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Background There are currently no reference standards for the development of competence in bronchoscopy. Research Question The aims of this study were to (1) develop learning curves for bronchoscopy skill development and (2) estimate the number of bronchoscopies required to achieve competence. Study Design and Methods Trainees from seven North American academic centers were enrolled at the beginning of their pulmonology training. Performance during clinical bronchoscopies was assessed by supervising physicians using the Ontario Bronchoscopy Assessment Tool (OBAT). Group-level learning curves were modeled using a quantile regression growth model, where the dependent variable was the mean OBAT score and the independent variable was the number of bronchoscopies performed at the time the OBAT was completed. Results A total of 591 OBAT assessments were collected from 31 trainees. The estimated regression quantiles illustrate significantly different learning curves based on trainees’ performance percentiles. When competence was defined as the mean OBAT score for all bronchoscopies rated as being completed without need for supervision, the mean OBAT score associated with competence was 4.54 (95% CI, 4.47-4.58). Using this metric, the number of bronchoscopies required to achieve this score varied from seven to 10 for the 90th percentile of trainees and from 109 to 126 for the lowest 10th percentile of trainees. When competence was defined as the mean OBAT score for the first independent bronchoscopy, the mean was 4.40 (95% CI, 4.20-4.60). On the basis of this metric, the number of bronchoscopies required varied from one to 11 for the 90th percentile of trainees and from 83 to 129 for the lowest 10th percentile of trainees. Interpretation We were able to generate learning curves for bronchoscopy across a range of trainees and centers. Furthermore, we established the average number of bronchoscopies required for the attainment of competence. This information can be used for purposes of curriculum planning and allows a trainee’s progress to be compared with an established norm.

The novel coronavirus (SARS-CoV-2) pandemic has forced clinicians across Canada to abruptly adapt to a new reality of reduced contact with patients in ambulatory care settings With minimal lead time, ambulatory clinics in Canada were required to adopt aggressive infection prevention and control measures while facing shortages of personal protective equipment and to enact strategies to urgently reorganize patient care These adaptations included the conversion of ambulatory clinics to telephone or virtual care platforms In 2015, the Public Health Agency of Canada (PHAC) published pandemic influenza preparedness guidelines highlighting the importance of pandemic preparedness planning in primary and ambulatory care settings, including clinic continuity planning and provisions for non in-person care Despite these recommendations, hospital-based ambulatory clinics lacked formal strategic plans to adapt patient care processes during the SARS-CoV-2 outbreak Thus, in a short time frame, clinics individually adapted their own patient care processes This creates the potential for gaps in quality of medical care for patients across Canada In this report, we describe the evolution and operationalization of care planning in our hospital-based respirology clinic, highlight the challenges faced, and make recommendations for respirology clinic adaptations based on available guidance This process may be used as a foundation to guide future policy, discussion and guidelines for hospital-based respiratory care to ensure optimal preparedness during the next inevitable respiratory viral pandemic or possible worsening of the current pandemic © 2020 Canadian Thoracic Society

BACKGROUND: Severe asthma patients require high-dose inhaled corticosteroids (ICS) plus a second controller medication to control their disease or remain inadequately controlled despite this. Benra...

Fatigue is a common, debilitating, and poorly understood symptom post-COVID-19. We sought to better characterize differences in those with and without post-COVID-19 fatigue using cardiopulmonary exercise testing. Despite elevated dyspnoea intensity ratings, V̇O

