Assessment and monitoring of cystic fibrosis lung disease in infants and young children

Abstract

Chronic airway infection and inflammation are the hallmarks of cystic fibrosis (CF) lung disease. As these events occur early in life, it is critical to develop techniques for the assessment and monitoring of early-CF lung disease in infants and young children. In the last several years, there have been major advances in the development of imaging technology to assess structural damage in CF lung disease, noninvasive markers of CF airway inflammation and measurement of lung function in infants and young children with CF. In this article, we will review these advances and techniques, and discuss future directions for research and clinical applications.

Keywords::

• computed tomography
• inflammation
• lung function
• magnetic resonance imaging
• oscillometry

Financial & competing interests disclosure

CL Ren is a consultant to Genentech, Inc. regarding the use of preschool pulmonary function testing as an outcome measure for cystic fibrosis clinical trials. The author has no other relevant affiliations or financial involvement with any organization or entity with a financial interest in or financial conflict with the subject matter or materials discussed in the manuscript apart from those disclosed.

No writing assistance was utilized in the production of this manuscript.

Notes

FVC: Forced vital capacity; PEF: Peak expiratory flow.

Adapted from [81,82].