ABSTRACT

I. Introduction Recent studies have confirmed that cystic fibrosis (CF) lung disease begins during infancy (1-3). Historically, a paucity of sensitive, repeatable physiologic techniques for evaluating early lung disease led to a lack of detection and recognition of the “silent” airway damage in CF (4,5). Considerable progress in infant pulmonary function testing (PFT) within the past 20 to 30 years has furthered the understanding of pulmonary physiology in infants and toddlers, thereby revealing previously hidden disease burden in the lungs of the youngest CF population (4-7). This chapter discusses the techniques currently available for measurement of lung function in infants, the particular challenges associated with these studies, and pertinent research findings utilizing physiologic measures in young children with CF.