Effects of treadmill exercise versus Flutter® on respiratory flow and sputum properties in adults with cystic fibrosis: a randomised, controlled, cross-over trial.

Background: Treadmill exercise and airway clearance with the Flutter® device have previously been shown to improve mucus clearance mechanisms in people with cystic fibrosis (CF) but have not been compared. It is therefore not known if treadmill exercise is an adequate form of airway clearance that could replace established airway clearance techniques, such as the Flutter®. The aim of this study was to evaluate respiratory flow, sputum properties and subjective responses of treadmill exercise and Flutter® therapy, compared to resting breathing (control).

A randomised trial of hypertonic saline during hospitalisation for exacerbation of cystic fibrosis.

Background: The mucoactive effects of hypertonic saline should promote exacerbation resolution in people with cystic fibrosis (CF).

Objectives: To determine the effects of hypertonic saline inhalation during hospitalisation for exacerbation of CF on length of stay, lung function, symptoms, oxygenation, exercise tolerance, quality of life, bacterial load and time to next hospitalisation.

Methods: 132 adults with an exacerbation of CF were randomised to inhale three nebulised doses a day of either 4 mL 7% saline or a taste-masked control of 0.

Non-invasive ventilation used as an adjunct to airway clearance treatments improves lung function during an acute exacerbation of cystic fibrosis: a randomised trial.

Question: During an acute exacerbation of cystic fibrosis, is non-invasive ventilation beneficial as an adjunct to the airway clearance regimen?

Design: Randomised controlled trial with concealed allocation and intention-to-treat analysis.

Participants: Forty adults with moderate to severe cystic fibrosis lung disease and who were admitted to hospital for an acute exacerbation.

Intervention: Comprehensive inpatient care (control group) compared to the same care with the addition of non-invasive ventilation during airway clearance treatments from Day 2 of admission until discharge (experimental group).

Physical activity in people with asbestos related pleural disease and dust-related interstitial lung disease: An observational study.

This study aimed to measure the levels of physical activity (PA) in people with dust-related pleural and interstitial lung diseases and to compare these levels of PA to a healthy population. There is limited data on PA in this patient population and no previous studies have compared PA in people with dust-related respiratory diseases to a healthy control group. Participants with a diagnosis of a dust-related respiratory disease including asbestosis and asbestos related pleural disease (ARPD) and a healthy age- and gender-matched population wore the SenseWear(®) Pro3 armband for 9 days.

Exercise training for asbestos-related and other dust-related respiratory diseases: a randomised controlled trial.

Background: The study aimed to determine the short and long-term effects of exercise training on exercise capacity and health-related quality of life (HRQoL) compared to usual care in people with dust-related pleural and interstitial respiratory diseases. No previous studies have specifically evaluated exercise training in this patient population.

Methods: Participants with a diagnosis of a dust-related respiratory disease including asbestosis and asbestos related pleural disease were recruited and randomised to an eight-week exercise training group (EG) or a control group (CG) of usual care.

Functional exercise capacity and health-related quality of life in people with asbestos related pleural disease: an observational study.

Background: Functional exercise capacity in people with asbestos related pleural disease (ARPD) is unknown and there are no data on health-related quality of life (HRQoL). The primary aims were to determine whether functional exercise capacity and HRQoL were reduced in people with ARPD. The secondary aim was to determine whether functional exercise capacity was related to peak exercise capacity, HRQoL, physical activity or respiratory function.

Shared Pseudomonas aeruginosa genotypes are common in Australian cystic fibrosis centres.

Recent molecular-typing studies suggest cross-infection as one of the potential acquisition pathways for Pseudomonas aeruginosa in patients with cystic fibrosis (CF). In Australia, there is only limited evidence of unrelated patients sharing indistinguishable P. aeruginosa strains.

Arm exercise training in chronic obstructive pulmonary disease: a randomised controlled trial.

