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BackgroundRisk factors for COPD in high-income settings are well understood; however, less attention has been paid to contributors of COPD in low-income and middle-income countries (LMICs) such as pulmonary tuberculosis. We sought to study the association between previous tuberculosis disease and COPD by using pooled population-based cross-sectional data in 13 geographically diverse, low-resource settings.MethodsWe pooled six cohorts in 13 different LMIC settings, 6 countries and 3 continents to study the relationship between self-reported previous tuberculosis disease and lung function outcomes including COPD (defined as a postbronchodilator forced expiratory volume in one second (FEV1)/forced vital capacity (FVC) below the lower limit of normal). Multivariable regressions with random effects were used to examine the association between previous tuberculosis disease and lung function outcomes.ResultsWe analysed data for 12 396 participants (median age 54.0 years, 51.5% male); 332 (2.7%) of the participants had previous tuberculosis disease. Overall prevalence of COPD was 8.8% (range 1.7%–15.5% across sites). COPD was four times more common among those with previous tuberculosis disease (25.7% vs 8.3% without previous tuberculosis disease, p1(−0.70, 95% CI −0.84 to −0.55), FVC (−0.44, 95% CI −0.59 to −0.29) and the FEV1:FVC ratio (−0.63, 95% CI −0.76 to −0.51) when compared with those without previous tuberculosis disease.ConclusionsPrevious tuberculosis disease is a significant and under-recognised risk factor for COPD and poor lung function in LMICs. Better tuberculosis control will also likely reduce the global burden of COPD.

Context Chronic obstructive lung disease (COPD) is a progressive lung disease characterized by persistent airflow limitation. An increasing amount of evidence suggests an effect of dietary quality on the risk of COPD in the general population and pulmonary function decline in patients with COPD. Objective The association of dietary intake and nutrient status with COPD risk and onset, as well as pulmonary function decline (change in forced expiratory volume in 1 second, forced vital capacity, or the ratio of the former to the latter) in patients with COPD was investigated in this systematic review. Data Sources The PubMed database was searched by combining terms of pulmonary function or COPD with diet, nutrient status, or nutritional supplementation. Data Extraction Original studies and systematic reviews and meta-analyses were included. Articles obtained were independently screened for relevance on the bases of title and abstract by 2 researchers. Eventually, 89 articles were included in the analysis. Results The unhealthy Western-style diet is associated with an increased risk of COPD and an accelerated decline of pulmonary function. Intake of fruit, vegetables, dietary fibers, vitamins C and E, polyphenols, and β-carotene were individually associated with lower COPD risk, whereas consumption of processed meat was associated with higher COPD risk. Data on the effect of dietary quality on pulmonary function decline in patients with COPD are limited and inconsistent. Strong evidence for beneficial effects on pulmonary function decline was found only for vitamin D supplementation. Conclusion Considering the increasing burden of COPD, more attention should be given to dietary quality as a modifiable factor in disease development and progression in patients with COPD. Systematic Review Registration PROSPERO registration no. CRD42021240183.

Nontuberculous mycobacterial (NTM) infections are difficult to diagnose and treat. Biomarkers to identify patients with active infection or at risk of disease progression would have clinical utility. Sputum is the most frequently used matrix for the diagnosis of NTM lung disease.Can sputum proteomics be used to identify NTM-associated inflammatory profiles in sputum?Patients with NTM lung disease and a matched cohort of patients with COPD, bronchiectasis (BE), and cystic fibrosis (CF) without NTM lung disease were enrolled from two hospitals in the United Kingdom. Liquid chromatography-tandem mass spectrometry was used to identify proteomic biomarkers associated with underlying diagnosis (COPD, BE, and CF), the presence of NTM lung disease defined according to American Thoracic Society/Infectious Diseases Society of America criteria, and severity of NTM. A subset of patients receiving guideline-concordant NTM treatment were studied to identify protein changes associated with treatment response.This study analyzed 95 sputum samples from 55 subjects (BE, n = 21; COPD, n = 19; CF, n = 15). Underlying disease and infection with Pseudomonas aeruginosa were the strongest drivers of sputum protein profiles. Comparing protein abundance in COPD, BE, and CF found that 12 proteins were upregulated in CF compared with COPD, including MPO, AZU1, CTSG, CAT, and RNASE3, with 21 proteins downregulated, including SCGB1A1, IGFBP2, SFTPB, GC, and CFD. Across CF, BE, and COPD, NTM infection (n = 15) was not associated with statistically significant differences in sputum protein profiles compared with those without NTM. Two proteins associated with iron chelation were significantly downregulated in severe NTM disease. NTM treatment was associated with heterogeneous changes in the sputum protein profile. Patients with NTM and a decrease in immune response proteins had a subjective symptomatic improvement.Sputum proteomics identified candidate biomarkers of NTM severity and treatment response. However, underlying lung disease and typical bacterial pathogens such as P aeruginosa are also key determinants of the sputum proteomic profile.

Background Small airways are known to be affected early in the course of chronic obstructive pulmonary disease (COPD); however, traditional spirometric indices may not accurately identify small airways disease. Research Question Can FEV3/FEV6 identify early airflow abnormalities and predict future clinically important respiratory-related outcomes, including development of COPD? Study Design and Methods We included eight hundred thirty-two current and former smokers with post-bronchodilator FEV1/FVC ≥0.7 from the SPIROMICS cohort. Participants were classified as having a reduced pre-bronchodilator FEV3/FEV6 based on lower limit of normal (LLN) values. Repeatability analysis was performed for FEV3 and FEV6. Regression modeling was used to evaluate the relationship between baseline FEV3/FEV6 and outcome measures including functional small airways disease on thoracic imaging and respiratory exacerbations. Interval censored analysis was used to assess progression to COPD. Results FEV3/FEV6 Interpretation FEV3/FEV6 is a routinely available and repeatable spirometric index which can be useful in the evaluation of early airflow obstruction in current and former smokers without COPD. A reduced FEV3/FEV6 can identify those at risk for future development of COPD and respiratory exacerbations.

Rationale: Tocilizumab, an anti–interleukin-6 receptor antibody, had no statistically significant effect on skin sclerosis but preserved lung function over 48 weeks in patients with early systemic ...

Background Individuals with COPD have increased sensitivity to traffic-related air pollution (TRAP) such as diesel exhaust (DE), but little is known about the acute effects of TRAP on exercise responses in COPD. Research question Does pre-exercise exposure to TRAP (DE300, PM2.5=300 μg/m3) have greater adverse effects on exercise endurance, exertional dyspnea, and cardiorespiratory responses to exercise in participants with mild-to-moderate COPD compared to former smokers with normal spirometry and healthy controls? Study design and Methods In this double-blind, randomized, placebo-controlled, crossover study, 11 healthy never-smokers, 9 ex-smokers without COPD and 9 ex-smokers with COPD were separately exposed to filtered air (FA) and DE300 for 2 hours separated by a minimum of 4 weeks. Participants performed symptom limited constant load cycling tests within 2.5 hours of exposure with detailed cardiorespiratory and exertional symptom measurements. Results There was a significant negative effect of TRAP on exercise endurance time in healthy controls (DE300 vs. FA: 10.2±8.2 vs. 12.9±9.5 min, respectively; p=0.03), but not in ex-smokers without COPD (10.1±6.9 vs. 12.2±8.0 min; respectively, p=0.57) or ex-smokers with COPD (9.8±6.4 vs. 8.4±6.6 min, respectively, p=0.31). Furthermore, significant increases in inspiratory duty cycle and absolute end-expiratory and end-inspiratory lung volumes were observed, and dyspnea ratings were elevated at select submaximal measurement times only in healthy controls. Interpretation Contrary to our hypothesis, it was the healthy controls rather than the ex-smokers with and without COPD that were negatively impacted by TRAP during exercise.

BACKGROUND: Emerging data from longitudinal studies suggest that preserved ratio impaired spirometry (PRISm), defined by proportionate reductions in FEV(1) and FVC, is a heterogeneous population with frequent transitions to other lung function categories relative to individuals with normal and obstructive spirometry. Controversy regarding the clinical significance of these transitions exists (eg, whether transitions merely reflect measurement variability or noise). RESEARCH QUESTION: Are individuals with PRISm enriched for transitions associated with substantial changes in lung function? STUDY DESIGN AND METHODS: Current and former smokers enrolled in the Genetic Epidemiology of COPD (COPDGene) study with spirometry available in phases 1 through 3 (enrollment, 5-year follow-up, and 10-year follow-up) were analyzed. Postbronchodilator lung function categories were as follows: PRISm (FEV(1) < 80% predicted with FEV(1)/FVC ratio ≥ 0.7), Global Initiative for Chronic Obstructive Lung Disease grade 0 (FEV(1) ≥ 80% predicted and FEV(1)/FVC ≥ 0.7), and obstruction (FEV(1)/FVC < 0.7). Significant transition status was affirmative if a subject belonged to two or more spirometric categories and had > 10% change in FEV(1) % predicted and/or FVC % predicted between consecutive visits. Ever-PRISm was present if a subject had PRISm at any visit. Logistic regression examined the association between significant transitions and ever-PRISm status, adjusted for age, sex, race, FEV(1) % predicted, current smoking, pack-years, BMI, and ever-positive bronchodilator response. RESULTS: Among subjects with complete data (N = 1,775) over 10.1 ± 0.4 years of follow-up, the prevalence of PRISm remained consistent (10.4%-11.3%) between phases 1 through 3, but nearly one-half of subjects with PRISm transitioned into or out of PRISm at each visit. Among all subjects, 19.7% had a significant transition; ever-PRISm was a significant predictor of significant transitions (unadjusted OR, 10.3; 95% CI, 7.9-13.5; adjusted OR, 14.9; 95% CI, 10.9-20.7). Results were similar with additional adjustment for radiographic emphysema and gas trapping, when lower limit of normal criteria were used to define lung function categories, and when FEV(1) alone (regardless of change in FVC % predicted) was used to define significant transitions. INTERPRETATION: PRISm is an unstable group, with frequent significant transitions to both obstruction and normal spirometry over time. CLINICAL TRIAL REGISTRATION: ClinicalTrials.gov; No.: NCT000608764; URL: www.clinicaltrials.gov

