Short-term effects of positive expiratory pressure mask on ventilation inhomogeneity in children with cystic fibrosis: A randomized, sham-controlled crossover study.

BACKGROUND: Can physiotherapy with a positive expiratory pressure (PEP) mask improve peripheral ventilation inhomogeneity, a typical feature of children with cystic fibrosis (cwCF)? To answer this question, we used the nitrogen multiple-breath washout (N2MBW) test to measure diffusion-convection-dependent inhomogeneity arising within the intracinar compartment (Sacin*VT). METHODS: For this randomized, sham-controlled crossover trial, two N2MBW tests were performed near the hospital discharge date: one before and the other after PEP mask therapy (1 min of breathing through a flow-dependent PEP device attached to a face mask, followed by three huffs and one cough repeated 10 times) by either a standard (10-15 cmH20) or a sham (<5 cmH20) procedure on two consecutive mornings. Deception entailed misinforming the subjects about the nature of the study; also the N2MBW operators were blinded to treatment allocation. Study outcomes were assessed with mixed-effect models. RESULTS: The study sample was 19 cwCF (ten girls), aged 11.4 (2.7) years. The adjusted Sacin*VT mean difference between the standard and the sham procedure was -0.015 (90% confidence interval [CI]: -∞ to 0.025) L-1. There was no statistically significant difference in Scond*VT and lung clearance index between the two procedures: -0.005 (95% CI: -0.019 to 0.01) L-1 and 0.49 (95% CI: -0.05 to 1.03) turnovers, respectively. CONCLUSION: Our findings do not support evidence for an immediate effect of PEP mask physiotherapy on Sacin*VT with pressure range 10-15 cmH20. Measurement with the N2MBW and the crossover design were found to be time-consuming and unsuitable for a short-term study of airway clearance techniques.

Six minute walk test in Italian children with cystic fibrosis aged 6 and 11.

Six minute walk test (6MWT) is a field exercise test widely used in clinical practice, both in adults and in pediatric patients. The primary aim of the study is to evaluate the physical performance of the subjects and compare them with the predicted Italian values. The secondary aim is to verify the possible relationship between the 6MWT distance (6MWD) and the clinical variables of the sample. Italian children between 6-11 years affected by CF were recruited from 9 regional centers for CF. Short questionnaire assessments about their health state and physical activity routine was administered. Anthropometric characteristics were measured before the test and, peripheral oxygen saturation (SpO2), heart and respiratory rate were measured before and after a 6-minute walk test. The tests were performed according to the American Thoracic Society (ATS) guidelines. 6MWD was compared with the predicted distance calculated by the reference equation for healthy subjects of the same age. A total of 132 children were recruited (70 male) and completed the assessment. The mean (±SD) for 6MWD was 557.4 (±69.9), male = 551.4 (±80.0), female = 560.4 (±63.3), however the predicted distance mean was 605 m. A total of 101(76.5%) subjects practice regular physical activity. A total of 31 (23%) had a FEV1 lower than their lower limits of normal (LLN). Functional performance on the 6MWT was poorer among the CF patients than among the predicted distance estimated with Italian values. The correlation with the amount of physical activity and 6MWD has been verified.

Hospital staff practical skills and theoretical knowledge in inhaled aerosol therapy: a single centre cross-sectional observational study.

Inhaled therapy is widely used for treatment of many respiratory disorders. Drug delivery in lungs is dependent on the correct use of aerosol devices and patients' training is vital for a correct therapy administration. Therefore, is very important to assess the skills of professionals involved in training patients to the correct use of inhaler devices. The aim of this study was to check the practical skills and the theoretical knowledge of health care personnel in our University Hospital in using aerosol therapy and to determine differences among professional figures in the management of inhaler devices. Three hundred and fifteen (315) volunteers including physicians, residents, nurses and respiratory physiotherapists were enrolled; an independent professional, not working in our University Hospital, assessed their theoretical knowledge related to aerosol throughout a questionnaire. Practical skills were assessed through placebo simulation with the devices that participants declared to use frequently with patients. None of the respondents correctly answered all questions evaluating theoretical knowledge on the proper use of aerosol therapy. Respiratory physiotherapists obtained significantly better results with 17.2 (1.3) average points compared to 10.3 (3.7) obtained by doctors, 9.0 (3.0) by nurses and 9.1 (4.5) by residents. Analysing in detail physicians' results, pneumologists showed better theoretical knowledge than other specialists. Concerning the practical skills, about 80% of those stating they knew how to use metered dose inhalers, made mistakes in the basic steps for proper inhalation technique and over 50% of respondents were unable to properly simulate placebo administration of dry powder inhalers. Also here, respiratory physiotherapists and pneumologists had significant better performances, when compared to other health professionals. Our data are in line with those published in the literature in other international clinical settings, noting inadequate practical and theoretical knowledge of the available devices for aerosol therapy.

