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Monitoring of Structure and Function in Early Cystic Fibrosis Lung Disease.

Concurrent with improving survival in cystic fibrosis (CF), lung function has also steadily improved. Forced expiratory volume in one second (FEV1), the measure of airway obstruction most widely monitored in patients with CF, often does not fall below the normal range until late adolescence. Clearly, FEV1 is losing relevance for clinicians monitoring lung disease in children with CF, as it is frequently normal, declines slowly, and cannot be measured by conventional techniques in children <6 years of age. As clinicians increasingly aim to identify and treat early CF lung disease before irreversible structural airway damage occurs, there is a compelling need for better tools with which to monitor for early lung structural and physiologic abnormalities in our youngest CF patients. Three promising measures are infant and preschool lung function testing, chest computed tomography, and multiple breath washout measures of the lung clearance index. The current state of these tests as they relate to the clinical monitoring of early CF lung disease is reviewed, and improvements on the horizon that may allow them to be adopted in clinical practice explained.