RATIONALE: In predominantly White populations, early chronic obstructive pulmonary disease (COPD) (i.e., COPD in people aged

Background Pirfenidone slows the progression of idiopathic pulmonary fibrosis. We investigated the efficacy and safety of pirfenidone by dose and disease severity in real-world patients with idiopathic pulmonary fibrosis.Methods This multi-centre retrospective cohort study investigated 338 patients treated with pirfenidone between July 2012 and March 2018. Demographics, pulmonary function, mortality, and pirfenidone-related adverse events were recorded. Efficacy was analysed according to pirfenidone dose and disease severity using linear mixed-effects models to assess the annual decline rate of forced vital capacity (FVC) and diffusing capacity (DL CO ) of the lungs for carbon monoxide.Results The mean %FVC predicted and %DL COpredicted were 72.6±13.1% and 61.4±17.9%, respectively. Pirfenidone treatment lasted 16.1±9.0 months. In the standard-dose (1800 mg/day) group, the mean %FVC predicted was -6.56% (95% CI -9.26, -3.87) per year pre-pirfenidone treatment, but -4.43% (95% CI -5.87, -3.00) per year post-treatment. In the lower-dose group, the mean %FVC predicted was -4.96% (95% CI -6.82, -3.09) per year pre-pirfenidone treatment, but -1.79% (95% CI -2.75, -0.83) per year post-treatment. The FVC decline rate was significantly reduced regardless of GAP stage. However, the DL CO decline rate was significantly reduced in the GAP stage II–III group, but not stage I group. Adverse events and mortality were similar across dose groups, but were more frequent in the GAP stage II–III group than in the stage I group.Conclusions The effect of pirfenidone on reducing disease progression persisted even with a consistent lower dose of pirfenidone.Trial registration Retrospectively registered

Background The adherence to and clinical efficacy of pulmonary rehabilitation in idiopathic pulmonary fibrosis (IPF), particularly in comparison to people with chronic obstructive pulmonary disease (COPD), remains uncertain. The objectives of this real-world study were to compare the responses of patients with IPF with a matched group of patients with COPD undergoing the same supervised, outpatient pulmonary rehabilitation program, and to determine whether pulmonary rehabilitation is associated with survival in IPF. Research question Do people with IPF improve to the same extent with pulmonary rehabilitation as a matched group of individuals with COPD, and are non-completion of and/or non-response to pulmonary rehabilitation associated with one-year all-cause mortality in IPF? Study design and methods Using propensity score matching, 163 patients with IPF were matched 1:1 with a control group of 163 patients with COPD referred to pulmonary rehabilitation. We compared between-group pulmonary rehabilitation completion rates and response. Survival status in the IPF cohort was recorded over one-year following pulmonary rehabilitation discharge. Cox proportional-hazards regression explored the association between pulmonary rehabilitation status and all-cause mortality. Results Similar pulmonary rehabilitation completion rates (IPF: 69%; COPD: 63%; p=0.24) and improvements in exercise response were observed in both groups with no significant mean (95% confidence interval (CI)) between-group differences in incremental shuttle walk (ISW) change (2 (-18 to 22) meters). Pulmonary rehabilitation non-completion (hazard ratio (HR) (95%CI) 5.62 (2.24 to 14.08)) and non-response (HR (95%CI) 3.91 (1.54 to 9.93)) were independently associated with increased one-year all-cause mortality in IPF. Interpretation Compared with a matched group of patients with COPD, this real-word study demonstrates that patients with IPF have similar completion rates and magnitude of response to pulmonary rehabilitation. In IPF, non-completion of and non-response to pulmonary rehabilitation were associated with increased all-cause mortality. These data reinforce the benefits of pulmonary rehabilitation in patients with IPF.

Using data from the SENSCIS trial, these analyses were undertaken to assess the effects of nintedanib versus placebo in subgroups of patients with systemic sclerosis-associated interstitial lung disease (SSc-ILD), based on characteristics previously identified as being associated with the progression of SSc-ILD.Patients with SSc-ILD were randomized to receive either nintedanib or placebo, stratified by anti-topoisomerase I antibody (ATA) status. We assessed the rate of decline in forced vital capacity (FVC) (expressed in ml/year) over 52 weeks in subgroups based on baseline ATA status, modified Rodnan skin thickness score (MRSS) (18 versus ≥18), and SSc subtype (limited cutaneous SSc [lcSSc] versus diffuse cutaneous SSc [dcSSc]).At baseline, 60.8% of 576 patients who received treatment with either nintedanib or placebo were positive for ATA, 51.9% had dcSSc, and 77.5% of 574 patients with MRSS data available had an MRSS of18. The effect of nintedanib versus placebo on reducing the rate of decline in FVC (ml/year) was numerically more pronounced in ATA-negative patients compared to ATA-positive patients (adjusted difference in the rate of FVC decline, 57.2 ml/year [95% confidence interval (95% CI) -3.5, 118.0] versus 29.9 ml/year [95% CI -19.1, 78.8]), in patients with a baseline MRSS ≥18 compared to those with a baseline MRSS of18 (adjusted difference in the rate of FVC decline, 88.7 ml/year [95% CI 7.7, 169.8] versus 26.4 ml/year [95% CI -16.8, 69.6]), and in patients with dcSSc compared to those with lcSSc (adjusted difference in the rate of FVC decline, 56.6 ml/year [95% CI 3.2, 110.0] versus 25.3 ml/year [95% CI -28.9, 79.6]). However, all exploratory interaction P values were nonsignificant (all P 0.05), indicating that there was no heterogeneity in the effect of nintedanib versus placebo between these subgroups of patients.In patients with SSc-ILD, reduction in the annual rate of decline in FVC among patients receiving nintedanib compared to those receiving placebo was not found to be heterogenous across subgroups based on ATA status, MRSS, or SSc subtype.

Chronic hypersensitivity pneumonitis (cHP) is a heterogeneous condition, where both small airway involvement and fibrosis may simultaneously occur. Computer-aided analysis of CT lung imaging is increasingly used to improve tissue characterization in interstitial lung diseases (ILD), quantifying disease extension, and progression. We aimed to quantify via a convolutional neural network (CNN) method the extent of different pathological classes in cHP, and to determine their correlation to pulmonary function tests (PFTs) and mosaic attenuation pattern.The extension of six textural features, including consolidation (C), ground glass opacity (GGO), fibrosis (F), low attenuation areas (LAA), reticulation (R) and healthy regions (H), was quantified in 27 cHP patients (age: 56 ± 11.5 years, forced vital capacity [FVC]% = 57 ± 17) acquired at full-inspiration via HRCT. Each class extent was correlated to PFTs and to mosaic attenuation pattern.H showed a positive correlation with FVC%, FEV1% (forced expiratory volume), total lung capacity%, and diffusion of carbon monoxide (DLCO)% (r = 0.74, r = 0.78, r = 0.73, and r = 0.60, respectively, p0.001). GGO, R and C negatively correlated with FVC% and FEV1% with the highest correlations found for R (r = -0.44, and r = -0.46 respectively, p0.05); F negatively correlated with DLCO% (r = -0.42, p0.05). Patients with mosaic attenuation pattern had significantly more H (p = 0.04) and lower R (p = 0.02) and C (p = 0.0009) areas, and more preserved lung function indices (higher FVC%; p = 0.04 and DLCO%; p = 0.05), but did not show more air trapping in lung function tests.CNN quantification of pathological tissue extent in cHP improves its characterization and shows correlation with PFTs. LAA can be overestimated by visual, qualitative CT assessment and mosaic attenuation pattern areas in cHP represents patchy ILD rather than small-airways disease.

Introduction Idiopathic pulmonary fibrosis (IPF) is progressive and irreversible. Some discrepancies about IPF staging exists, especially in mild phases. Forced vital capacity (FVC) higher than 80% has been considered early or mild IPF even for the design of clinical trials. Methods Spanish multicentre, observational, retrospective study of IPF patients diagnosed between 2012 and 2016, based on the ATS/ERS criteria, which presented FVC greater or equal 80% at diagnosis. Clinical and demographic characteristics, lung function, radiological pattern, treatment, and follow-up were analyzed. Results 225 IPF patients were included, 72.9% were men. The mean age was 69.5 years. The predominant high-resolution computed tomography (HRCT) pattern was consistent usual interstitial pneumonia (UIP) (51.6%). 84.7% of patients presented respiratory symptoms (exertional dyspnea and/or cough) and 33.33% showed oxygen desaturation below 90% in the 6 min walking test (6MWT). Anti-fibrotic treatment was initiated at diagnosis in 55.11% of patients. Median FVC was 89.6% (IQR 17) and 58.7% of patients had a decrease of diffusion lung capacity for carbon monoxide (DLCO) below 60% of theoretical value; most of them presented functional progression (61.4%) and higher mortality at 3 years (20.45%). A statistically significant correlation with the 3-years mortality was observed between DLCO Conclusions Patients with preserved FVC but presenting UIP radiological pattern and moderate–severe DLCO decrease at diagnosis associate an increased risk of progression, death or lung transplantation. Therefore, in these cases, preserved FVC would not be representative of early or mild IPF.

