of mucus plugging and improvement of bronchiectasis are observed after 1 year of ETI therapy (B). ETI, elexacaftor–tezacaftor–ivacaftor.

Figure 7.

Case 4 – A 15-year-old female patient with moderate cystic fibrosis presenting severe peribronchial thickening, bronchiectasis and mucus plugging (A). CT imaging performed 1 year after initiation of triple therapy (B) demonstrates a reduction in bronchial wall thickening and mucus plugging, along with improvement of bronchiectasis. CT, computed tomography.
Case 4 – A 15-year-old female patient with moderate cystic fibrosis presenting severe peribronchial thickening, bronchiectasis and mucus plugging (A). CT imaging performed 1 year after initiation of triple therapy (B) demonstrates a reduction in bronchial wall thickening and mucus plugging, along with improvement of bronchiectasis. CT, computed tomography.

Figure 8.

Increase in mean ppFEV1 value by 13.38% after 1 year of ETI therapy. ETI, elexacaftor–tezacaftor–ivacaftor.
Increase in mean ppFEV1 value by 13.38% after 1 year of ETI therapy. ETI, elexacaftor–tezacaftor–ivacaftor.

Figure 9.

Decrease in mean sweat chloride value by 43.1 mmol/L after 1 year of ETI therapy. ETI, elexacaftor–tezacaftor–ivacaftor.
Decrease in mean sweat chloride value by 43.1 mmol/L after 1 year of ETI therapy. ETI, elexacaftor–tezacaftor–ivacaftor.

Classes of CFTR gene mutations (5)_

Type of mutationType of CFTR mutationPercent of people with CF who have at least 1 mutation
NormalCFTR protein is created and moves to the cell surface, allowing the transfer of chloride and water.—
Class INo functional CFTR protein is created22%
Class IICFTR protein is created but misfolds, keeping it from moving to the cell surface. This is called a trafficking defect.88%
Class IIICFTR protein is created and moves to the cell surface but the channel gate does not open. This is called defective channel regulation.6%
Class IVCFTR protein is created and moves to the cell surface but the channel function is faulty. This is called decreased channel conductance.6%
Class VNormal CFTR protein is created and moves correctly to the cell surface but not enough amount of the protein. This is called reduced synthesis of CFTR.5%
Class VICFTR protein is created but it does not work properly at the cell membrane. This is called decreased CFTR stability.5%

Statistically significant changes in group mean values of ppFEV1, sweat chloride test, and Bhalla score_

ParameterPrePostDifference95% CIp
ppFEV1 (%)82.7996.1613.38(6.5213, 20.2351)0.002
Sweat test (mmol/L)111.5568.45–43.09(-49.9478, -36.234)< 0.001
Total Bhalla score14.5518.273.73(2.8511, 4.6035)< 0.001

Interpretation of the Bhalla score (11)_

Bhalla scoreDisease severity
16–25 pointsMild bronchiectasis
9–15 pointsModerate bronchiectasis
0–8 pointsSevere bronchiectasis

Comparison of mean values of CT parameters assessed before and after therapeutic intervention_

ScorePrePostDifference95% CIp
Bronchiectasis1.731.36–0.36(–0.6479, –0.0794)0.125
Wall thickening1.090.18–0.91(–1.3039, –0.5143)0.008
Mucus plugging1.640.55–1.09(–1.3948, –0.7877)0.002
Total Bhalla score14.5518.273.73(2.8511, 4.6035)< 0.001
DOI: https://doi.org/10.2478/pneum-2025-0040 | Journal eISSN: 2247-059X | Journal ISSN: 2067-2993
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Language: English
Page range: 149 - 158
Published on: Feb 19, 2026
Published by: Romanian Society of Pneumology
In partnership with: Paradigm Publishing Services
Publication frequency: Volume open
Keywords:
cystic fibrosis,
CFTR mutation,
ivacaftor,
tezacaftor,
elexacaftor,
Bhalla score
Related subjects:
Medicine,
Clinical medicine,
Clinical medicine, other,
Internal medicine,
Pneumology

© 2026 Andreea-Valentina Stănică, Dana-Maria Moşescu, Simona Moşescu, Anda Mincu, Alexandru Ulmeanu, published by Romanian Society of Pneumology
This work is licensed under the Creative Commons Attribution-NonCommercial-NoDerivatives 4.0 License.

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