The aim of this study was to compare the effects of arm endurance training, arm strength training, a combination of arm endurance and strength training, and no arm training on endurance arm exercise capacity. A randomised controlled trial was undertaken with chronic obstructive pulmonary disease subjects randomised into one of four groups to complete 8 weeks of training: (a) arm endurance training (endurance group) consisting of supported and unsupported arm exercises, (b) arm strength training (strength group) using weight machines, (c) a combination of arm endurance and arm strength training (combined group), or (d) no arm training (control group). The primary outcome measurement was endurance arm exercise capacity measured by an endurance arm crank test.

Adults with cystic fibrosis prefer hypertonic saline before or during airway clearance techniques: a randomised crossover trial.

Question: Among adults with cystic fibrosis, does the timing of hypertonic saline relative to airway clearance techniques affect lung function, perceived efficacy, tolerability, or satisfaction with the entire airway clearance regimen, and is the preferred timing regimen stable over time?

Design: A randomised crossover trial with concealed allocation, intention-to-treat analysis, and blinded assessors.

Participants: 50 adults with cystic fibrosis and stable lung function at the end of a hospital admission.

Intervention: Participants performed 3 sessions of airway clearance techniques per day for 3 days.

Cystic fibrosis in Australia, 2009: results from a data registry.

Objectives: To describe the demographics, clinical features and outcomes among people with cystic fibrosis (CF) in Australia and to estimate incidence of the disease.

Design And Setting: Cross-sectional analysis using data from the Australian Cystic Fibrosis Data Registry for 2009.

Main Outcome Measures: Numbers of diagnoses, pulmonary and anthropometric measurements, microbiological culture results, rates of hospitalisation and transplantation, and numbers of medical complications and deaths.

The role of exercise in maintaining health in cystic fibrosis.

Purpose Of Review: Consensus statements about the care of people with cystic fibrosis (CF) recommend exercise as part of a wider management strategy. Many of these recommendations are based on high-quality evidence that regular exercise improves some important clinical outcomes, such as lung function and quality of life. However, the evidence about the effect of exercise on other clinical outcomes is less extensive or lower in quality.

Pregnancy outcomes in the current era of cystic fibrosis care: a 15-year experience.

Background: With improvement in clinical care and longer survival of patients with cystic fibrosis (CF), pregnancy has become commonplace. However, the impact of pregnancy on maternal health and fetal outcomes requires ongoing review.

Methods: A retrospective study of 20 pregnancies from 18 women with CF during the period 1995-2009 was performed.

Gas exchange in disease: asthma, chronic obstructive pulmonary disease, cystic fibrosis, and interstitial lung disease.

Ventilation-perfusion (VA/Q) inequality is the underlying abnormality determining hypoxemia and hypercapnia in lung diseases. Hypoxemia in asthma is characterized by the presence of low VA/Q units, which persist despite improvement in airway function after an attack. This hypoxemia is generally attenuated by compensatory redistribution of blood flow mediated by hypoxic vasoconstriction and changes in cardiac output, however, mediator release and bronchodilator therapy may cause deterioration.

Pregnancy and cystic fibrosis.

The management of a pregnancy in a woman with cystic fibrosis is usually achieved with successful outcomes for mother and child with appropriate multidisciplinary care. The process begins prior to conception and requires frequent monitoring of the mother's respiratory status, level of glycaemic control and obstetric wellbeing. Recent reports have suggested that pregnancy can be managed without a persisting decrement in lung function beyond what may be expected in women with cystic fibrosis who are not pregnant.

Multi-centre research in Australia: analysis of a recent National Health and Medical Research Council-funded project.

Background And Objective: Human research ethics committees provide essential review of research projects to ensure the ethical conduct of human research. Several recent reports have highlighted a complex process for successful application for human research ethics committee approval, particularly for multi-centre studies. Limited resources are available for the execution of human clinical research in Australia and around the world.

Recombinant activated factor VII for massive hemoptysis in patients with cystic fibrosis.