There are currently no reference standards for the development of competence in bronchoscopy.The aims of this study were to (1) develop learning curves for bronchoscopy skill development and (2) estimate the number of bronchoscopies required to achieve competence.Trainees from seven North American academic centers were enrolled at the beginning of their pulmonology training. Performance during clinical bronchoscopies was assessed by supervising physicians using the Ontario Bronchoscopy Assessment Tool (OBAT). Group-level learning curves were modeled using a quantile regression growth model, where the dependent variable was the mean OBAT score and the independent variable was the number of bronchoscopies performed at the time the OBAT was completed.A total of 591 OBAT assessments were collected from 31 trainees. The estimated regression quantiles illustrate significantly different learning curves based on trainees' performance percentiles. When competence was defined as the mean OBAT score for all bronchoscopies rated as being completed without need for supervision, the mean OBAT score associated with competence was 4.54 (95% CI, 4.47-4.58). Using this metric, the number of bronchoscopies required to achieve this score varied from seven to 10 for the 90th percentile of trainees and from 109 to 126 for the lowest 10th percentile of trainees. When competence was defined as the mean OBAT score for the first independent bronchoscopy, the mean was 4.40 (95% CI, 4.20-4.60). On the basis of this metric, the number of bronchoscopies required varied from one to 11 for the 90th percentile of trainees and from 83 to 129 for the lowest 10th percentile of trainees.We were able to generate learning curves for bronchoscopy across a range of trainees and centers. Furthermore, we established the average number of bronchoscopies required for the attainment of competence. This information can be used for purposes of curriculum planning and allows a trainee's progress to be compared with an established norm.

Accurate diagnosis of interstitial lung disease (ILD) is crucial for management and prognosis but can be challenging even for experienced clinicians. Expert multidisciplinary discussion (MDD) is considered the reference standard for ILD diagnosis; however, there remain concerns regarding lack of validation studies and relative limited information on the impact of MDD in real-life clinical practice. The goal of this study was to assess the effect of MDD in providing a specific ILD diagnosis, changing the diagnosis provided upon referral, and to determine how often and in which way MDD altered management. Material and Methods. Retrospective observational study in an ILD referral tertiary academic center. MDD diagnoses were categorized as specific, provisional, and unclassifiable ILD. Pre-MDD and MDD diagnoses were compared for change in diagnosis and concordance rates for specific diagnoses. Relevant change in management including initiation or change in pharmacological treatment, referral to surgical biopsy, and nonpharmacological management were recorded. Results. 126 cases were included (79M, 47F, 36–93 years, mean 70 y). Specific MDD diagnosis was provided in 62% (78/126); 12% (15/126) had provisional diagnosis, and 21% (27/126) was unclassifiable. Overall agreement for specific pre-MDD and MDD diagnosis was 41% (52/126) and 80% for idiopathic pulmonary fibrosis (IPF) diagnosis. MDD altered diagnosis in 37% (47/126) and changed management in 39% (50/126). Amongst concordant diagnoses, management was altered in 46% (24/52). In summary, MDD provided a specific diagnosis discordant with pre-MDD diagnosis in a significant proportion of cases and was particularly valuable in the diagnosis of non-IPF ILD. MDD often altered management and had relevant impact on management even in cases with concordant pre-MDD diagnosis.

Frailty is a comprehensive health measure characterised by an individual’s vulnerability and diminished reserve when faced with health stressors.1–3 As one becomes increasingly frail, the ability to recover from acute illnesses is impaired, leading to progressive disability, increased risk of hospitalisation, need for supportive living environments and death.1 4–6 Current estimates suggest that 40% of community-dwelling adults are at risk of becoming frail, while 40% of hospitalised patients are ‘vulnerable’ or ‘mildly frail’.5 7 8 Hospitalisation is a key risk factor for the progression of frailty, especially among older adults.5 9–11 Frailty is measured with the Clinical Frailty Scale (CFS), a validated measure that is correlated with a comprehensive Frailty Index.1 Assessing frailty during patient encounters could help clarify the appropriateness of interventions and improve prognostication and shared decision making, which are essential components of patient-centred care.2 12–15 Our health system is currently organised to address single organ illnesses and frailty is often overlooked as a consequence of normal ageing.16 This pilot project was designed to assess the feasibility of implementing the CFS among hospitalised patients with chronic obstructive pulmonary disease (COPD), to assess the differences in frailty assessments between health providers, and to understand the distribution of frailty among hospitalised patients with COPD. Over an 11-month period, the CFS was included in routine nursing assessments on a respiratory ward at a tertiary care hospital (online supplementary appendix 1). Frailty assessments were linked to a clinical pathway designed to allocate supportive resources …

