Relationship between lung function and Modified Shuttle Test performance in adult patients with cystic fibrosis: a cross-sectional, retrospective study.

OBJECTIVES: To investigate the relationship between lung function and exercise capacity in adults with cystic fibrosis (CF), and to develop a CF-specific equation to predict Modified Shuttle Test (MST) performance from baseline data. DESIGN: Cross-sectional, retrospective study. SETTING: Adult CF centre. PARTICIPANTS: One hundred and twenty-seven patients with CF [61 male; mean age 25 years (range 17 to 52 years), mean forced expiratory volume in 1second (FEV1) 56% predicted (range 15 to 124%)]. MAIN OUTCOME MEASURES: MST and FEV1. RESULTS: Overall, a moderate-to-good relationship was found between lung function and MST performance (walking distance vs FEV1% predicted: r=0.64, P=0.01). This relationship between FEV1 and MST shows an obvious threshold at an FEV1 of 67% predicted. Above this threshold, no significant association was observed between FEV1 and MST performance. However, a strong relationship (MST vs FEV1% predicted: r≥0.74, P<0.01 for men and r=0.79, P<0.01 for women) was found below an FEV1 of 67% predicted. CONCLUSIONS: This study suggests that a strong association exists between lung function (FEV1% predicted) and MST (walking distance) in adult patients with moderate-to-severe CF (FEV1<67% predicted). A reference equation for MST performance was developed for those patients with FEV1 ≤67% predicted, providing a tool to make an a-priori prediction of MST walking distance.

Respiratory muscle strength in stable adolescent and adult patients with cystic fibrosis.

BACKGROUND: Since available studies have provided conflicting results, this study investigated respiratory muscle function and its relationship with exercise capacity, degree of dyspnoea and leg discomfort, and quality of life in patients with Cystic Fibrosis (CF). METHODS: Using a cross-sectional design, 27 clinically stable adolescent and adult patients (f/m: 14/13, age: 26+/-7 years) were included. Data of respiratory muscle strength (P(i)max and P(e)max), lung function (spirometry), peripheral muscle strength (peak isometric quadriceps and hand-grip strength), symptom-limited exercise capacity (modified shuttle test, MST), post-exercise dyspnoea and leg discomfort (Borg scores), and quality of life (CFQ-14+, MRC) were obtained for further analysis. RESULTS: P(i)max of the total patient group was significantly higher than reference values (P(i)max=124+/-32% predicted), and correlated positively with the walk/run distance of the MST (r(s)=0.59, p=0.00). Female patients showed more dyspnoea and a more impaired lung function than male patients. However, P(i)max and P(e)max (% predicted) showed a tendency to be higher in female than in male patients. CONCLUSION: Increased work of breathing will have a conditioning effect on the respiratory muscles, suggesting that training-related inspiratory muscle strength can play a positive role in the limited exercise capacity of CF patients.