Early Detection of Lung Disease in Children with Cystic Fibrosis Using Lung Function

Ranganathan, Sarath; Linnane, Barry; Nolan, Gary; Gangell, Catherine; Hall, Graham

doi:10.1016/j.prrv.2008.05.002

« Previous Next »Paediatric Respiratory Reviews
Volume 9, Issue 3 , Pages 160-167, September 2008

Early Detection of Lung Disease in Children with Cystic Fibrosis Using Lung Function

Sarath Ranganathan
- Department of Respiratory Medicine, Royal Children's Hospital Melbourne, Australia
- Infection, Immunity & Environment Theme, Murdoch Children's Research Institute, Melbourne, Australia
- Department of Paediatrics, University of Melbourne, Australia
Barry Linnane
- Department of Respiratory Medicine, Royal Children's Hospital Melbourne, Australia
- Infection, Immunity & Environment Theme, Murdoch Children's Research Institute, Melbourne, Australia
Gary Nolan
- Respiratory Medicine, Princess Margaret Hospital for Children, Perth, Australia
- Clinical Sciences, Telethon Institute for Child Health Research, Centre for Child Health Research, University of Western Australia, Perth, Australia
Catherine Gangell
- Clinical Sciences, Telethon Institute for Child Health Research, Centre for Child Health Research, University of Western Australia, Perth, Australia
- School of Paediatric and Child Health, University of Western Australia, Perth, Australia
Graham Hall
- Respiratory Medicine, Princess Margaret Hospital for Children, Perth, Australia
- Clinical Sciences, Telethon Institute for Child Health Research, Centre for Child Health Research, University of Western Australia, Perth, Australia
- School of Paediatric and Child Health, University of Western Australia, Perth, Australia
- Corresponding author. Respiratory Medicine, Princess Margaret Hospital, GPO Box D184, Perth WA 6840, Australia. Tel.: +61 8 9340 8830; Fax: +61 8 9340 8181.

Summary

Measurement of lung function is routine in older children and adults with cystic fibrosis (CF) but not in infants and preschool children. Pulmonary infection, neutrophil-dominated inflammation and clinical exacerbations in young children similar to those seen in older subjects have been identified and highlight the urgent need to evaluate lung function in early life. Mounting evidence suggests lung function techniques sensitive to changes in peripheral lung function may be required to detect the early functional abnormalities in infants and preschool children with CF. In addition, the majority of studies in young children with CF have not reported longitudinal data and therefore the prognostic potential of existing lung function methods to track disease progression is poorly understood. This review aims to describe recent research findings in infants and preschool children and to outline currently available lung function techniques, issues around their standardization and their relative advantages and disadvantages in young children with CF.

Keywords: Cystic fibrosis, Infant lung function, Pre-school lung function, Respiratory physiology