To assess the effect on exercise capacity and tolerability of resveratrol, 13 healthy, sedentary adult volunteers were enrolled in a randomized crossover study comparing resveratrol and placebo over two 4-week periods, with a 2-week time between periods when subjects received no treatment. No significant changes in exercise duration or aerobic capacity (peak oxygen uptake) were observed. Gastrointestinal side effects were more common during resveratrol treatment (77% vs. 15%, p = 0.0048). A small reduction in fasting glucose and small but statistically significant increases in liver enzymes, total cholesterol, and triglycerides were observed, although mean results remained within normal limits. There was no change in complete blood count, inflammatory markers, renal function, or other measures of liver function.

Birt-Hogg-Dube syndrome (BHDS) is a rare form of classically cystic lung disease that may present with spontaneous pneumothorax. The associated skin manifestations (fibrofolliculomas) are not always present. This article describes a case of spontaneous pneumothorax secondary to bullous emphysema in an otherwise healthy gentleman caused by a novel mutation in the folliculin (FLCN) gene.

Flexible bronchoscopy is performed by clinicians representing multiple medical specialties in a variety of settings. Given the increasing importance of competency-based assessment in postgraduate training, it is important that this skill be assessed within a competency-based framework using a valid measurement tool.The purpose of this study was to design and validate a practical, competency-based bronchoscopy assessment tool that could be applied to trainees in a clinical setting.Focus groups of expert physicians were formed in Ottawa, Canada representing adult medical specialties routinely engaged in preparing trainees to perform flexible bronchoscopy (respiratory medicine, critical care, thoracic surgery and anesthesia). The focus groups were charged with identifying themes and items relevant to the assessment of competency in bronchoscopy. By an iterative process, a bronchoscopy assessment tool was developed, the Ontario Bronchoscopy Assessment Tool (OBAT). The tool was evaluated by first using it to assess learners in a pilot study, refining it based on the results, and then testing the OBAT again in a validation study.The initial tool consisted of 19 items, organized into the following groups: preprocedure planning, sedation and monitoring, technical skill, diagnostic skill, and post-procedure planning. The tool demonstrated high reliability (0.91) and discriminated junior from senior trainees. Based on the results of the pilot, the tool was simplified to a 12-item scale with three subscales: preprocedure planning, technical skills, and post-procedure planning. In the validation study, the assessment tool remained highly reliable (0.92) and discriminated junior from senior trainees with an estimated eight assessments per trainee.The OBAT demonstrates promise as a reliable tool to assess trainee competence for bronchoscopy in clinical settings.

Acute exacerbations of chronic obstructive disease (AECOPD) are characterised by worsening dyspnoea that is variably prolonged. In this study, physiological changes during moderate AECOPD were examined and the factors associated with dyspnoea resolution over time were determined. In total, 20 patients experiencing an AECOPD were evaluated within 72 h of initial worsening of symptoms (day 0) with pulmonary function testing, metabolic testing and symptom assessment using the dyspnoea domain of the Chronic Respiratory Disease Questionnaire (CRQ). Treatment was optimised and testing was repeated after 7, 14, 30 and 60 days. At day 0, patients were very short of breath (CRQ-dyspnoea mean+/-SEM 2.4+/-0.3) and showed significant airflow obstruction (forced expiratory volume in one second (FEV1) 41+/-3% predicted) and lung hyperinflation (forced residual capacity (FRC) 164+/-7% pred). By day 60 CRQ-dyspnoea improved to 4.6+/-0.5 (some shortness of breath); FRC and residual volume decreased by 5 and 11%, respectively; inspiratory capacity (IC) and slow vital capacity increased by 18 and 17%, respectively; and FEV1 increased by 18% with no change in FEV1/FVC. Total lung capacity did not change during AECOPD, and thus, changes in IC reliably reflected changes in end-expiratory lung volume. In conclusion, moderate acute exacerbation of chronic obstructive pulmonary disease is characterised by worsening airflow obstruction and lung hyperinflation. Improvement of dyspnoea following acute exacerbations of chronic obstructive pulmonary disease was associated with reduction in lung hyperinflation and consequent increase in expiratory flow rates.