Serratus anterior plane block (SAPB) has been proven to be an efficient way to control postoperative pain. This study explored whether the use of continuous SAPB in combination with flurbiprofen could improve early pulmonary function in lung cancer patients undergoing video-assisted thoracoscopic surgery (VATS).From July 2019 to April 2020, patients who scheduled for elective lung resection undergoing VATS were randomly allocated to receive patient-controlled SAPB in combination with intravenous flurbiprofen or patient-controlled intravenous analgesia. Postoperative pulmonary function variables, including forced expiratory volume in 1 second, and forced vital capacity were collected before and 24, 48, and 72 hours after Surgical Procedure. Pain intensity was measured at rest and on coughing. Comfort scores during breathing exercises, postoperative pulmonary complications, and adverse events were recorded.A substantial reduction in lung function was exhibited in both groups after Surgical Procedure (P .001), but lung function variables in the continuous SAPB group were significantly higher (P .001) throughout the postoperative period up to 72 hours, regardless of the surgical procedure type. Meanwhile, there were significant differences of pain intensity at rest and on coughing between the groups (P .001). The incidence of pneumonia, pulmonary atelectasis, hypoxemia, vomiting, and the comfort score in the continuous SAPB group was significantly lower (P.05).Postoperative acute pain treatment with continuous SAPB in combination with flurbiprofen enhanced pulmonary function and reduced postoperative pulmonary complications in lung cancer patients undergoing VATS.

Nitric oxide is a gas produced in the airways of asthmatic subjects and related to T2 inflammation. It can be measured as fractional nitric oxide (FeNO) in the exhaled air and used as a non-invasive, easy to evaluate, rapid marker. It is now widely used in many settings to determine airway inflammation. The aim of this narrative review is to report relationship between FeNO and the physiopathologic characteristics of asthmatic patients. Factors affecting FeNO levels have also been analysed as well as the impact of corticosteroid, target therapies and rehabilitation programs. Considering the availability of the test, spreading this method-ology to low income countries has also been considered as a possibility for evaluating airway inflammation and monitoring adherence to inhaled corticosteroid therapy. PubMed data search has been performed restricted to English language papers. Research was limited to studies in adults unless studies in children were the only ones reported for a particular issue. This revision could be useful to summarize the role of FeNO in relation to asthma charac-teristics and help in the use of FeNO in different clinical settings particularly in low income countries. (c) 2020 Sociedade Portuguesa de Pneumologia. Published by Elsevier Espana, S.L.U. This is an open access article under the CC BY-NC-ND license (http://creativecommons.org/licenses/by-nc-nd/4.0/).

Introduction Military personnel with a diagnosis of asthma report increased respiratory symptoms in the deployment and post-deployment periods. The long-term effect of deployment on pulmonary function in this population is unknown. This study sought to determine the effect of deployment on post-deployment pulmonary function in active duty military personnel with asthma. Materials and Methods A retrospective chart review of active duty military personnel with deployment to southwest Asia and an ICD-9 diagnosis of asthma with documented pre- and post-deployment spirometry was performed. Results A total of 642 active duty individuals with a diagnosis of asthma and documented spirometry with deployment to southwest Asia between 2006 and 2015 were identified. Of these, 76 individuals were identified with pre- and post-deployment spirometry. There was no significant change in the post-deployment forced expiratory volume at 1 second (% predicted), from 86.0 ± 14.8 to 87.6 ± 14.4 (P = .30). There was no significant change in post-deployment forced vital capacity (% predicted), from 93.8 ± 12.4 to 94.9 ± 12.1 (P = .42). The absolute change in forced expiratory volume at 1 second (L) after bronchodilator administration was decreased from pre-deployment to post-deployment (+0.31 ± 0.26 to +0.16 ± 0.23; P = .02). Conclusions There was no significant post-deployment change in spirometry in this military population with asthma deployed to southwest Asia. These findings suggest that deployment itself is not associated with any short-term deleterious effect on post-deployment spirometric measures of lung function in many military personnel with asthma.

Background:Studies have associated HIV with an increased risk of obstructive lung disease (OLD). Objectives:We aimed to identify the predictive factors for impaired lung function in an urban, African, HIV-positive population. Method:A cross-sectional study was performed in Johannesburg, South Africa, from July 2016 to November 2017. A questionnaire was administered and pre- and post-bronchodilator spirometry conducted. The predictors investigated included age, sex, antiretroviral treatment (ART) duration, body mass index, history of tuberculosis (TB) or pneumonia, occupational exposure, environmental exposure, smoking and symptoms of OLD (cough, wheeze, mucus and dyspnoea). Impaired lung function was defined as a forced expiratory volume in 1 second/forced vital capacity (FEV1/FVC) ratio of < 0.70, or below the 20th percentile of normal. Results:The 98 ART-naïve participants (mean age = 34.0, standard deviation [s.d.] = 8.2), 85 participants on first-line ART (mean age = 36.9, s.d. = 6.6) and 189 participants on second-line ART (mean age = 43.5, s.d. = 7.9) were predominantly female (65.6%). Of the participants, 64 (17.2%) had impaired lung function and 308 had normal lung function. Linear regression identified age (β = –0.003,P< 0.01), male sex (β = –0.016,P= 0.03) and history of TB or pneumonia (β = –0.024,P< 0.01) as independent predictors of a lower FEV1/FVC ratio. Following logistic regression, only a history of TB or pneumonia (odds ratio = 2.58, 95% confidence interval = 1.47–4.52) was significantly related to impaired lung function (area under the receiver operating characteristic curve = 0.64). Conclusion:Our data show that a history of TB or pneumonia predicts impaired lung function. In order to improve timely access to spirometry, clinicians should be alert to the possibility of impaired lung function in people with a history of TB or pneumonia.

BACKGROUND Pulmonary rehabilitation (PR) has proven useful in patients with chronic obstructive pulmonary disease (COPD),but the benefits decrease over time. We evaluated the effects of a structured follow-up program after PR on patient pulmonary function, dyspnea, body composition, exercise capacity, quality of life, psychological status, i-BODE index, hospitalization status, 5-year survival rate. We explored whether this follow-up program could serve as a maintenance program. METHOD COPD patients who completed PR attended follow-up visits over 5 years. We administered incremental (ISWT), endurance shuttle walk tests (ESWT), measured body (BMI), fat-free mass indices (FFMI), recorded modified Medical Research Council (mMRC), St. George?s Respiratory Questionnaire (SGRQ), anxiety-depression scores. We also noted the forced expiratory volume in 1s (FEV1), the forced vital capacity (FVC), the forced mid-expiratory flow (FEF25-75), hospitalization, survival rates before, after PR, and in years 1, 2, 3, 5. This was a retrospective observational study. RESULTS Thirty-three COPD patients with a mean age of 58±8 years were enrolled. Twenty-seven (82%) were male. The mean FEV1 was 47±16% of the predicted. After PR, the mMRC scale, SGRQ, anxiety, depression scores; i-BODE index; ISWT, ESWT results improved (all p

Background: Advances in aerosol technology have improved drug delivery efficiency during noninvasive ventilation (NIV). Clinical evaluation of the efficacy of aerosol therapy during NIV in the treatment of acute exacerbation of chronic obstructive pulmonary disease (COPD) is very limited. The aim of our study was to compare the efficacy of bronchodilators administered through a vibrating mesh nebulizer (VMN) and jet nebulizer (JN) during NIV in patients with acute exacerbation of COPD. Methods: Prospective randomized cross-over study included 30 patients treated with NIV for acute exacerbation of COPD in an acute care hospital. Patients were consented and enrolled after stabilization of acute exacerbation (3-5 days after admission). Subjects were randomly assigned into two treatment arms receiving salbutamol (2.5 mg): with VMN (Aerogen Solo) and JN (Sidestream) positioned between the leak port and the nonvented oronasal mask during bilevel ventilation with a single-limb circuit. Measurements (clinical data, pulmonary function tests [PFTs], and arterial blood gases) were performed at baseline, 1, and 2 hours after treatment. Results: All measured PFT parameters significantly increased in both groups, but numerically results were better after inhalation with VMN than with JN: for forced expiratory volume in 1 second (FEV1) (mean increase from baseline to 120 minutes-165 ± 64 mL vs. 116 ± 46 mL, p = 0.001) and for forced vital capacity (FVC) (mean increase-394 ± 154 mL vs. 123 ± 57 mL, p < 0.001). There was also a statistically significant reduction in respiratory rate and in Borg dyspnea score after therapy with VMN in comparison with the conventional JN. In both groups, there were improvements in PaCO2, but with VMN these changes were significantly higher. Conclusion: Bronchodilator administration in patients with acute exacerbation of COPD during NIV with VMN resulted in clinically significant improvements in FVC and in Borg dyspnea score. Additional studies required to determine the impact on clinical outcomes.

