Mean FEV1 Research Articles

Outcomes of Modulator Therapy Discontinued After Short-Term Use in Adult Cystic Fibrosis.

Cystic fibrosis transmembrane conductance regulator modulator therapies (CFTR-MT) have altered management, reducing exacerbations and slowing pulmonary function decline. Nevertheless, it is still uncertain if the benefits of CFTR-MTs last when they are stopped. This study aimed to assess pulmonary function changes, and exacerbation rates during and after CFTR-MT use in adult cystic fibrosis patients. Between 2018 and 2022, we conducted a study involving adult CF patients who initially used CFTR-MTs but later discontinued them due to reimbursement issues. The study was divided into three phases: predrug (T1), in-drug (T2), and postdrug (T3). We recorded pulmonary function tests, laboratory and culture results, and the number of exacerbations. The study involved 33 patients, with 28 (84.8%) receiving Elexacaftor/Tezacaftor/Ivacaftor and 5 (15.2%) receiving Ivacaftor. The median treatment and interruption durations were 3.1 (IQR = 2.9-5.7), and 2.5 (IQR = 1.5-4.0) months, respectively. The mean FEV1% was 54.3% (± 26.6), 70.4% (± 27.4), and 60.2% (± 26.5) during T1, T2, and T3, respectively (p < 0.001). The mean FVC% was 65.5% (± 23.9) in T1, increased to 81.5% (± 24.5) in T2, and decreased to 71.6% (± 25.9) in T3 (p < 0.001). The number of Psedomonas aeruginosa, and Aspergillus positive sputum cultures decreased significantly with drug use (T1: 72.7%, 39.4%; T2: 48.5%, 9.1%; T3: 45.5%, 18.2%; p = 0.014, p = 0.004, respectively). The median number of hospitalizations was 1.0 (0-5.0) in T1, 0 (0-0) in T2, and 0 (0-1.0) in T3. This study revealed that CFTR-MTs are effective even in the short term for adult CF patients, but their beneficial effects quickly diminish after discontinuation. Real-life data obtained as a result of discontinuation of drugs due to reimbursement problems has highlighted the significance of regular and uninterrupted use of modulators.

Real-world impact of Elexacaftor-Tezacaftor-Ivacaftor treatment in young people with Cystic Fibrosis: A longitudinal study

BackgroundElexacaftor, Tezacaftor, Ivacaftor (ETI) became available in the UK in August 2020 to treat people with Cystic Fibrosis (CF) aged > 12 years. We report a real-world study of clinical outcomes in young people treated with ETI at our CF centre within the first two years of its availability. MethodsParticipants aged 12-17 were identified within our clinic, with demographic data supplemented by the UK CF registry. Comprehensive outcome data spanning two years pre- and two years post-initiation of CFTR modulators were compiled from various local sources, including patient records, medication delivery logs, and clinical notes. ResultsOf the 62 patients started on ETI (32 male, mean age 13.3 years), most (76%) were homozygous for the F508del mutation. Three discontinuations occurred: one pregnancy, two related to side effects. Adherence was high (Proportion of Days covered >90% both years). Following ETI initiation there was a significant increase in mean FEV1% (+11.7 units; 95% CI 7.4 – 15.6), sustained throughout the two-year treatment period. There was no association between baseline lung function and the degree of improvement or rate of decline post-treatment. Improvements were similar for all treatable genotypes. BMI z-score increased by 0.25 units after four months of treatment, returning to baseline by 24 months. Intravenous antibiotic use decreased by 88% (median IV days/year reduced from 32 to 4 days, p < 0.01). ConclusionsETI use in adolescents in a real-world setting led to sustained improvements in health outcomes, consistent with those seen in open trial extension studies

Comparison of the Determinants of the "Chronic Obstructive Pulmonary Disease Assessment Test" (CAT) and the "Asthma Control Test" (ACT) in Patients with Asthma-COPD Overlap.

Objective: The objective of this study was to investigate which of two short questionnaires, the Asthma Control Test (ACT) or the COPD Assessment Test (CAT), correlates better with severity variables and whether they share similar determinants in patients with asthma-COPD overlap. Method: This observational, cross-sectional, multicentric study included smokers and former smokers of more than 10 pack-years, with non-fully reversible airflow obstruction and either a concomitant diagnosis of asthma or signs of type 2 inflammation, from 15 centres in Spain. Results: A total of 157 patients were included, 109 (69.4%) were men, the mean age was 63.3 (SD: 9) years and the mean FEV1 (%) was 59.7% (SD: 20.5%). The mean CAT score was 14.5 (SD: 8.7), and the mean ACT score was 17.9 (SD: 5.2). Both scores showed good correlations (r = 0.717; p < 0.001). In the multivariate analysis, the Hospital Anxiety and Depression Scale and mMRC dyspnoea scores were independently and significantly associated with both the CAT and ACT scores; however, age was only significantly associated with the CAT, and the EQ-5D scores and the number of exacerbations in the previous year were only significantly associated with the ACT scores. The ACT had a slightly better predictive value for exacerbations than the CAT (AUC = 0.70 (95% CI: 0.62 to 0.79 vs. 0.65 (95% CI: 0.56 to 0.74))). Conclusions: There is a good correlation between ACT and CAT scores in patients with ACO. However, severe patients scored worse on the CAT than the ACT. Anxiety, depression and dyspnoea were significantly associated with both the CAT and ACT scores. The ACT was a slightly better predictor of exacerbations than the CAT in this population.

