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Putting lung function and physiology into perspective: cystic fibrosis in adults
Author(s) -
Horsley Alex,
Siddiqui Salman
Publication year - 2015
Publication title -
respirology
Language(s) - English
Resource type - Journals
SCImago Journal Rank - 1.857
H-Index - 85
eISSN - 1440-1843
pISSN - 1323-7799
DOI - 10.1111/resp.12382
Subject(s) - medicine , cystic fibrosis , air trapping , bronchiectasis , small airways , lung , pulmonary function testing , airway , disease , airway obstruction , lung function , lung volumes , magnetic resonance imaging , pathology , intensive care medicine , cardiology , radiology , surgery
Adult cystic fibrosis ( CF ) is notable for the wide heterogeneity in severity of disease expression, both between patients and within the lungs of individuals. Although CF airways disease appears to start in the small airways, in adults there is typically widespread bronchiectasis, increased airway secretions, and extensive obstruction and inflammation of the small airways. The complexity and heterogeneity of airways disease in CF means that although there are many different methods of assessing and describing lung ‘function’, none of these single‐dimensional tests is able to provide a comprehensive assessment of lung physiology across the spectrum seen in adult CF . The most widely described measure, the forced expiratory volume in 1 s, remains a useful and simple clinical tool, but is insensitive to early changes and may be dissociated from other more detailed assessments of disease severity such as computed tomography. In this review, we also discuss the use of more sensitive novel assessments such as multiple breath washout tests and impulse oscillometry, as well as the role of cardiopulmonary exercise testing. In the future, hyperpolarized gas magnetic resonance imaging techniques that combine regional structural and functional information may help us to better understand these measures, their applications and limitations.