Pulmonary function testing (PFT) is an important component of the evaluation, monitoring, and management of patients with suspected or established lung disease. Spirometry is easily accomplished in the primary care office setting; determination of lung volumes and diffusion capacity is performed in a pulmonary laboratory. Spirometry evaluates vital capacity of the lungs and expiratory flow rates and provides both numeric data and a graphic depiction of respiratory air flow. Characteristic patterns in spirometry-derived data allow the clinician to identify potential lung disease, as well as establish relative reversibility of airflow abnormalities.

Asthma is one of the most common chronic diseases, which is a growing public health concern worldwide. In recent years, there has been an increasing number of interests in the relationship between vitamin D level and asthma control. Hence, the objective of this study was to assess the effect of high doses of vitamin D3 injection on asthmatic patient’s respiratory condition and quality of life. This was a single arm, before and after interventional study involving 18 patients with moderate to severe asthma. Spirometry test, St George’s respiratory questionnaire (SGRQ) and serum vitamin D assay were performed. Subjects with 25-hydroxyvitamin D3 (25-(OH) D3)

Rationale Identifying neonatal and post-discharge exposures among extremely low gestational age newborns (ELGANs) that drive increased pulmonary morbidity and abnormal lung function at 1 year of age proves challenging. Objective The NIH-sponsored Prematurity and Respiratory Outcomes Program (PROP), evaluated infant pulmonary function tests (iPFTs) at one year corrected age to determine which demographic and clinical factors are associated with abnormal lung function. Methods Infant pulmonary function tests were performed on a PROP subcohort of 135 participants following IRB- approved written consent. Demographic data, Neonatal Intensive Care Unit (NICU) clinical care and post-NICU exposures were analyzed for association with iPFTs. Main results A significant decrease in forced expiratory volume at 0.5 seconds (FEV0.5 ) and/or forced expiratory flows at 75% of forced vital capacity (FEF75 ), were associated with male sex and African American race. Clinical factors including longer duration of ventilatory support, exposure to systemic steroids, and weight less than the 10th percentile at 36 weeks post-menstrual age were also associated with airflow obstruction, whereas supplemental oxygen requirement and bronchopulmonary dysplasia were not. Additionally, the need for respiratory medications, technology or hospitalizations during the first year, ascertained by a quarterly survey, were the only post- NICU factors associated with decreased FEV0.5 and FEF75 . Only 7% of infants had reversible airflow obstruction. Conclusions Neonatal demographic factors, respiratory support in the NICU, and a history of greater post-NICU medical utilization for respiratory disease had the strongest association with lower lung function at one year in ELGANs. This article is protected by copyright. All rights reserved.

Background: Spinal cord injury (SCI) is the injury of the spinal cord from the foramen magnum to the cauda equina which occurs as a result of compulsion, incision or contusion which is a life threatening condition that carries a high risk of morbidity and mortality. The Incidence of SCI varies from 9.2 to 56.1 per million. In the Indian setup, Approx. 20,000 new cases of SCI are added every year. Spinal cord injury (SCI) patients are at increased risk of chronic respiratory symptoms as Respiratory muscle paralysis both restricts maximum inflation of the lungs and impair the ability to cough. Patients with tetraplegia have decreased chest wall and lung compliance, rib cage stiffness with paradoxical chest wall movements. Material and method: A Pilot study was carried out on 04 Traumatic Spinal cord injury patients. The purpose of the study was To find out effectiveness of respiratory PNF on pulmonary functions (FVC & FEV1) & Chest Expansion in patients with spinal cord injury. The pulmonary Functions (FVC & FEV1) and chest expansion was measured by using Spirometer (PFT machine) and inch tape. The data was analyzed using standard statistical software. Result: In this study After 4 weeks of Intervention there was Significant Improvement in Pulmonary Functions ( Pre Mean of FEV1 & FVC 0.99 L & 1.03L and Post Mean of FEV1 & FVC was 1.25 L & 1.35L respectively) and Chest Expansion (Pre Mean was 1.15 cm and Post Mean was 1.62 cm at Xiphoid Process Level). Conclusion: This Study concluded that Respiratory PNF increases Pulmonary Functions and chest expansion in Patients with Spinal Cord Injury after 4 weeks of Intervention. Keywords: Spinal Cord Injury, Forced Expiratory Volume (FEV1), Forced vital Capacity, Peak expiratory flow rate, Respiratory Proprioceptive Neuromuscular Facilitation Technique.

BACKGROUND: The idiopathic pulmonary fibrosis-specific version of the St George's Respiratory Questionnaire (SGRQ-I) is a valid tool to assess health-related quality of life in patients with interstitial lung diseases (ILDs). OBJECTIVE: To translate and cross-culturally adapt the SGRQ-I to Brazilian-Portuguese, and to assess its measurement properties. METHODS: Phase one consisted of the translation and cross-cultural adaptation of the questionnaire. In phase two, intra- and inter-assessor reliability (intraclass correlation coefficient [ICC]), internal consistency (Cronbach's α), minimal detectable change (MDC), ceiling/floor effects, convergent validity (correlation with SF-36 questionnaire), and discriminative validity (according to clinical characteristics) were investigated. RESULTS: No significant adaptations were needed during the translation process of the SGRQ-I. In phase two, 30 patients with ILD were included (15 men; age 59 ± 10 years; Forced Vital Capacity 73 [61–80]%predicted). The total score on the SGRQ-I presented excellent intra-assessor (ICC: 0.93; 95%CI: 0.85, 0.97]) and inter-assessor (ICC: 0.88; 95%CI: 0.77, 0.94) agreement. Internal consistency was considered adequate for the domains impact, activity, and total score (0.79

Summary Background INCREASE was a randomised, placebo-controlled, phase 3 trial that evaluated inhaled treprostinil in patients with interstitial lung disease (ILD) and associated pulmonary hypertension. Treprostinil improved exercise capacity from baseline to week 16, assessed with the use of a 6-min walk test, compared with placebo. Improvements in forced vital capacity (FVC) were also reported. The aim of this post-hoc analysis was to further characterise the effects of inhaled treprostinil on FVC in the overall study population and in various subgroups of interest. Methods In this post-hoc analysis, we evaluated FVC changes in the overall study population and in various subgroups defined by cause of disease or baseline clinical parameters. The study population included patients aged 18 years and older who had a diagnosis of ILD based on evidence of diffuse parenchymal lung disease on chest CT done within 6 months before random assignment (not centrally adjudicated). All analyses were done on the intention-to-treat population, defined as individuals who were randomly assigned and received at least one dose of study drug. The INCREASE study is registered with ClinicalTrials.gov, NCT02630316. Findings Between Feb 3, 2017, and Aug 30, 2019, 326 patients were enrolled in the INCREASE trial. Inhaled treprostinil was associated with a placebo-corrected least squares mean improvement in FVC of 28·5 mL (SE 30·1; 95% CI −30·8 to 87·7; p=0·35) at week 8 and 44·4 mL (35·4; −25·2 to 114·0; p=0·21) at week 16, with associated percentage of predicted FVC improvements of 1·8% (0·7; 0·4 to 3·2; p=0·014) and 1·8% (0·8; 0·2 to 3·4; p=0·028). Subgroup analysis of patients with idiopathic interstitial pneumonia showed FVC differences of 46·5 mL (SE 39·9; 95% CI −32·5 to 125·5; p=0·25) at week 8 and 108·2 mL (46·9; 15·3 to 201·1; p=0·023) at week 16. Analysis of patients with idiopathic pulmonary fibrosis showed FVC differences of 84·5 mL (52·7; −20·4 to 189·5; p=0·11) at week 8 and 168·5 mL (64·5; 40·1 to 297·0; p=0·011) at week 16. The most frequent adverse events included cough, headache, dyspnoea, dizziness, nausea, fatigue, and diarrhoea. Interpretation In patients with ILD and associated pulmonary hypertension, inhaled treprostinil was associated with improvements in FVC versus placebo at 16 weeks. This difference was most evident in patients with idiopathic interstitial pneumonia, particularly idiopathic pulmonary fibrosis. Inhaled treprostinil appears to be a promising therapy for idiopathic pulmonary fibrosis that warrants further investigation in a prospective, randomised, placebo-controlled study. Funding United Therapeutics Corporation.

