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Methods

A total of five healthy control subjects (mean±SD age, 10.8±3.9 yr) and 22 clinically stable patients with CF (age, 14.5±10.6 yr) were imaged under an Institutional Review Board–approved protocol, with a Food and Drug Administration Investigational New Drug (123577) for 129 Xe; informed consent was obtained from adult subjects or parents, and age-appropriate assent from pediatric subjects (demographic data in Table 1). Spirometry was obtained in each subject before imaging according to American Thoracic Society and European Respiratory Society guidelines. Subjects were imaged on a Philips 3T Achieva (Philips Healthcare) by coaching subjects to FRC before inhalation of a breath of HP 129 Xe (one-sixth TLC, based on height and sex [7]) for ventilation imaging via a gradient-echo sequence (two-dimensional multislice, TR/TE= 8/4 ms; voxel size= 3× 3× 15 mm 3; readouts= 96–196; phase …