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Lung clearance index in CF: a sensitive marker of lung disease severity

¹ Department of Gene Therapy, Imperial College, London, UK
² Department of Paediatric Respiratory Medicine, Royal Brompton Hospital, London, UK
³ UK Cystic Fibrosis Gene Therapy Consortium, UK
⁴ Department of Paediatric Respiratory Medicine, Hospital for Sick Children, Edinburgh, UK
⁵ Department of Respiratory Medicine, Royal Brompton Hospital, London, UK
⁶ Respiratory Unit, Western General Hospital, Edinburgh, UK

Correspondence to:
Dr J C Davies, Department of Gene Therapy, Imperial College, London, UK; j.c.davies@imperial.ac.uk

The first 150 words of the full text of this article appear below.

Despite airways which are thought to be normal at birth, 90% of patients with cystic fibrosis (CF) ultimately die from respiratory complications of the disease. The steps involved in the progressive destruction of the airways, and strategies aimed at limiting these processes, are therefore major areas of research. For both research and clinical purposes, measures of lung involvement should ideally be (1) sensitive enough to detect abnormalities early and directly reflect changes in disease severity, either naturally occurring or in response to interventions; (2) feasible and reproducible in all age groups; and (3) repeatable over time.

With regard to the first of these criteria, there is increasing concern that conventional measures such as spirometry and chest radiography are insufficiently sensitive, particularly at the mild and moderate stages of disease. Use of these investigations has enabled a generation of clinicians to improve pulmonary status significantly, but these clinical improvements themselves serve . . . [Full text of this article]

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