The oral and nasal contributions to inhaled ventilation were simultaneously quantified during sleep in 10 healthy subjects (5 men, 5 women) aged 43 ± 5 yr, with normal nasal resistance (mean 2.0 ± 0.3 cmH2O · l−1 · s−1) by use of a divided oral and nasal mask. Minute ventilation awake (5.9 ± 0.3 l/min) was higher than that during sleep (5.2 ± 0.3 l/min; P < 0.0001), but there was no significant difference in minute ventilation between different sleep stages ( P = 0.44): stage 2 5.3 ± 0.3, slow-wave 5.2 ± 0.2, and rapid-eye-movement sleep 5.2 ± 0.2 l/min. The oral fraction of inhaled ventilation during wakefulness (7.6 ± 4%) was not significantly different from that during sleep (4.3 ± 2%; mean difference 3.3%, 95% confidence interval −2.1–8.8%, P = 0.19), and no significant difference ( P = 0.14) in oral fraction was observed between different sleep stages: stage two 5.1 ± 2.8, slow-wave 4.2 ± 1.8, rapid-eye-movement 3.1 ± 1.7%. Thus the inhaled oral fraction in normal subjects is small and does not change significantly with sleep stage.

117 Background: As childhood cancer survival approaches 85%, it is increasingly recognized that childhood cancer survivors (CCS) are at risk for long-term therapy-related complications. Data suggest that pulmonary function test (PFT) abnormalities occur in as many as 84.1% of CCS. Our aim was to assess the prevalence and nature of respiratory abnormalities in adult CCS and to correlate these abnormalities with therapeutic exposures and health behaviors. Methods: Patients who received pulmonary toxic therapy routinely undergo PFTs at their first visit to the POGO AfterCare clinic in Ottawa, Canada. We conducted a retrospective review of all patients seen in clinic from August 2015 until December 2016. PFT results, treatment exposures and health habits were documented. We used NHANES-III prediction equations for PFT interpretation, with expiratory flows and lung volumes of ≥80% predicted as cutoffs for normal. Results: Of a total of 200 patients, 48 received pulmonary toxic therapy and 38 underwent PFTs. Mean age at the time of PFT was 28.8 years (18.5-57.5 years) and the mean time from diagnosis was 17.4 years (3.4-47.5 years). Although no patients reported respiratory symptoms, 42% (16/38) demonstrated a mild restrictive ventilatory defect, 11% (4/38) had mild airflow obstruction, 18% (7/38) had an isolated reduction in diffusion capacity and 1 patient had a moderate mixed obstructive/restrictive abnormality. Only 26% (10/38) had normal lung function. Of those with restrictive defects, all received radiation and 50% were also exposed to pulmonary toxic chemotherapy. All patients with obstructive defects received radiation therapy in combination with bleomycin. Tobacco or marijuana use was reported in 26% (10/38) of the cohort, and in 29% (8/28) of those with PFT abnormalities. Conclusions: PFT abnormalities are common among adult CCS who received pulmonary toxic therapy. Studies evaluating the change in pulmonary function abnormalities over time and their correlation to cardiopulmonary exercise testing and lung imaging are required to inform the health impact on these survivors.

Cardiopulmonary exercise testing (CPET) permits measurement of oxygen uptake (Vo2), an indicator of overall cardiopulmonary fitness and a useful measurement in the assessment of operative risk for lung cancer patients. The evidence supporting the use of CPET in pre-operative assessment of the lung cancer surgery patient is examined. CPET methodology and limitations, as well as alternatives to CPET for risk assessment are discussed.