Summary: Background: Although advanced medical imaging technologies give detailed diagnostic information, a low-dose, fast, and inexpensive option for early detection of respiratory diseases and follow-ups is still lacking. The novel method of x-ray dark-field chest imaging might fill this gap but has not yet been studied in living humans. Enabling the assessment of microstructural changes in lung parenchyma, this technique presents a more sensitive alternative to conventional chest x-rays, and yet requires only a fraction of the dose applied in CT. We studied the application of this technique to assess pulmonary emphysema in patients with chronic obstructive pulmonary disease (COPD). Methods: In this diagnostic accuracy study, we designed and built a novel dark-field chest x-ray system (Technical University of Munich, Munich, Germany)—which is also capable of simultaneously acquiring a conventional thorax radiograph (7 s, 0·035 mSv effective dose). Patients who had undergone a medically indicated chest CT were recruited from the department of Radiology and Pneumology of our site (Klinikum rechts der Isar, Technical University of Munich, Munich, Germany). Patients with pulmonary pathologies, or conditions other than COPD, that might influence lung parenchyma were excluded. For patients with different disease stages of pulmonary emphysema, x-ray dark-field images and CT images were acquired and visually assessed by five readers. Pulmonary function tests (spirometry and body plethysmography) were performed for every patient and for a subgroup of patients the measurement of diffusion capacity was performed. Individual patient datasets were statistically evaluated using correlation testing, rank-based analysis of variance, and pair-wise post-hoc comparison. Findings: Between October, 2018 and December, 2019 we enrolled 77 patients. Compared with CT-based parameters (quantitative emphysema ρ=–0·27, p=0·089 and visual emphysema ρ=–0·45, p=0·0028), the dark-field signal (ρ=0·62, p

Summary Background We previously described the contributions of increased total airway mucin concentrations to the pathogenesis and diagnosis of the chronic bronchitic component of chronic obstructive pulmonary disease (COPD). Here, we investigated the relative contribution of each of the major airway gel-forming mucins, MUC5AC and MUC5B, to the initiation, progression, and early diagnosis of airways disease in COPD. Methods SPIROMICS was a multicentre, observational study in patients aged 40–80 years recruited from six clinical sites and additional subsites in the USA. In this analysis, MUC5AC and MUC5B were quantitated by stable isotope-labelled mass spectrometry in induced sputum samples from healthy never-smokers, ever-smokers at risk for COPD, and ever-smokers with COPD. Participants were extensively characterised using results from questionnaires, such as the COPD assessment test (CAT) and St George's Respiratory Questionnaire; quantitative CT, such as residual volume/total lung capacity ratio (RV/TLC) and parametric response mapping-functional small airway disease (PRM-fSAD); and pulmonary function tests, such as FEV1, forced vital capacity (FVC), and forced expiratory flow, midexpiratory phase (FEF25–75%). Absolute concentrations of both MUC5AC and MUC5B were related to cross-sectional (baseline, initial visit) and 3-year follow-up longitudinal data, including lung function, small airways obstruction, prospective acute exacerbations, and smoking status as primary outcomes. This study is registered with ClinicalTrials.gov (NCT01969344). Findings This analysis included 331 participants (mean age 63 years [SEM 9·40]), of whom 40 were healthy never-smokers, 90 were at-risk ever-smokers, and 201 were ever-smokers with COPD. Increased MUC5AC concentrations were more reliably associated with manifestations of COPD than were MUC5B concentrations, including decreased FEV1 and FEF25–75%, and increased prospective exacerbation frequency, RV/TLC, PRM-fSAD, and COPD assessment scores. MUC5AC concentrations were more reactive to cigarette smoke exposure than were MUC5B concentrations. Longitudinal data from 3-year follow-up visits generated a multivariate-adjusted odds ratio for two or more exacerbations of 1·24 (95% CI 1·04–1·47, p=0·015) for individuals with high baseline MUC5AC concentration. Increased MUC5AC, but not MUC5B, concentration at baseline was a significant predictor of FEV1, FEV1/FVC, FEF25–75%, and CAT score decline during the 3-year follow-up. Moreover, current smokers in the at-risk group showed raised MUC5AC concentrations at initial visits and decreased lung function over 3 years. By contrast, former smokers in the at-risk group showed normal MUC5AC concentrations at the initial visit and preserved lung function over 3 years. Interpretation These data indicate that increased MUC5AC concentration in the airways might contribute to COPD initiation, progression, exacerbation risk, and overall pathogenesis. Compared with MUC5B, greater relative changes in MUC5AC concentrations were observed as a function of COPD severity, and MUC5AC concentration seems to be an objective biomarker to detect disease in at-risk and pre-COPD individuals. These data suggest that MUC5AC-producing pathways could be potential targets for future therapeutic strategies. Thus, MUC5AC could be a novel biomarker for COPD prognosis and for testing the efficacy of therapeutic agents. Funding National Institutes of Health; National Heart, Lung, and Blood Institute.

In patients with IPF, this study aimed (i) to examine the relationship between serial change in CT parameters of lung volume and lung function, (ii) to identify the prognostic value of serial change in CT parameters of lung volume, and (iii) to define a threshold for serial change in CT markers of lung volume that optimally captures disease progression. Serial CTs were analysed for progressive volume loss or fibrosis progression in 81 IPF patients (66 males, median age = 67 years) with concurrent forced vital capacity (FVC) (median follow-up 12 months, range 6–23 months). Serial CT measurements of volume loss comprised oblique fissure posterior retraction distance (OFPRD), aortosternal distance (ASD), lung height corrected for body habitus (LH), and automated CT-derived total lung volumes (ALV) (measured using commercially available software). Fibrosis progression was scored visually. Serial changes in CT markers and FVC were compared using regression analysis, and evaluated against mortality using Cox proportional hazards. There were 58 deaths (72%, median survival = 17 months). Annual % change in ALV was most significantly related to annual % change in FVC (R2 = 0.26, p

International audience; Introduction/aims: Respiratory status is a key determinant of prognosis in patients with Duchenne muscular dystrophy (DMD). We aimed to evaluate the determinants of diaphragm ultrasound and its performance in predicting restrictive respiratory patterns in DMD.Methods: This was a retrospective study of DMD patients followed in our center and admitted for an annual checkup from 2015 to 2018. We included DMD patients who underwent diaphragm ultrasound and pulmonary functional tests.Results: This study included 74 patients with DMD. The right diaphragm thickening fraction (TF) was significantly associated with age (P = .001), Walton score (P = .012), inspiratory capacity (IC) (P = .004), upright forced vital capacity (FVC) (P < .0001), supine FVC (P = .038), and maximal inspiratory pressure (MIP) (P = .002). Right diaphragm excursion was significantly associated with age (P < .0001), steroid use (P = .008), history of spinal fusion (P < .0001), body mass index (BMI) (P = .002), Walton score (P < .0001), IC (P < .0001), upright FVC (P < .0001), supine FVC (P < .0001), and MIP (P < .0001). A right diaphragm TF >28% and a right diaphragm excursion>25.4 mm were associated with an FVC >50% with, respectively, an area under the curve (AUC) of 0.95 (P = .001) and 0.93 (P < .001). A left diaphragm TF >26.8% and a left diaphragm excursion >21.5 mm were associated with an FVC >50% with, respectively, an AUC of 0.95 (P = .011) and 0.97 (P < .001).Discussion: Diaphragm excursion and diaphragm TF can predict restrictive pulmonary insufficiency in DMD.

PURPOSE Whether the modified shuttle test (MST) achieves maximal effort in children and adolescents with asthma is unclear. The aim was to compare the physiological responses of MST to the cardiopulmonary exercise test (CPET) in pediatric patients with asthma, to observe its convergent validity. PATIENTS AND METHODS A cross-sectional study with volunteers with asthma (6-17 years of age) under regular treatment. The MST is an external-paced test, and the participants were allowed to walk/run. CPET was performed on a cycle ergometer to compare with MST. Gas exchange (VO2 , VCO2 , and VE) and heart rate (HR) were the outcomes and were continuously assessed in both tests. RESULTS Forty-seven volunteers were included, normal lung function expiratory forced volume at 1st second/forced vital capacity (FEV1 /FVC) 88.6 (7.7). VO2peak was higher at MST (2.0 ± 0.6 L/min) compared to CPET (1.6 ± 0.5 L/min), p

Introduction. Possible causes of chronic bronchitis are anatomically incorrect location of internal organs, the presence of congestion, impaired blood circulation, impaired mobility level, disorders of innervation and metabolic processes in the respiratory organs, decreased mobility of the chest, tension of the thoracic diaphragm, imbalance of the myofascial system, diseases of the spine (osteochondrosis, scoliosis, etc.). These disorders and dysfunctions are extremely rarely taken into account in the prescription of conventional therapy regimens in children with chronic bronchitis. At the same time, it is known that many drugs and various therapeutic effects in some cases are not able to give long-term remission and recovery. This causes the need to search for new methods of treating chronic bronchitis in children, one of which could be osteopathic correction. At the same time, in the available literature sources, there is very little information on this problem, and the presented data are sometimes contradictory.The aim of the study is to evaluate the clinical effectiveness of osteopathic correction in the treatment of children with chronic bronchitis in a sanatorium.Materials and methods. The controlled randomized trial was conducted from June 2020 to April 2021. The study included 40 patients with chronic bronchitis at the age from 6 to 16 years, 24 boys, 16 girls, who were undergoing sanatorium-resort treatment in the children′s sanatorium «Raduga» (Sterlitamak). Depending on the applied treatment method, the patients were divided by simple randomization into two comparable groups (main and control) of 20 people each. All patients received sanatorium resort treatment, which included physical therapy, halotherapy, inhalations, phytotherapeutic procedures. Patients of the main group, in addition to the above-described therapy, received osteopathic correction. In total, 2 sessions were carried out with an interval of 7 days. All patients, regardless of the group, before the start of treatment, as well as after the completion of the therapy course, underwent an assessment of the osteopathic status, chest excursion and respiratory function.Results. During the treatment with inclusion of osteopathic correction, in patients of the main group, there is a significant decrease in the frequency of detection of somatic dysfunctions (global rhythmogenic disorder, neck region, chest region), as well as a significant positive dynamics of indicators characterizing the external respiration function (chest excursion, forced vital capacity of lungs, peak exhalation rate), compared with patients receiving only conventional therapy. Positive changes in the chest excursion and peak exhalation rate against the background of osteopathic correction persist reliably even 3 months after the completion of the therapy course, and it allows to suppose the prolonged effect of the therapy.Conclusion. The study showed that osteopathic correction increases the clinical effectiveness of the conventional methods of sanatorium resort treatment for pediatric patients with chronic bronchitis. This allows to recommend the inclusion of osteopathic methods of correction in complex treatment programs for this group of patients in a sanatorium.