Massive hemoptysis is a common complication in patients with cystic fibrosis (CF) and is associated with significant morbidity and mortality. Conventional treatment with antibiotic therapy and early bronchial artery embolization (BAE) is usually successful in achieving hemostasis in the majority of patients. Recombinant activated factor VII (rFVIIa), originally developed for use in patients with hemophilia, has emerged as a general hemostatic agent that is potentially useful in the management of many life-threatening bleeding conditions.

Evaluation of the SenseWear activity monitor during exercise in cystic fibrosis and in health.

Objective: Determine the SenseWear Pro3 Armband (SWA) accuracy for estimating energy expenditure (EE) and step count during treadmill walking in cystic fibrosis (CF) compared to healthy adults.

Hypothesis: SWA estimation of EE would be less accurate for CF, than for healthy subjects, due to interference with the SWA skin sensors caused by the high salt concentration in the sweat of CF subjects.

Methods: 17 CF (mean age 26 yr; FEV1 54% predicted) and 17 age-matched control subjects walked slightly faster than their comfortable pace on a treadmill for 20 min, whilst simultaneously wearing the SWA and breathing through an open-circuit indirect calorimetry (IC) system.

Low rates of Pseudomonas aeruginosa misidentification in isolates from cystic fibrosis patients.

Pseudomonas aeruginosa is an important cause of pulmonary infection in cystic fibrosis (CF). Its correct identification ensures effective patient management and infection control strategies. However, little is known about how often CF sputum isolates are falsely identified as P.

Sleep-disordered breathing before and after lung transplantation.

Background: Sleep-disordered breathing (SDB) is common in patients with severe chronic respiratory failure, but there are no data describing the prevalence of SDB among patients listed for lung transplantation or the effect of transplantation on SDB. We sought to determine the prevalence and impact of SDB before and after lung transplantation.

Methods: We performed polysomnography (PSG) on 117 of 183 (64%) consecutive patients (64 males, 53 females) listed for lung transplantation between 1998 and 2001.

Physiological responses to high intensity, constant-load arm exercise in COPD.

The aim of the study was to compare the metabolic, ventilatory and dyspnoea responses of a single bout of high intensity, constant-load arm exercise to peak arm exercise in people with chronic obstructive pulmonary disease (COPD). Thirty people with COPD (mean age+/-SD=65+/-8 years; FEV1% predicted=56+/-12%) were included. All subjects performed an incremental arm exercise test to peak work capacity on an arm ergometer and, on a separate day, a constant-load arm exercise test at 80% of the peak work rate achieved on the incremental test.

Other mucoactive agents for cystic fibrosis.

This review examines specific mucoactive agents from three classes: expectorants, which add water to the airway; ion-transport modifiers, which promote ion and water transport across the epithelium of the airway; and mucokinetics, which improve cough-mediated clearance by increasing airflow or reducing sputum adhesivity. The agents are isotonic and hypertonic saline, mannitol, denufosol and beta-agonists. Our understanding of these agents has recently improved through pre-clinical research, clinical trials and, in particular, extensive research into the nature of the liquid lining the surface of the airway, both in health and in cystic fibrosis (CF).

Glycosylation of sputum mucins is altered in cystic fibrosis patients.

Cystic fibrosis (CF) is characterized by chronic lung infection and inflammation, with periods of acute exacerbation causing severe and irreversible lung tissue damage. We used protein and glycosylation analysis of high-molecular mass proteins in saline-induced sputum from CF adults with and without an acute exacerbation, CF children with stable disease and preserved lung function, and healthy non-CF adult and child controls to identify potential biomarkers of lung condition. While the main high-molecular mass proteins in the sputum from all subjects were the mucins MUC5B and MUC5AC, these appeared degraded in CF adults with an exacerbation.

Inhaled hypertonic saline as a therapy for cystic fibrosis.

Purpose Of Review: The beneficial effect of a short course of nebulized hypertonic saline on lung function for people with cystic fibrosis was first identified in 1996. At that time, competing hypotheses about the pathogenesis of cystic fibrosis lung disease predicted very different responses to long-term inhalation of hypertonic saline.

Recent Findings: Recent benchtop research supports the hypothesis that the liquid layer lining the airways is depleted in cystic fibrosis.