Exercise capacity and ventilation inhomogeneity in cystic fibrosis: A cross-sectional study.

BACKGROUND: Lung clearance index (LCI2.5 ) is a marker of overall lung ventilation inhomogeneity and has proven to be able to detect early peripheral damage in subjects with cystic fibrosis (CF), with greater sensitivity than conventional spirometry. Combining its sensitivity with the output of an incremental exercise testing, we hypothesized that any sign of ventilation inhomogeneity in subjects without severe airflow obstruction and with a normal exercise tolerance could be relevant for the CF team, tracking early lung disease and potential exercise limiting factors. METHODS: Patients with CF in clinical stable conditions were recruited between 2015 and 2017. Available spirometry, nitrogen multiple-breath washout test and symptoms-limited exercise testing performed as parts of patients' annual routine evaluation were considered for this cross-sectional study. To describe the relationship between exercise intensity and ventilation inhomogeneity, a linear regression analysis was performed using backward elimination based on Akaike information criteria. RESULTS: Seventy-seven patients (38 females) were included. Sacin and LCI2.5 were significantly higher in patients with an overall reduced exercise tolerance. Peak work developed during exercise was associated with body mass index (b = 5.25; 95% confidence interval [CI] = 1.53-8.98), forced expiratory volume in 1 second (FEV1 ; b = 3.71; 95% CI = 1.96-5.46), Pseudomonas aeruginosa chronic infection (b = -8.84; 95% CI = -15.84 to -1.84) but not with LCI2.5 . CONCLUSION: Exercise capacity and airflow obstruction are associated in this Italian CF cohort. Considering the greater discriminatory power of LCI2.5 over FEV1 and peak work, the Godfrey protocol without gas analysis cannot provide detailed information about lung function or efficiency. However, this incremental protocol without gas exchange measures can still provide the CF team with information about exercise tolerance and disability.

Quantitative multivolume proton-magnetic resonance imaging in patients with cystic fibrosis lung disease: comparison with clinical indicators.

This cross-sectional study aims to verify the relationship between quantitative multivolume proton-magnetic resonance imaging (1H-MRI) and clinical indicators of ventilatory abnormalities in cystic fibrosis (CF) lung disease.Non-enhanced chest MRI, spirometry and multiple breath washout was performed by 28 patients (10-27 years) with CF lung disease. Images acquired at end-inspiration and end-expiration were registered by optical flow to estimate expiratory-inspiratory proton-density change (Δ1H-MRI) as a measure of regional ventilation. Magnetic resonance images were also evaluated using a CF-specific scoring system.Biomarkers of CF ventilation impairment were defined from the Δ1H-MRI as follows: Δ1H-MRI median, Δ1H-MRI quartile coefficient of variation (QCV) and percentage of low-ventilation volume (%LVV). Imaging biomarkers correlated to all the clinical measures of ventilation abnormality, with the strongest correlation between Δ1H-MRI median and forced expiratory volume in 1 s (r2=0.44, p<0.001), Δ1H-MRI QCV and lung clearance index (LCI) (r2=0.51, p<0.001) and %LVV and LCI (r2=0.66, p<0.001). Correlations were also found between imaging biomarkers of ventilation and morphological scoring.The study showed a significant correlation between quantitative multivolume MRI and clinical indicators of CF lung disease. MRI, as a non-ionising imaging technique, may be particularly attractive in CF care for longitudinal evaluation, providing a new imaging biomarker to detect early ventilatory abnormalities.