Nha Voduc1, Gonzalo G Alvarez1, Kayvan Amjadi1, Caroline Tessier2, Elham Sabri3, Shawn D Aaron11University of Ottawa, Division of Respirology, 2The Ottawa Hospital (General Campus), 3OHRI Methods Centre, Ottawa, ON, CanadaBackground: Many patients with chronic obstructive pulmonary disease continue to experience significant functional limitation despite the use of both long-acting anticholinergic and beta-agonist inhalers. Theophylline is a widely available medication which may further improve lung function and exercise performance. Previous studies evaluating the effects of theophylline on exercise capacity in chronic obstructive pulmonary disease (COPD) have demonstrated heterogeneous results.Methods: We performed a randomized placebo-controlled double-blind pilot study assessing the effects of theophylline on constant load exercise duration and lung function, involving 24 COPD patients already treated with long-acting inhaled beta-agonist and long-acting anticholinergic bronchodilator therapy.Results: Analyzable data was available in 10 of 12 subjects in the treatment arm and 11 of 12 subjects in the control arm. Theophylline was associated with a 26.1% (95% confidence interval [CI]: –17.3–69.5) improvement in exercise duration compared to placebo. Four of 10 treated patients demonstrated an improvement in exercise duration exceeding the minimum clinically important difference of 33%, compared to 1 of 11 controls (P = 0.15). Furthermore, peak ventilation was reduced by 11.1%, (95% CI: 0.77–21.5) which may suggest improvements in gas exchange. There were no significant observed differences in resting lung function nor measures of dyspnea between the two treatment groups.Conclusions: Our study demonstrated a trend, but not a statistically significant improvement in exercise duration and a reduction in peak ventilation with theophylline. Based on the observed mean differences and standard deviations in this pilot study, a randomized controlled trial would require 45 subjects in each arm to detect a significant change in exercise duration.Keywords: theophylline, chronic obstructive pulmonary disease, exercise capacity

BACKGROUND: Tracheal bronchus is a congenital malformation comprising an abnormal bronchus originating from the trachea or other bronchus. This malformation has been associated with recurrent pneumonia in children, but is rarely associated with infection in adults.Actinomycesspecies are rare causes of necrotizing pneumonias that often masquerade as malignancy, lung abscesses and tuberculosis.METHODS AND RESULTS: A case involving a 46-year-old man with a tracheal bronchus and chronic pneumonia syndrome is presented. Bronchialveolar lavage and transthoracic needle biopsy demonstrated the presence ofActinomyces meyeriandFusobacteriumspecies.CONCLUSIONS: The present article reports the first documented case of actinomycosis occurring in a patient with a tracheal bronchus.

Trans-resveratrol is a polyphenol, which is found in red wine and has cancer chemo-preventive properties and disease-preventive properties. The pharmacokinetics of trans-resveratrol have been investigated in single-dose studies and in studies with relatively low dosages. The present study aimed to investigate the steady-state pharmacokinetics and tolerability of trans-resveratrol 2000 mg twice daily with food, quercetin and alcohol (ethanol). This was a two-period, open-label, single-arm, within-subject control study in eight healthy subjects. The steady-state 12-hour pharmacokinetics of trans-resveratrol 2000 mg twice daily were studied with a standard breakfast, a high-fat breakfast, quercetin 500 mg twice daily and 5% alcohol 100 mL. Trans-resveratrol plasma concentrations were determined using liquid chromatography with tandem mass spectrometry. The mean (SD) area under the plasma concentration-time curve from 0 to 12 hours (AUC12) and maximum plasma concentration (Cmax) of trans-resveratrol were 3558 (2195) ng •h/mL and 1274 (790) ng/mL, respectively, after the standard breakfast. The high-fat breakfast significantly decreased the AUC12 and Cmax by 45% and 46%, respectively, when compared with the standard breakfast. Quercetin 500 mg twice daily or 5% alcohol 100mL did not influence trans-resveratrol pharmacokinetics. Diarrhoea was reported in six of the eight subjects. Significant but not clinically relevant changes from baseline were observed in serum potassium and total bilirubin levels. Trans-resveratrol 2000 mg twice daily resulted in adequate exposure and was well tolerated by healthy subjects, although diarrhoea was frequently observed. In order to maximize trans-resveratrol exposure, it should be taken with a standard breakfast and not with a high-fat meal. Furthermore, combined intake with quercetin or alcohol did not influence trans-resveratrol exposure.