BACKGROUND Ultrasonography (USG) of the diaphragm is a promising alternative to pulmonary function tests (PFT) for assessing respiratory function in amyotrophic lateral sclerosis/motor neuron disease (ALS/MND). METHODS We studied 33 patients fulfilling Awaji criteria (definite = 14; probable = 12; possible = 7) and 33 age and gender-matched controls. Diaphragm thickness was measured using USG at the end of expiration (DTex) and end of inspiration (DTin). The thickness ratio (TR) was calculated as DTin/DTex. The mean age at onset and duration were 49.73 ± 12.7 years and 13.57 ± 9.7 months, respectively. Men = 25 (75.8%); Limb onset ALS/MND = 24 patients (72.7%); bulbar onset = 9 (27.3%). RESULTS Compared to controls, ALS/MND patients had reduced mean DTex (2.22 ± 0.29 mm vs. 2.02 ± 0.32 mm, p = .012) and DTin (4.0 ± 0.71 mm vs. 3.41 ± 0.38 mm, p

OBJECTIVES In epidemiological studies, childhood asthma is usually assessed with questionnaires directed at parents or children, and these may give different answers. We studied how well parents and children agreed when asked to report symptoms of wheeze and investigated whose answers were closer to measurable traits of asthma. METHODS LuftiBus in the school is a cross-sectional survey of respiratory health among Swiss schoolchildren aged 6-17 years. We applied questionnaires to parents and children asking about wheeze and exertional wheeze in the past year. We assessed agreement between parent-child answers with Cohen's kappa (k), and associations of answers from children and parents with fractional exhaled nitric oxide (FeNO) and forced expiratory volume in 1 s over forced vital capacity (FEV1 /FVC), using quantile regression. RESULTS We received questionnaires from 3079 children and their parents. Agreement was poor for reported wheeze (k = 0.37) and exertional wheeze (k = 0.36). Median FeNO varied when wheeze was reported by children (19 ppb, interquartile range [IQR]: 9-44), parents (22 ppb, IQR: 12-46), both (31 ppb, IQR: 16-55), or neither (11 ppb, IQR: 7-19). Median absolute FEV1 /FVC was the same when wheeze was reported by children (84%, IQR: 78-89) and by parents (84%, IQR: 78-89), lower when reported by both (82%, IQR: 78-87), and higher when reported by neither (87%, IQR: 82-91). For exertional wheeze findings were similar. Results did not differ by age or sex. CONCLUSION Our findings suggest that surveying both parents and children and combining their responses can help us to better identify children with measurable asthma traits.

STUDY OBJECTIVES: Duchenne muscular dystrophy (DMD) is a neuromuscular disorder that leads to chronic respiratory insufficiency and failure. Use of home noninvasive ventilation (NIV) has been linked to improved outcomes including reduced mortality. Despite the importance of NIV, factors promoting optimal NIV usage and determinants of nonadherence have not been rigorously examined. Moreover, given that respiratory issues in DMD span between childhood and adulthood, examination across a broad age group is needed. The objectives of this study were to (1) evaluate NIV usage across a broad spectrum of patients with DMD, including both children and adults, and (2) identify biological and socioeconomic determinants of NIV usage and NIV nonadherence. METHODS: We performed a retrospective review of all patients with DMD from February 2016 to February 2020 who underwent evaluation at associated pediatric and adult neuromuscular disease clinics. NIV use was determined objectively from device downloads. A priori, we defined nonadherence as < 4 hours use per night, quantified as the percentage of nights below this threshold across a 30-day period within 6 months of a clinic visit. We also assessed the average hours of NIV usage over this time period. Predictors examined included demographics, social determinants, and pulmonary function. RESULTS: 33 patients with DMD were identified, 29 (87%) of whom were using NIV (13 age < 21 years). Mean age was 22.9 ± 6.6 years (range 13–39 years), body mass index was 23.4 ± 10.4 kg/m(2), and seated forced vital capacity was 23% ± 18% predicted. Mean nightly NIV usage was 7.4 ± 3.8 hours and mean percentage of nonadherent nights was 13% ± 30%. In univariable analysis, age did not predict use. Those with lower forced vital capacity had higher NIV usage hours (P = .01) and a trend toward less nonadherence (P = .06). Higher estimated household income demonstrated a trend toward increased usage hours and less nonadherence (both P = .08). Multivariable analysis found increased usage hours were predicted best by higher income, higher inspiratory positive airway pressure, and higher bicarbonate. Nonadherence was higher in those with lower income or higher forced vital capacity. CONCLUSIONS: In this cohort of adult and pediatric patients with DMD, most individuals were using NIV. While usage hours were higher with lower lung function, substantial variability remains unexplained by examined factors. Nonadherence was observed in some individuals, including those with advanced disease. Further investigations should focus on evaluating patient-oriented outcomes to define optimal NIV usage across the spectrum of disease and determine strategies to counteract issues with nonadherence. CITATION: Hurvitz MS, Bhattacharjee R, Lesser DJ, Skalsky AJ, Orr JE. Determinants of usage and nonadherence to noninvasive ventilation in children and adults with Duchenne muscular dystrophy. J Clin Sleep Med. 2021;17(10):1973–1980.

Case Presentation A 58-year-old woman presented to a pulmonology clinic for evaluation of bilateral pulmonary nodules. Two years previously, she had presented with atrioventricular nodal reentrant tachycardia. During evaluation for her tachyarrhythmia, transthoracic echocardiogram (TTE) revealed a large, homogenous, highly mobile right atrial and ventricular mass. She underwent electrophysiologic ablation, tricuspid valve annular ring replacement, and resection of the mass, which pathology confirmed to be a myxoma. Now, a recent abdomen and pelvis CT study obtained for history of nephrolithiasis incidentally noted bilateral lower lobe pulmonary nodules. Follow-up noncontrast chest CT confirmed bilateral peribronchovascular solid pulmonary nodules up to 8 mm in diameter throughout all lobes. The nodules appeared contiguous with the segmental and subsegmental bronchovascular bundles, and many occurred at branch points. There was no mediastinal or hilar lymphadenopathy. To evaluate the pulmonary nodules, she was referred to a pulmonology clinic. She reported only stable, nonlimiting dyspnea on exertion. She did not have a cough. She denied a history of fevers, weight loss, or night sweats. She had no rash or skin changes, visual changes, joint pain or swelling, or palpitations. She had no history of oropharyngeal or genital ulcerations. Social history was notable for a 40-pack-year smoking history, with quit date 2 years prior. She had no risk factors for TB exposure and no exposures to sandblasting, stone cutting, or other environmental risk factors for silicosis. Family history was negative for autoimmune conditions, sarcoidosis, and lymphoproliferative disorders.

Background Acute exacerbations (AEs) and disease progression in interstitial lung disease (ILD) pose important challenges to clinicians and patients. AEs of ILD are variable in presentation but may result in rapid progression of ILD, respiratory failure and death. However, in many cases AEs of ILD may go unrecognised so that their true impact and response to therapy is unknown. The potential for home monitoring to facilitate early, and accurate, identification of AE and/or ILD progression has gained interest. With increasing evidence available, there is a need for a systematic review on home monitoring of patients with ILD to summarise the existing data. The aim of this review was to systematically evaluate the evidence for use of home monitoring for early detection of exacerbations and/or progression of ILD. Method We searched Ovid-EMBASE, MEDLINE and CINAHL using Medical Subject Headings (MeSH) terms in accordance with the PRISMA guidelines (PROSPERO registration number CRD42020215166). Results 13 studies involving 968 patients have demonstrated that home monitoring is feasible and of potential benefit in patients with ILD. Nine studies reported that mean adherence to home monitoring was >75%, and where spirometry was performed there was a significant correlation (r=0.72–0.98, p, First systematic review that provides supportive evidence for the feasibility and utility of home monitoring in ILD; further studies are necessary to evaluate approaches to detect exacerbation and/or progression https://bit.ly/2Y8OCJL

[Purpose] We aimed to analyze parameters of pulmonary function and physiological, psychological, and physical factors in patients with chronic obstructive pulmonary disease (COPD) receiving pulmonary rehabilitation (PR) and music therapy (MT). [Participants and Methods] This randomized crossover comparative study included in-patients diagnosed with COPD and a ratio of forced expiratory volume measured at the first second and forced vital capacity (FEV1/FVC) of

Background Interstitial lung disease (ILD) is a common and potentially life-threatening complication for rheumatoid arthritis (RA) patients. However, there is a lack of clear prognostic factors in rheumatoid arthritis-associated interstitial lung disease (RA-ILD) patients. The purpose of this study was to complete a systematic review and meta-analysis of the factors associated with mortality in RA-ILD patients. Methods Medline, EMBASE and the Cochrane Library were searched up to September 1, 2020. The Newcastle–Ottawa Scale (NOS) was applied to assess the methodological quality of the eligible studies. Study characteristics and magnitude of effect sizes were extracted. Then, pooled hazard ratios (HRs) with the corresponding 95% confidence intervals (CIs) and pooled risk ratios (RRs) with 95% CIs were calculated to assess the factors associated with mortality in RA-ILD. Results Twenty-three of 3463 articles were eligible, and ten factors associated with mortality for RA-ILD were evaluated in the meta-analysis. Older age (HRs = 1.04, 95% CI 1.03–1.05), male sex (HRs = 1.44, 95% CI 1.21–1.73), having a smoking history (HRs = 1.42, 95% CI 1.03–1.96), lower diffusing capacity of the lung for carbon monoxide (DLCO)% predicted (HRs = 0.98, 95% CI 0.97–1.00), forced vital capacity (FVC)% predicted (HRs = 0.99, 95% CI 0.98–1.00), composite physiological index (CPI) (HRs = 1.04, 95% CI 1.02–1.06), usual interstitial pneumonia (UIP) pattern on HRCT (HRs = 1.88, 95% CI 1.14–3.10 and RRs = 1.90, 95% CI 1.50–2.39), emphysema presence (HRs = 2.31, 95% CI 1.58–3.39), and acute exacerbation of ILD (HRs = 2.70, 95% CI 1.67–4.36) were associated with increased mortality in RA-ILD, whereas rheumatoid factor (RF) positive status was not associated. Conclusions Through this systematic review and meta-analysis, we found that older age, male sex, smoking history, higher CPI, lower DLCO% predicted, lower FVC% predicted, UIP pattern on HRCT, emphysema presence and acute exacerbation of ILD were associated with an increased risk of mortality in RA-ILD.

