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Paediatric Pulmonology and Allergology
2007 April, Vol. X, No. 1 (3465-3485)
EARLY DETECTION AND MONITORING OF LUNG DISEASE IN YOUNG INFANTS WITH CYSTIC FIBROSIS
Andrew Bush
Imperial School of Medicine at National Heart and Lung Institute and Department of Paediatric Respiratory Medicine, Royal Brompton Hospital, UK
Babies with CF are born with essentially normal lungs, but rapidly develop inflammation and infection. Evaluation is generally on history and physical examination until school age, when children become able to perform spirometry. However, in these ‘silent years’ unrecognised lung damage can occur. Recently, techniques to improve the evaluation of infants and preschool children have been developed, and an assessment of where they fit into the clinical and research practice is now needed. Recent advances in the understanding of the nature of early CF lung disease are discussed first in this article, and then the different techniques available for evaluation are reviewed. These include physiological methods, particularly indices of gas mixing; imaging techniques, especially high resolution computed tomography scanning; bronchoscopic studies; and tests based on sputum, blood and exhaled breath condensate. Particularly important in the latter are non-invasive measurements of inflammation, and ways of better detecting infection short of bronchoalveolar lavage. Some patients currently require sophisticated apparatus (lung clearance index). A matter of great concern is to know what techniques are sufficiently reproducible, and indicative of prognosis, for use as end-points in clinical trials, so good long term, prospective comparisons of these techniques, with measurement of inflammatory markers are expected in the future.