BACKGROUND: Supplemental oxygen therapy has been shown to improve exercise performance in patients with chronic obstructive pulmonary disease (COPD). It is unknown whether the magnitude of this benefit would be affected by participation in a pulmonary rehabilitation program.OBJECTIVE: To compare the effects of supplemental oxygen on exercise capacity in nonhypoxemic COPD patients before and after participation in a pulmonary rehabilitation program.METHODS: Sixteen patients with COPD underwent two pairs of constant-load exercise tests before and after participation in a three-month outpatient pulmonary rehabilitation program. Each pair of exercise tests consisted of a test performed with room air and a second test performed with 50% supplemental oxygen, in random order. The primary outcome was the difference in exercise duration between tests performed with supplemental oxygen and with room air. This difference was compared before and after participation in a pulmonary rehabilitation program.RESULTS: Supplemental oxygen therapy improved exercise duration by 75 s before participation in a pulmonary rehabilitation program and by 153 s following pulmonary rehabilitation. Rehabilitation alone improved exercise duration by 28 s, but rehabilitation appeared to augment the exercise benefits of supplemental oxygen therapy by a mean of 78 s (95% CI 11 s to 145 s; P=0.03).CONCLUSION: The effects of supplemental oxygen therapy were augmented after pulmonary rehabilitation. The improvement in exercise duration with supplemental oxygen following rehabilitation was greater than either supplemental oxygen or pulmonary rehabilitation alone.

Chronic obstructive pulmonary disease (COPD) is a major respiratory illness in Canada that is preventable and treatable but unfortunately remains underdiagnosed. The purpose of the present article from the Canadian Thoracic Society is to provide up-to-date information so that patients with this condition receive optimal care that is firmly based on scientific evidence. Important summary messages for clinicians are derived from the more detailed Update publication and are highlighted throughout the document. Three key messages contained in the update are: use targeted screening spirometry to establish a diagnosis and initiate prompt management (including smoking cessation) of mild COPD; improve dyspnea and activity limitation in stable COPD using new evidence-based treatment algorithms; and understand the importance of preventing and managing acute exacerbations, particularly in moderate to severe disease.

Chronic obstructive pulmonary disease (COPD) is a major respiratory illness in Canada that is both preventable and treatable. Our understanding of the pathophysiology of this complex condition continues to grow and our ability to offer effective treatment to those who suffer from it has improved considerably. The purpose of the present educational initiative of the Canadian Thoracic Society (CTS) is to provide up to date information on new developments in the field so that patients with this condition will receive optimal care that is firmly based on scientific evidence. Since the previous CTS management recommendations were published in 2003, a wealth of new scientific information has become available. The implications of this new knowledge with respect to optimal clinical care have been carefully considered by the CTS Panel and the conclusions are presented in the current document. Highlights of this update include new epidemiological information on mortality and prevalence of COPD, which charts its emergence as a major health problem for women; a new section on common comorbidities in COPD; an increased emphasis on the meaningful benefits of combined pharmacological and nonpharmacological therapies; and a new discussion on the prevention of acute exacerbations. A revised stratification system for severity of airway obstruction is proposed, together with other suggestions on how best to clinically evaluate individual patients with this complex disease. The results of the largest randomized clinical trial ever undertaken in COPD have recently been published, enabling the Panel to make evidence-based recommendations on the role of modern pharmacotherapy. The Panel hopes that these new practice guidelines, which reflect a rigorous analysis of the recent literature, will assist caregivers in the diagnosis and management of this common condition.