Objectives Insulin resistance may be associated with impaired lung function. The objective of this study was to examine the relationship between insulin resistance and lung function in asthmatic children. Methods We performed a cross-sectional study on asthmatic children aged 6–18 years at Tertiary Care Center, India. Fasting serum glucose and insulin levels were analyzed to calculate the homeostatic model assessment of insulin resistance (HOMA-IR) in 90 children. Lung function tests were performed. Spearman correlation was used to assess the relationship between insulin resistance and lung function. Linear regression was done to adjust the potential confounders. Results Insulin resistance was present in 15.5% of patients. We found a statistically significant inverse relationship between HOMA-IR and spirometric parameters such as forced expiratory volume in 1 s/forced vital capacity ratio and forced expiratory flow between 25 and 75% on Spearman correlation. However, adjusted regression for age, gender, and body mass index showed no significant association of lung function parameters with HOMA-IR. Conclusions Insulin resistance may be considered for the decline in lung function in asthmatic children.

OBJECTIVES The aim of this study was to identify risk factors associated with mortality in patients with systemic sclerosis (SSc), particularly those with interstitial lung disease (ILD), over a long-term follow-up in a large Brazilian SSc cohort. METHODS We conducted a medical records review study of 380 scleroderma patients from 1982 to 2019. Systemic sclerosis ILD was considered in those with evidence of ILD on chest high-resolution computed tomography (HRCT). Causes of death were determined. RESULTS Among the 380 SSc patients, SSc-ILD on chest HRCT was observed in 227 patients (59.7%). Seventy-two patients (18.9%) died during a mean follow-up of 7.2 years since the SSc diagnosis; among them, 57 (79.2%) had SSc-ILD, compared with 15 (20.8%) without SSc-ILD (p < 0.001). Of the 72 deaths, 51.4% were considered related to SSc, and ILD was the leading cause of death. The overall survival rates at 5, 10, and 15 years were 87.9%, 81.5%, and 74.9%, respectively. Kaplan-Meier analysis showed a significantly worse prognosis among patients with SSc-ILD than among those without ILD (p < 0.001). Among patients with SSc-ILD, disease duration of less than 4 years (p < 0.001), forced vital capacity

Aim To determine the diagnostic accuracy of pulmonary artery to aorta ratio in screening for pulmonary hyperten - sion in advanced chronic obstructive pulmonary disease (COPD) patients. Methods A prospective, diagnostic study was conducted in University Hospital Center Zagreb between January 2015 and March 2018. The study enrolled 100 patients who con - secutively underwent chest computed tomography (CT), echocardiographic exam, and right heart catheterization. Two independent observers measured pulmonary artery and ascending aorta diameters. The correlation between the ratio and mean pulmonary artery pressure, measured invasively, was assessed. Patients with echocardiographic signs of moderate systolic or diastolic left ventricular dys - function were excluded (n =44). Results Sixty-six patients (55.5% men), with a median age of 61, were identified. Median forced expiratory vol - ume during the first second (FEV1) was 34 ±12, FEV1/ forced vital capacity

Dysphagia and progressive swallowing problems due to motoneuron death is one of amyotrophic lateral sclerosis (ALS) symptoms. Malnutrition and body weight loss result in immunological disturbances, fatigability and increase risk of secondary complications in ALS patients, percutaneous endoscopic gastrostomy tube (PEG) placement representing a well-recognized method for malnutrition correction and potentially increasing life expectancy. However, despite nutritional correction, occasional rapid neurological deterioration may develop after PEG placement. We have hypothesized that this decline can be a result of exteroceptive stress during PEG placement and promote neurodegeneration in ALS patients. Intravenous sedation may decrease stress during invasive procedures and it is safe during PEG placement in ALS patients. The aim of the study was comparing different PEG placement protocols of anesthesia (local anesthesia or local anesthesia plus intravenous sedation) in ALS from perspectives of stress load and neurological deterioration profile. During 1.5 years 94 ALS patients were admitted; gastrostomy was performed in 79 patients. After screening according to inclusion and exclusion criteria, 30 patients were included in the prospective consecutive study. All patients were divided in two groups, with local anesthesia and with combination of local anesthesia and intravenous sedation. Routine biochemical indices, neurodegeneration and stress markers were measured. The age of ALS patients was 61 ± 10 years; 20 patients were included at stage 4A and 10 at stage 4B (King’s College staging). PEG was placed at average14 months after the diagnosis and 2.2 years after first symptoms. Mean ALS Functional Rating Scale-Revised was 27.8, mean forced vital capacity of lung 46.3% (19—91%). After one year of observation only 8 patients survived. Mean life duration after PEG was 5 months (5 days—20 months). Comparison of two PEG placement protocols did not reveal differences in survival time, stress load and inflammation level. Higher saliva cortisol levels, serum cortisol, glucose, C-reactive protein and interleukin-6 were detected after PEG placement, confirming considerable stress response. PEG is a stressful factor for ALS patients, PEG placement representing a natural model of exteroceptive stress. Stress response was detected as increased cortisol, C-reactive protein, interleukin-6, and glucose levels. Intravenous sedation did not increase the risk of PEG placement procedure, however, sedation protocol did not affect stress load.

Background . Bronchial asthma (BA) already in childhood can be accompanied by cardiovascular complications in the form of acute pulmonary heart disease, ventricular dysfunction, metabolic cardiomyopathy, rhythm and conduction disorders, which not only aggravates the course of the disease, but also reduces the quality of life of patients. The heart remodeling in BA is preceded by clinical manifestations of heart failure and can independently aggravate systolic and diastolic dysfunction of the ventricles. The purpose of the study was to improve the prediction of cardiovascular complications in the period of bronchial asthma exacerbation in children by identifying the risk factors for ventricular dysfunction and developing additional informative clinical and echocardiographic diagnostic criteria for right ventricular heart failure. Materials and methods. Under our supervision, there were 85 children with asthma aged 5 to 17 years. Pulse oximetry, spirography, electrocardiography, Doppler, and echocardiography were performed. The examination was carried out in the period of asthma exacerbation, during hospitalization of the patient and during the period of improvement, on the days 5–6 of staying in the pulmonary department of the Municipal Institution “Regional Children’s Clinical Hospital” of Dnipropetrovsk Regional Council. The criteria for inclusion in the study were allergic and mixed persistent, moderate and severe asthma during the period of exacerbation. The study did not include patients with asthma in remission period, children with an intermittent course of asthma and a mild persistent asthma. To determine the type and degree of ventilation insufficiency, a spirographic method was used to evaluate the indicators of vital lung capacity (VLC), forced expiratory volume in 1 second (FEV1), FEV1/VLC. The mathematical and statistical processing of the research materials was carried out using the methods of biostatistics implemented in the packages of Microsoft Excel programs (Office Home Business 2KB4Y-6H9DB-BM47K-749PV-PG3KT), Statistica 6.1 (StatSoft Inc., Serial number AGAR909E415822FA). Results. The prognostic clinical risk factors for the development of systolic and diastolic dysfunction of the cardiac ventricles in case of a possible attack of bronchial asthma were identified: the presence of a severe bronchial asthma in a sick child; presence of tachycardia at rest; violation of the ventricular myocardial repolarization on the electrocardiogram; presence of concomitant chronic tonsillitis; bacteria in the nasopharynx; female sex. Additional informative diagnostic criteria for the development of right ventricular heart failure during the period of exacerbation of bronchial asthma in children have been developed. It has been found that the use of euphylline in combination with b 2 -agonists for the bronchodilator therapy of children in the period of exacerbation of bronchial asthma negatively affects the diastolic function of the cardiac ventricles and promotes the preservation of elevated pressure in the pulmonary artery. Conclusions. The issue of improving the prediction of cardiac ventricular dysfunction and timely diagnosis of cardiovascular complications in the period of asthma exacerbation in children is solved by identifying clinical risk factors for ventricular dysfunction and developing additional informative, clinical and echocardiographic diagnostic criteria of right ventricular heart failure.

