Abstract
This chapter will address the pathogenic mechanisms of the disease, the way they influence lung function, and how it interferes with the way noninvasive ventilation is applied in CF patients.
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Abbreviations
- ADA:
-
American Diabetes Association
- CF:
-
Cystic fibrosis
- CFF:
-
Cystic Fibrosis Foundation
- CFTR:
-
CF transmembrane conductance regulator
- CPET:
-
Cardiopulmonary exercise testing
- FEV 1:
-
Forced expiratory volume in 1 s
- FOT:
-
Forced oscillation techniques
- FRC:
-
Functional residual capacity
- LCI:
-
Lung clearance index
- NIV:
-
Noninvasive ventilation
- PES:
-
Pediatric Endocrine Society
References
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Horsley AR, Davies JC, Gray RD, et al. Changes in physiological, functional and structural markers of cystic fibrosis lung disease with treatment of a pulmonary exacerbation. Thorax. 2013;68:532–9.
Boucher RC. New concepts of the pathogenesis of cystic fibrosis lung disease. Eur Respir J. 2004;23:146–58.
Horsley A, Siddiqui S. Putting lung function and physiology into perspective: cystic fibrosis in adults. Respirology. 2015;20:33–45.
Moran F, Bradley JM, Piper AJ. Non-invasive ventilation for cystic fibrosis. Cochrane Database Syst Rev. 2017;2:CD002769.
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Moreira, I., Magalhães, A., Cabral, M. (2021). Cystic Fibrosis. In: Esquinas, A.M. (eds) Pulmonary Function Measurement in Noninvasive Ventilatory Support. Springer, Cham. https://doi.org/10.1007/978-3-030-76197-4_17
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DOI: https://doi.org/10.1007/978-3-030-76197-4_17
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