This study examined the effects of bronchodilator-induced reductions in lung hyperinflation on breathing pattern, ventilation and dyspnoea during exercise in chronic obstructive pulmonary disease (COPD). Quantitative tidal flow/volume loop analysis was used to evaluate abnormalities in dynamic ventilatory mechanics and their manipulation by a bronchodilator. In a randomised double-blind crossover study, 23 patients with COPD (mean +/- SEM forced expiratory volume in one second 42 +/- 3% of the predicted value) inhaled salmeterol 50 microg or placebo twice daily for 2 weeks each. After each treatment period, 2 h after dose, patients performed pulmonary function tests and symptom-limited cycle exercise at 75% of their maximal work-rate. After salmeterol versus placebo at rest, volume-corrected maximal expiratory flow rates increased by 175 +/- 52%, inspiratory capacity (IC) increased by 11 +/- 2% pred and functional residual capacity decreased by 11 +/- 3% pred. At a standardised time during exercise, salmeterol increased IC, tidal volume (VT), mean inspiratory and expiratory flows, ventilation, oxygen uptake (VO2) and carbon dioxide output. Salmeterol increased peak exercise endurance, VO2 and ventilation by 58 +/- 19, 8 +/- 3 and 12 +/- 3%, respectively. Improvements in peak VO2 correlated best with increases in peak VT; increases in peak VT and resting IC were interrelated. The reduction in dyspnoea ratings at a standardised time correlated with the increased VT. Mechanical factors play an important role in shaping the ventilatory response to exercise in chronic obstructive pulmonary disease. Bronchodilator-induced lung deflation reduced mechanical restriction, increased ventilatory capacity and decreased respiratory discomfort, thereby increasing exercise endurance.

McArdle's disease is a rare, inherited deficiency of myophosphorylase, an enzyme required for the utilization of glycogen. Patients with myophosphorylase deficiency classically present with exercise intolerance, leg pain and muscle fatigue. The case of a young woman with exertional dyspnea and leg cramps is described. Exercise testing confirmed the presence of exercise intolerance and demonstrated an accelerated heart rate response, despite the absence of an anaerobic threshold and a respiratory exchange ratio of less than 1.0. Subsequent ischemic forearm testing and muscle biopsy confirmed the diagnosis of myophosphorylase deficiency. Evaluation of lung mechanics with esophageal pressure measurements demonstrated the presence of respiratory muscle weakness and early fatiguability, suggesting that the patient's dyspnea might have been attributable to an increased respiratory effort. Dyspnea is not a classic symptom associated with myophosphorylase deficiency, although subclinical respiratory muscle impairment may be present. No previous studies have evaluated respiratory muscle function during exercise in patients with myophosphorylase deficiency.

Background: Interventional bronchoscopic procedures can re-establish airway patency in the majority of individuals suffering from endobronchial obstruction. However, literature provides limited data on the impact of these procedures on the individual’s quality of life (QoL). Objectives: We evaluated the impact of interventional bronchoscopic procedures aimed at re-establishing airway patency on symptoms and QoL of individuals who do not receive concomitant chemotherapy or radiation therapy. Methods: Over a 6-month period, we prospectively enrolled patients with symptomatic central airway obstruction related to malignancy and performed laser ablation, cryotherapy, and/or airway stenting in order to re-establish airway patency. QoL was evaluated by the European Organization for Research and Treatment of Cancer Quality of Life Questionnaire at one day before, 7 days after, and 1 month after procedure. Dyspnea was also assessed by the Borg scale before the procedure and one day after. Results: Final analysis could be performed on 83% (20/24) of the participants. Improvement in airway diameter was achieved in all patients, and >80% patency was established in 80% (16/20) of the patients. Dyspnea scores had improved in 85% of participants (p = 0.01). Thirteen patients experienced an improvement in QoL, although QoL scores remained stable for the group as a whole. Conclusion: Endoscopic procedures can effectively reduce dyspnea associated with malignant central airway obstruction, but this may not always translate into improvements in overall QoL. Administration of QoL questionnaires is feasible, and may help identify domains contributing to patient’s decline, and thereby improve overall patient care.