Background: Asthma is an inflammatory disorder, usually chronic, and is related to the hypersensitivity of the airways to allergens leading to dyspnoea, wheezing, cough and tightness in chest. Increased severity of asthma and poor control has been attributed to lower levels of Vitamin D levels. Materials and Methods: Blood was collected from 78 patients who were diagnosed with asthma and tested for Vitamin D levels eosinophil counts, IgE levels, and regular biochemical tests. All these patients underwent Asthma Control Test, Pulmonary Function tests for FEV1%, FVC%, FEV1/FVC%. Results : The serum eosinophil count, sputum eosinophil count and IgE levels were significantly higher in Vitamin D deficient patients compared to the sufficient patients. The most common severity of asthma among the patients was due to atopy as seen in 53.8% of the patients. In 39.6% of the patients who were Vit D deficient, the asthma was very severe as was the case in 8% of the Vit D sufficient people. The Forced expiratory volume, Forced vital capacity and FEV1/FVC1 was 92.69 ± 0.9 %, 91.3 ± 12.1% and 76.2 ± 8.7 in patients with deficient Vitamin D levels and 97.36 ± 1.1%, 99.1 ± 7.3% and 81.6 ± 13.1 in patients with sufficient Vit D levels respectively. Conclusions: There seems to be a strong association of Vitamin D levels and asthma among patients. High vitamin D levels are beneficial to the lung function and also slow the asthma exacerbations. Keywords: Vitamin D deficiency, Asthma, Exacerbations

In amyotrophic lateral sclerosis (ALS) lower plasma creatinine level has been associated with shorter survival and faster functional decline. It has not been clear if creatinine is associated with respiratory outcome. We analyzed retrospectively a population of unselected ALS patients. Multiple-regression and Cox-regression analyses were performed. We included 233 patients, mean age 62.8, mean disease duration of 18.6 months. At baseline, creatinine was significantly associated with ALSFRS-R, but not with its decline rate. No predictive value was disclosed for FVC, its decline rate, or with survival. We did not confirm that creatinine is a marker of respiratory outcome.

Objective: This study aims to evaluate respiratory functions in patients Coronavirus disease-2019 (COVID-19) with and without pneumonia. Material and Methods: This single-center, prospective study included a total of 72 patients who were diagnosed with COVID-19 infection as confirmed by real-time reverse transcriptase polymerase chain reaction (rtPCR). The patients were divided into two groups according to the physical and thoracic computed tomography (CT) findings as mild symptomatic patients without COVID-19 pneumonia (n=26) and symptomatic cases with COVID-19 pneumonia (n=46). Respiratory functions were evaluated by spirometry in the second and fourth months of the disease onset. Results: The average age of 72 patients, 41 of whom were men, was 40.5±12.27 years. Thoracic CT revealed infiltrations compatible with COVID-19 pneumonia in 46 (63.9%) patients. Hypertension (12.5%) and diabetes (5.6%) were the most common comorbidities. When the results of the patients with and without pneumonia at the second and fourth months were compared, there was no significant difference between the forced expiratory volume in the first second (FEV1) (p1=0,975, p2=0,291), forced vital capacity (FVC) (p1=0,668,p2=0,481) and FEV1/FVC ratio (FER) (p1=0,378, p2=0,980) values. When the repeated anova test was used in the comparison of the two visit differences between the groups, it was seen that there was no difference in any heading (FVC: p=0.077; FEV1: p=0.150; FER: p=0.355). Conclusions: Our study results show no significant difference in the pulmonary function tests of patients with mild and moderate COVID-19 pneumonia at two and four months, compared to those without pneumonia However, additional studies are needed for severe and critical cases.

Background. New opportunities for improving the respiratory function are associated with the use of high-frequency chest wall oscillation, which allows us to restore the drainage function of the bronchial tree and optimize pulmonary ventilation. The purpose was to study and estimate the respiratory function in children with community-acquired pneumonia. Materials and methods. The study involved 107 children (the main group — 55 persons and the control group — 52 persons) aged 6–17 years with acute and uncomplicated community-acquired pneumonia. Children of the main group received basic therapy with high-frequency chest wall oscillation, while patients of the control group received only basic therapy. The evaluation of respiratory function was performed in the dynamics of treatment using spirometry. Results. At the beginning of therapy for community-acquired pneumonia, the respiratory function indices in the studied groups had insignificant differences. Consequently, analysis of respiratory function parameters in children of the main group on the 10th day of basic therapy has shown the improvement of forced expiratory vo­lume in 1 second (FEV1) (88.36 ± 1.55 %, p = 0.02), vital capacity (VC) (88.18 ± 1.53 %, p = 0.02), forced vital capacity (FVC) (86.77 ± 1.37 %, p = 0.03), maximal expiratory flow at 25 % of FVC (MEF25) (90.10 ± 2.99 %, p = 0.02) and maximal voluntary ventilation (MVV) (88.31 ± 1.70 %, p = 0.04). Moreover, when comparing indicators of the respiratory function in the control group children, a less pronounced dynamics was noted, in particular FEV1 (81.65 ± 2.44 %, p = 0.02), VC (82.95 ± 2.56, p = 0.02), FVC (80.85 ± 2.09, p = 0.03), MEF25 (82.63 ± 3.08 %, p = 0.02) and also MVV (85.65 ± 1.99 %, p = 0.04). As a result of the receiver operating characteristic (ROC) analysis, the dynamics of the respiratory function restoration in the main group children is more significant due to the improvement in MVV, as evidenced by the large area under the ROC curve (AUC) — 0.99, FEV1 — AUC 0.94 and FEV1/FVC — AUC 0.94. In control group children, the dynamics of the studied parameters of the respiratory function was less pronounced, which is confirmed by the MVV — AUC 0.63, FEV1 — AUC 0.79 and FEV1/FVC — AUC 0.89. Conclusions. To improve the ventilation function of the lungs in children with community-acquired pneumonia, it is necessary to carry out bronchial drainage procedures using high-frequency chest wall oscillation as a part of basic therapy, as evidenced by the positive results obtained from spirometry.

Background The global COVID-19 pandemic was particularly concerning for the pediatric PH population due to immature immune systems and developmental comorbidities. This study aims to describe a single-center experience of pediatric PH patients diagnosed with COVID-19 disease. Methods Retrospective cohort study of all pediatric patients followed by the PH Center at Texas Children's Hospital diagnosed with COVID-19 infection from 04/2020 through 02/2021. Results We identified 23 patients with a median age of 58 months (IQR 25-75th, 21-132 months), 48% being Hispanics. Eight patients (35%) required hospitalization; median length of stay 6 days (IQR 25-75th , 5-8 days). Only three of these 8 patients required increased respiratory support. Targeted PH therapy was escalated in four patients (two in dual and two in triple therapy). There was one mortality in a patient with failing Fontan physiology. Ninety-one percent of patients have had post-COVID outpatient follow-up, median of 101 days (IQR 25-75th , 50-159 days) from diagnosis. Of the five patients with 6 minute walk test (6MWT) data, three (60%) children walked less distance, median -12 meters (IQR 25-75th , -12 - +49 meters) compared to pre-COVID testing. Post-infection pulmonary function testing (PFT) was notable for decrease in predicted forced vital capacity (FVC) (median -6%, range -11%-+6%) and forced expiratory volume in one second (FEV1) (median -14%, range -12- -18%) in 75% of the patients with PFT data. Conclusions In our institution, COVID-19 was found more frequently in Hispanics, and associated with low mortality. This article is protected by copyright. All rights reserved.

BACKGROUND Atrial fibrillation (AF) is common in chronic obstructive pulmonary disease (COPD) patients. It is known that impaired forced expiratory volume in 1 s (FEV1) is one of the risk factors of cardiovascular disease. Two-dimensional speckle tracking echocardiography (2D-STE) can detect deterioration of left atrial mechanical functions in the subclinical stage. We hypothesized that reduced lung functions, measured by FEV1 in COPD patients, may be associated with impaired left atrial (LA) mechanical functions. OBJECTIVES Present study included 127 consecutive COPD patients. We divided study population into two groups: patients with normal LA strain (n = 20) or with impaired LA strain (n = 107). RESULTS In univariate logistic regression analysis, age (p: 0.001), FEV1% (p

Objectives This study aimed to quantify nailfold capillary (NFC) abnormalities in anti–melanoma differentiation–associated gene 5 (MDA5) -positive DM patients and to evaluate the association with clinical parameters, including serum biomarkers. In addition, we aimed to clarify the period leading to remission of NFC abnormalities during immunosuppressive treatment in patients with DM. Methods A prospective observational study was conducted including patients (n = 10) who first visited Hiroshima University Hospital and were diagnosed with DM or clinically amyopathic DM with anti-MDA5 antibodies. We compared the NFC abnormalities detected by nailfold-video capillaroscopy (NVC), physical findings, blood tests, respiratory function tests, and vascular-related growth factors measured using a LEGENDplexTM Multi-Analyte Flow Assay Kit. Results NFC abnormalities improved in all patients from 2 to 17 weeks after the initiation of immunosuppressive treatment. The NVC scores were inversely correlated with anti-MDA5 antibody titres at baseline. NVC scores and forced vital capacity were positively correlated. Baseline values of M-CSF and stem cell factor were correlated with anti-MDA-5 titres. Conclusion Our study suggested that NVC scores and disease activity were inversely correlated before treatment. Vascular-related growth factors, such as M-CSF and stem cell factor, may be associated with the disease mechanism in patients with anti-MDA5 antibody-positive DM.