Airway infection with a novel Cupriavidus species in persons with cystic fibrosis.

We describe the recovery and identification of a bacterium that represents a new species of the genus Cupriavidus from cultures of respiratory tract specimens from two patients with cystic fibrosis (CF). The elucidation of the role of this species in CF lung disease will require an evaluation of a greater number of cases.

Whole body vibration: a new therapeutic approach to improve muscle function in cystic fibrosis?

Disease progression in cystic fibrosis (CF) leads to muscle wasting and loss of muscle function. The aim of this prospective pilot study was to evaluate the effects of whole body vibration (WBV) on muscle function in adult patients with CF. Ten patients (three males; seven females) of the CF Center Cologne, Germany, have completed the 3-month study (age: 24-47 years; forced expiratory volume in 1 s (FEV1) 17-109% predicted (49+/-29) and body mass index (BMI) 16.6-24.4 kg/m2 (19.3+/-2.5). WBV was provided by a vibration platform (Galileo 2000). The patients were standing in an upright position receiving vertical vibration of frequencies between 20 and 25 Hz. The vibration exercise evokes muscle contractions via stretch reflexes improving muscular activity. The training schedule consisted of three 3-min sessions twice a day, 5 days per week for 3 months. Every 4 weeks the following tests were carried out: FEV1, forced vital capacity (FVC), BMI, chair-rising test (CRT), one-leg and two-leg jump test as well as maximal isometric grip force. The study has been approved by the local ethics committee. After 3 months of WBV all parameters in the CRT significantly improved: chair-rising time (P=0.03), maximal force (P=0.02), maximal power (P=0.01) as well as velocity (P=0.02). The peak jump force (P=0.02) and velocity (P=0.01) of the two-leg jump significantly improved. Parameters in the one-leg jump as well as maximal isometric grip force showed no significant improvement. Weight and BMI showed a slightly positive trend whereas FEV1 and FVC did not significantly change. Any change in mechanographic parameters did not correlate with FEV1 or FVC in this study. These results demonstrate that WBV can improve muscle function in CF patients.

Effect of treatment with dornase alpha on airway inflammation in patients with cystic fibrosis.

Recombinant human deoxyribonuclease (rhDNase) has been shown to improve lung function and reduce the number of pulmonary exacerbations in patients with cystic fibrosis (CF), but its long-term effect on airway inflammation remains unknown. In this study, we used bronchoalveolar lavage (BAL) to investigate the long-term effect of rhDNase on inflammation in patients with CF having mild lung disease. A total of 105 patients with CF (> or =5 years of age) having normal lung function were randomized to receive rhDNase (2.5 mg/day) or no rhDNase. Patients with a normal percentage of neutrophils in BAL fluid at baseline were not randomized and served as the control group. The percentage of neutrophils in the pooled BAL sample was similar in both randomized groups at baseline. A significant increase in neutrophils was observed over the 3-year study period in both untreated patients and control subjects, whereas neutrophils remained unchanged in patients treated with rhDNase. Elastase activities and interleukin-8 concentrations also increased in untreated patients and remained stable in patients on rhDNase. We conclude that in patients with CF, an increase in neutrophilic airway inflammation is found that is positively influenced by rhDNase treatment.

Improvement of alveolar glutathione and lung function but not oxidative state in cystic fibrosis.

Chronic neutrophilic inflammation leads to oxidative damage, which may play an important role in the pathogenesis of cystic fibrosis lung disease. Bronchoalveolar lavage levels of the antioxidant glutathione are diminished in patients with cystic fibrosis. Here we evaluated the effects of glutathione aerosol on lower airway glutathione levels, lung function, and oxidative status. Pulmonary deposition of a radiolabeled monodisperse aerosol generated with a Pari LC Star nebulizer (Allergy Asthma Technology, Morton Grove, IL) connected to an AKITA inhalation device (Inamed, Gauting, Germany) was determined in six patients. In 17 additional patients bronchoalveolar lavage fluid was assessed before and after 14 days of inhalation with thrice-daily doses of 300 or 450 mg of glutathione. Intrathoracic deposition was 86.3 +/- 1.4% of the emitted dose. Glutathione concentration in lavage 1 hour postinhalation was increased three- to fourfold and was still almost doubled 12 hours postinhalation. FEV(1) transiently dropped after inhalation but increased compared with pretreatment values after 14 days (p < 0.001). This improvement was not related to the lavage content of oxidized proteins and lipids, which did not change with treatment. These results show that, using a new inhalation device with high efficacy, glutathione treatment of the lower airways is feasible. Reversion of markers of oxidative injury may need longer treatment, higher doses, or different types of antioxidants.