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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 SpO2 (P = .001), SpO2 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 FEV1 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, FEV1, 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 SpO2, greater SpO2 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. [ABSTRACT FROM AUTHOR]

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 ≥ 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 ± 68.6m 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. [ABSTRACT FROM AUTHOR]

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Objective: To identify the predictive factors of oxygen desaturation during the six-minute walk test (6MWT) in patients with cystic fibrosis (CF)., Methods: Prospective cross-sectional study involving clinically stable patients with CF aged > or = 10 years. The patients were submitted to nutritional evaluations, oral glucose tolerance tests, pulmonary function tests, chest X-rays and 6MWTs., Results: The study included 88 patients (43 females and 45 males; mean age, 19.9 + or - 7.2 years; mean FEV(1), 65.4 + or - 28.4%). We observed oxygen desaturation in 13 patients (OD+ group) and no oxygen desaturation in 75 (ODmicro group). In comparison with ODmicro group patients, OD+ group patients presented higher mean age (p = 0.004), worse clinical score (p < 0.001), worse radiological score (p < 0.001), higher incidence of glucose intolerance (p = 0.004), lower incidence of methicillin-sensitive Staphylococcus aureus infection (p < 0.001), higher incidence of methicillin-resistant S. aureus infection (p = 0.016), higher incidence of Pseudomonas aeruginosa infection (p = 0.008), lower mean resting SpO(2) (p < 0.001) and lower mean FEV(1) (p < 0.001). In the logistic regression analysis, oxygen desaturation during the 6MWT correlated with resting SpO(2) (OR = 0.305, p < 0.001) and FEV(1) (OR = 0.882, p = 0.025). The parameters maximizing the predictive value for oxygen desaturation were resting SpO(2) < 96% and FEV(1) < 40%. In this sample, 15% of the patients with CF aged > or = 10 years presented oxygen desaturation during the 6MWT., Conclusions: Resting SpO(2) < 96% and FEV(1) < 40% can predict oxygen desaturation during the 6MWT.

Objective: To determine the submaximal exercise capacity of patients with cystic fibrosis (CF) by means of the 6-minute walk test (6MWT), correlating the results with clinical score, nutritional status, radiographic score, and pulmonary function tests., Methods: This was a prospective, cross-sectional study involving patients aged 16 or older enrolled in a program for adults with CF. The patients were submitted to clinical evaluation, determination of maximal respiratory pressures, 6MWT, spirometry, and chest X-ray., Results: The study comprised 41 patients. The mean age was 23.7 +/- 6.5 years, and the mean forced expiratory volume in one second (FEV1) was 55.1 +/- 27.8%. On the 6MWT, 30 (73.2%) of the patients covered a distance (mean, 556.7 +/- 76.5 m) that was less than the predicted normal value. The distance walked did not correlate significantly with body mass index, clinical score, radiographic score, maximal respiratory pressures, peripheral oxygen saturation at rest, desaturation during the 6MWT, sensation of dyspnea, or fatigue, although it did so with age at diagnosis, FEV1 in liters, and forced vital capacity in liters. Worsening of pulmonary function was associated with greater desaturation during the 6MWT., Conclusion: This study showed that most patients attending an adult CF program had reduced submaximal exercise capacity. The 6MWT can be valuable for identifying patients who might experience oxygen desaturation and physical impairment in daily activities.