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Objective: To determine the sex-specific relationships between glucose intolerance and Shwachman-Kulczycki clinical score, lung function, Brasfield chest-radiograph score, and exercise capacity in patients with cystic fibrosis (CF)., Methods: We used a cross-sectional study design and included CF patients ≥ 10 years old. All patients had clinical and nutritional evaluation, oral glucose tolerance test, spirometry, chest radiograph, and 6-min walk test (6MWT). Patients were classified as having normal glucose tolerance, impaired glucose tolerance, or CF-related diabetes mellitus., Results: We included 88 patients: 59 with normal glucose tolerance, 15 with impaired glucose tolerance, and 14 with CF-related diabetes. Shwachman-Kulczycki clinical score (P = .04), at-rest S(pO(2)) (P = .001), S(pO(2)) difference before versus after 6MWT (P = .001), and Brasfield chest-radiograph score (P = .01) were significantly lower in the impaired-glucose-tolerance group than in the normal-glucose-tolerance group, but did not differ from the CF-related-diabetes group. In female patients only, percent-of-predicted FEV(1) was significantly lower in the impaired-glucose-tolerance group than in the normal-glucose-tolerance group (P = .02), but did not differ from the CF-related-diabetes group (P = .10). There was a significant relationship between glucose intolerance and sex when clinical score, 6-min walk distance, FEV(1), and radiograph score were combined in a multivariate analysis., Conclusions: In patients with CF, glucose intolerance was associated with poor clinical score, lower at-rest S(pO(2)), greater S(pO(2)) difference before versus after 6MWT, poor lung function, and lower radiograph score. Overall, multivariate analysis indicated poorer performance in the latter variables in female patients with glucose intolerance than in male patients with glucose intolerance.

Objective: To determine the repeatability of the 6-minute walk test (6MWT) in adolescents and adults with cystic fibrosis (CF)., Methods: This was a prospective cross-sectional study. We included consecutive patients ages>or=15 years attending an adult CF program. The patients underwent the 6MWT, pulmonary function tests, and clinical evaluation. The second 6MWT was performed following a rest period of 60 min., Results: Thirty-one patients were included. The mean+/-SD age was 23.5+/-6.7 y, and the mean FEV1 was 61+/-28% of predicted. The mean+/-SD walked distance in the first 6MWT was 583.6+/-68.6 m and in the second 6MWT was 590.0+/-72.2 m. The mean difference between the first and second 6MWT was -6.5 m, with limits of agreement between -74.9 m and 61.9 m, and the coefficient of variation was 4.3%. The mean oxygen desaturation in the first 6MWT was 2.5+/-4.5%, and in the second test it was 1.8+/-4.0%. The mean difference between the first and second test was 0.6%, and the coefficient of variation was 104%., Conclusions: Although the 6MWT distance was reproducible, the wide limits of agreement exceeded the minimum important difference for this test. These findings indicate that, in the routine evaluation of CF patients, at least two 6MWTs are required on any testing occasion to obtain a reliable estimate of the 6MWT distance.