Little is known about the long-term persistence of positive effects induced by a physical conditioning programme in cystic fibrosis. Therefore, this study determined the effects of a 6-month conditioning programme on peak oxygen uptake (primary outcome) and other markers of fitness, physical activity, anthropometry, lung function and quality of life (secondary outcomes), 18 and 24 months after the programme was initiated.Patients with cystic fibrosis aged 12-40 yrs were randomly assigned to an intervention (n523) and a control (n515) group. The intervention group consented to add 3 h of sports per week for o6 months to their previous activities. Controls were asked to maintain their level of activity for 12 months. Patients were seen at baseline and after 3, 6, 12, 18 and 24 months.There was no significant difference between groups at baseline. The intervention induced positive effects on peak oxygen uptake (difference in changes from baseline to the 18-and 24-month assessments between groups: 3.72¡1. ; p,0.05), forced vital capacity (6.06¡2.87% predicted; p,0.05) and perceived health (9.89¡4.72; p,0.05).A home-based partially supervised physical conditioning programme can improve physical fitness, lung function and perceived health long after the intervention has ended.

BackgroundHealth-related and disease-specific quality of life (HRQoL) has been increasingly valued as relevant clinical parameter in cystic fibrosis (CF) clinical care and clinical trials. HRQoL measures should assess – among other domains – daily functioning from a patient’s perspective. However, validation studies for the most frequently used HRQoL questionnaire in CF, the Cystic Fibrosis Questionnaire (CFQ), have not included measures of physical activity or fitness. The objective of this study was, therefore, to determine the cross-sectional and longitudinal relationships between HRQoL, physical activity and fitness in patients with CF.MethodsBaseline (n = 76) and 6-month follow-up data (n = 70) from patients with CF (age ≥12 years, FEV1 ≥35%) were analysed. Patients participated in two multi-centre exercise intervention studies with identical assessment methodology. Outcome variables included HRQoL (German revised multi-dimensional disease-specific CFQ (CFQ-R)), body composition, pulmonary function, physical activity, short-term muscle power, and aerobic fitness by peak oxygen uptake and aerobic power.ResultsPeak oxygen uptake was positively related to 7 of 13 HRQoL scales cross-sectionally (r = 0.30-0.46). Muscle power (r = 0.25-0.32) and peak aerobic power (r = 0.24-0.35) were positively related to 4 scales each, and reported physical activity to 1 scale (r = 0.29). Changes in HRQoL-scores were directly and significantly related to changes in reported activity (r = 0.35-0.39), peak aerobic power (r = 0.31-0.34), and peak oxygen uptake (r = 0.26-0.37) in 3 scales each. Established associates of HRQoL such as FEV1 or body mass index correlated positively with fewer scales (all 0.24 < r < 0.55).ConclusionsHRQoL was associated with physical fitness, especially aerobic fitness, and to a lesser extent with reported physical activity. These findings underline the importance of physical fitness for HRQoL in CF and provide an additional rationale for exercise testing in this population.Trial registrationClinicalTrials.gov, NCT00231686

Aspergillus colonization is a common phenomenon in adult cystic fibrosis (CF) patients. The clinical significance of Aspergillus for the pathogenesis of CF lung disease remains unclear and factors predisposing to such colonization are still completely unknown. We investigated the prevalence of Aspergillus colonization in 104 adult CF patients who attended our outpatient clinic in 1997. With respect to demographic and clinical data, and antibiotic therapy received, we further examined which factors were associated with Aspergillus colonization in these patients. Repeated investigations of CF sputum samples revealed Aspergillus species in 43/104 (41.3%; 95% confidence interval 30.2-52.5%) of the patients. We found no significant relationship between Aspergillus colonization and age (P > 0.4), gender (P = 0.4), colonization with pseudomonas species (P > 0.6), lower lung function values (P > 0.9), or worse chest radiography (P > 0.1). Surprisingly, the prevalence of Aspergillus colonization was higher in CF patients receiving prophylactic antibiotic therapy (oral antibiotics: P = 0.05; inhalative antibiotics: P = 0.035; both antibiotics: P = 0.048). Prophylactic antibiotics are widely used to eradicate or decrease chronic bronchopulmonary infection in CF. Our results indicate that long-term antibiotic therapy may predispose CF patients to Aspergillus colonization.

The new, noninvasive, low-cost H pylori antigen test in stool can replace the UBT for detection of H pylori infection in children with comparable reliability and accuracy.