Asset Details
Do you wish to reserve the book?
Assessment of the response to triple therapy in paediatric patients with cystic fibrosis using spirometry, sweat testing and chest computed tomography
Hey, we have placed the reservation for you!
By the way, why not check out events that you can attend while you pick your title.
Oops! Something went wrong.
Looks like we were not able to place the reservation. Kindly try again later.
Are you sure you want to remove the book from the shelf?
Assessment of the response to triple therapy in paediatric patients with cystic fibrosis using spirometry, sweat testing and chest computed tomography
Do you wish to request the book?
Assessment of the response to triple therapy in paediatric patients with cystic fibrosis using spirometry, sweat testing and chest computed tomography
Please be aware that the book you have requested cannot be checked out. If you would like to checkout this book, you can reserve another copy
We have requested the book for you!
Your request is successful and it will be processed during the Library working hours. Please check the status of your request in My Requests.
Oops! Something went wrong.
Looks like we were not able to place your request. Kindly try again later.
Journal Article
Assessment of the response to triple therapy in paediatric patients with cystic fibrosis using spirometry, sweat testing and chest computed tomography
2025
Overview
English: The availability of combination therapy with cystic fibrosis transmembrane conductance regulator (CFTR) modulators, elexacaftor–tezacaftor–ivacaftor (ETI), has significantly improved pulmonary outcomes and quality of life in patients with cystic fibrosis. This study aimed to evaluate structural bronchopulmonary changes using computed tomography (CT) after 1 year of ETI treatment, assessed by the Bhalla score, and to correlate these findings with clinical response parameters. This retrospective observational study included 11 patients under 18 years of age who underwent chest CT before and 1 year after ETI initiation. CT images were assessed using the Bhalla score. Scores range from 0 to 25, with lower values indicating more severe structural lung involvement. CT scores were correlated with changes in sweat test values and percent predicted forced expiratory volume in one second (ppFEV1). Statistical analysis included the paired Student’s t-test, Wilcoxon signed-rank test, and Pearson’s correlation coefficient. The mean Bhalla score improved from 14.55 to 18.27 (P < 0.001), with the greatest improvements in mucus plugging (P = 0.002) and peribronchial thickening (P = 0.008). Bronchiectasis improved in some patients but did not reach statistical significance. Structural changes correlated with clinical response: ppFEV1 increased by 13.38% (P = 0.002), while sweat chloride levels decreased by 43.1 mmol/L (P < 0.001). ETI therapy produced significant morphological and functional improvements. CT-detected structural changes correlated with clinical outcomes, and the Bhalla score proved useful for monitoring pulmonary disease.
Publisher
Sciendo,De Gruyter Brill Sp. z o.o., Paradigm Publishing Services
Subject
