Effect of Salbutamol on Lung Ventilation in Children with Cystic Fibrosis: Comprehensive Assessment Using Spirometry, Multiple-Breath Washout, and Functional Lung Magnetic Resonance Imaging.

Kieninger, Elisabeth; Willers, Corin; Röthlisberger, Katrin; Yammine, Sophie; Pusterla, Orso; Bauman, Grzegorz; Stranzinger, Enno; Bieri, Oliver; Latzin, Philipp; Casaulta, Carmen (2022). Effect of Salbutamol on Lung Ventilation in Children with Cystic Fibrosis: Comprehensive Assessment Using Spirometry, Multiple-Breath Washout, and Functional Lung Magnetic Resonance Imaging. Respiration, 101(3), pp. 281-290. Karger 10.1159/000519751

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BACKGROUND

Inhalation therapy is one of the cornerstones of the daily treatment regimen in patients with cystic fibrosis (CF). Recommendations regarding the addition of bronchodilators, especially salbutamol are conflicting due to the lack of evidence. New diagnostic measures such as multiple-breath washout (<underline>MBW)</underline> and functional magnetic resonance imaging (MRI) have the potential to reveal new insights into bronchodilator effects in patients with CF.

OBJECTIVE

The objective of the study was to comprehensively assess the functional response to nebulized inhalation with salbutamol in children with CF.

METHODS

Thirty children aged 6-18 years with stable CF performed pulmonary function tests, MBW, and matrix pencil-MRI before and after standardized nebulized inhalation of salbutamol.

RESULTS

Lung clearance index decreased (improved) by -0.24 turnover (95% confidence interval [CI]: -0.53 to 0.06; p = 0.111). Percentage of the lung volume with impaired fractional ventilation and relative perfusion decreased (improved) by -0.79% (CI: -1.99 to 0.42; p = 0.194) and -1.31% (CI: -2.28 to -0.35; p = 0.009), respectively. Forced expiratory volume (FEV1) increased (improved) by 0.41 z-score (CI: 0.24-0.58; p < 0.0001). We could not identify specific clinical factors associated with a more pronounced effect of salbutamol.

CONCLUSIONS

There is a positive short-term effect of bronchodilator inhalation on FEV1 in patients with CF, which is independent of ventilation inhomogeneity. Heterogeneous response between patients suggests that for prediction of a therapeutic effect this should be tested by spirometry in every patient individually.

Item Type:

 Journal Article (Original Article)

Division/Institute:

 04 Faculty of Medicine > Department of Gynaecology, Paediatrics and Endocrinology (DFKE) > Clinic of Paediatric Medicine
04 Faculty of Medicine > Department of Radiology, Neuroradiology and Nuclear Medicine (DRNN) > Institute of Diagnostic, Interventional and Paediatric Radiology
04 Faculty of Medicine > Department of Gynaecology, Paediatrics and Endocrinology (DFKE) > Clinic of Paediatric Medicine > Paediatric Pneumology

Graduate School:

 Graduate School for Cellular and Biomedical Sciences (GCB)

UniBE Contributor:

 Kieninger, Elisabeth, Willers, Christoph Corin, Yammine, Sophie, Stranzinger, Enno, Latzin, Philipp, Casaulta, Carmen

Subjects:

 600 Technology > 610 Medicine & health

ISSN:

 0025-7931

Publisher:

 Karger

Language:

 English

Submitter:

 Anette van Dorland

Date Deposited:

 07 Dec 2021 09:28

Last Modified:

 05 Dec 2022 15:55

Publisher DOI:

 10.1159/000519751

PubMed ID:

 34808631

Uncontrolled Keywords:

 Bronchodilator intervention study Children Cystic fibrosis Functional magnetic resonance imaging Lung clearance index Lung function

BORIS DOI:

 10.48350/161474

URI:

 https://boris.unibe.ch/id/eprint/161474

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