Respiratory muscle training improves respiratory muscle endurance but not exercise tolerance in children with cystic fibrosis.

BACKGROUND: Respiratory muscle endurance (RME) training has been shown to increase exercise endurance and lung function in adults with cystic fibrosis (CF). We conducted an interventional study to investigate the effectiveness of RME training on CF-related health outcomes in children. METHODS: In a crossover trial, 22 children, aged 9-18 years, with CF performed 8 weeks of RME training and standard chest physiotherapy in a randomized sequence separated by a 1 week washout period. All children underwent training sessions using the RME training device before beginning the study. The primary outcomes were RME (in minutes) and exercise endurance (in minutes). Data were analyzed according to the intention-to-treat principle. RESULTS: Sixteen of 22 children (73%) completed the study. Study dropouts tended to be older with more advanced lung disease. After RME training, respiratory muscle endurance significantly increased by 7.03 ± 8.15 min (mean ± standard deviation, P < 0.001), whereas exercise endurance was unchanged by RME training (0.80 ± 2.58 min, P = 0.169). No significant improvement in secondary outcomes (lung function, CF quality of life, and CF clinical score) were observed. The small sample size and short intervention time have to be acknowledged as limitations of our study. CONCLUSIONS: RME training led to a significant increase in respiratory muscle endurance in children with CF. However, RME training did not improve exercise endurance or other CF-related health outcomes. Thus, our results do not support the routine use of RME training in the care of children with CF. Future studies in larger populations and with prolonged intervention time may overcome the limitations of our study. Pediatr Pulmonol. 2017;52:331-336. © 2017 Wiley Periodicals, Inc.

Feasibility of nitrogen multiple-breath washout in inexperienced children younger than 7 years.

BACKGROUND: Multiple breath washout (MBW) is an attractive test to assess ventilation inhomogeneity, a marker of peripheral lung disease. Systematic research on MBW feasibility in preschool children is scanty. OBJECTIVES: We assessed feasibility of nitrogen MBW in children aged 3-7 years in a clinical setting applying current ERS/ATS consensus. METHODS: Sixty-two children with asthma were enrolled in the Swiss rehabilitation center Hochgebirgsklinik Davos without MBW experience and prior to consensus release. Age range was 3.1-6.7 years. All children were naïve to MBW, received training, and then aimed for triplicate nitrogen MBW within 20 min. We afterward assessed the number of MBW attempts and successful tests according to consensus asking for three technically acceptable trials with functional residual capacity (FRC) measurements within 25% of median FRC. Secondly, factors related to success rate and FRC variability were assessed. RESULTS: Out of 205 MBW attempts in 62 children, 103 trials (50%) in 51 children were successful. Only 15 children (24%) achieved three valid trials as suggested by consensus. At least two valid trials were obtained in 37 children (60%). Age was positively correlated with success rate. FRC variability was inversely correlated with variability of tidal volume. CONCLUSIONS: MBW was only feasible in one quarter of inexperienced children <7 years applying strict consensus criteria. Low FRC variability and low breathing variability seem to be mutually exclusive at this age group. Specific consensus recommendations for MBW in preschool children seem, thus, warranted. Pediatr Pulmonol. 2016;51:1183-1190. © 2016 Wiley Periodicals, Inc.

Impact of Software Settings on Multiple-Breath Washout Outcomes.

BACKGROUND AND OBJECTIVES: Multiple-breath washout (MBW) is an attractive test to assess ventilation inhomogeneity, a marker of peripheral lung disease. Standardization of MBW is hampered as little data exists on possible measurement bias. We aimed to identify potential sources of measurement bias based on MBW software settings. METHODS: We used unprocessed data from nitrogen (N2) MBW (Exhalyzer D, Eco Medics AG) applied in 30 children aged 5-18 years: 10 with CF, 10 formerly preterm, and 10 healthy controls. This setup calculates the tracer gas N2 mainly from measured O2 and CO2concentrations. The following software settings for MBW signal processing were changed by at least 5 units or >10% in both directions or completely switched off: (i) environmental conditions, (ii) apparatus dead space, (iii) O2 and CO2 signal correction, and (iv) signal alignment (delay time). Primary outcome was the change in lung clearance index (LCI) compared to LCI calculated with the settings as recommended. A change in LCI exceeding 10% was considered relevant. RESULTS: Changes in both environmental and dead space settings resulted in uniform but modest LCI changes and exceeded >10% in only two measurements. Changes in signal alignment and O2 signal correction had the most relevant impact on LCI. Decrease of O2 delay time by 40 ms (7%) lead to a mean LCI increase of 12%, with >10% LCI change in 60% of the children. Increase of O2 delay time by 40 ms resulted in mean LCI decrease of 9% with LCI changing >10% in 43% of the children. CONCLUSIONS: Accurate LCI results depend crucially on signal processing settings in MBW software. Especially correct signal delay times are possible sources of incorrect LCI measurements. Algorithms of signal processing and signal alignment should thus be optimized to avoid susceptibility of MBW measurements to this significant measurement bias.