Clinical Remission Predictors in Non-Colonized Bronchiectasis and Severe Asthma with Type 2-Targeted Biologic Therapy: A Retrospective Real-Life Pilot Study.

Background/Objective: Patients with severe asthma (SA) and non-cystic fibrosis bronchiectasis (BE) without microbiological colonization represent a unique and understudied population. Type 2-targeted biologic therapies have emerged as a promising treatment for these patients. However, predictive factors for achieving clinical remission remain unclear. This study aims to identify the predictive factors for achieving clinical remission in patients with severe asthma and non-colonized bronchiectasis undergoing type 2-targeted biologic therapies. Methods: A retrospective longitudinal analysis was conducted on 14 patients with severe asthma and non-cystic fibrosis bronchiectasis without microbiological colonization. Clinical remission was assessed at baseline (T0) and after 12 months (T1) of biologic therapy. Clinical remission was defined according to the Severe Asthma Network Italy (SANI) criteria, including the absence of oral corticosteroid use, no asthma-related symptoms, stable lung function, and no exacerbations. Logistic regression was performed to identify predictors of remission. ROC curves were constructed to evaluate the predictive accuracy of lung function parameters, specifically FEV1 and FVC. Results: After 12 months of biologic therapy, 28.6% of patients (n = 4) achieved clinical remission. The mean FEV1 percentage at baseline was significantly higher in the remission group (92.25 ± 15.64%) compared to the non-remission group (65.10 ± 23.36%, p = 0.034). Logistic regression analysis identified baseline FEV1 as a significant predictor of remission (OR = 1.008, p = 0.050). ROC curve analysis revealed that an FEV1 cutoff of 72.5% had a sensitivity of 100% and a specificity of 70% (AUC = 0.900, p = 0.024) for predicting clinical remission. Conclusions: FEV1 is a crucial predictor of clinical remission in patients with severe asthma and non-colonized bronchiectasis treated with type 2-targeted biologic therapies. An FEV1 threshold of 72.5% can guide clinicians in identifying patients most likely to achieve remission. These findings underline the importance of preserving lung function to optimize therapeutic outcomes in this complex population.

Inhaler personalisation based on peak inspiratory flow (PIF) among dry powder inhaler users: a pilot randomised control trial (RCT) in COPD.

Dry powder inhalers (DPIs) are commonly used among patients with Chronic Obstructive Pulmonary Disease (COPD). These inhalers are breath-actuated, and require patients to generate sufficient peak inspiratory flow (PIF) to disaggregate the drug powder into respirable fine particles and deliver it to the lower airway tracts. Inhaler personalisation based on PIF among DPI users has not been studied in Malaysia, thus we conducted the present pilot study to determine the feasibility of conducting such research among COPD patients. This was an open-label pilot randomised control trial, conducted from June 2021-January 2022 at the respiratory clinic of Hospital Canselor Tuanku Muhriz, Universiti Kebangsaan Malaysia. Measurement of PIF was performed with In-Check DIAL G16 among adult COPD patients treated with DPI and had suboptimal PIF. Eligible subjects were randomised using block randomisation into two groups, either the interventional group or the control group. Twenty-two COPD patients fulfilled the study criteria and were randomised to intervention (n = 11) and control (n = 11) groups. For the interventional group, there were statistically significant improvements between baseline and at 12 weeks for both FEV1 and CAT scores. The mean (% predicted) FEV1 were 54.6 ± 20.4% and 56.6 ± 19.8% (p = 0.026), pre-and post-intervention. The mean CAT score at baseline was 24.4 ± 5.8 and reduced to 19.6 ± 4.4 at 12 weeks (p = 0.012). For the control group, the mean (% predicted) FEV1 at baseline was 58.0 ± 21.9% and 56.5 ± 20.7% at 12 weeks, with no statistical significance difference (p = 0.143). However, there was a statistically significant difference in CAT scores at baseline and 12 weeks, with a mean of 26.5 ± 6.1 and 23.3 ± 5.6, respectively (p = 0.010). The findings from the present pilot RCT highlighted that inhaler personalisation based on PIF among COPD patients was feasible and practical.

Effect of pulmonary rehabilitation on all-cause mortality in patients with chronic respiratory disease: a retrospective cohort study in an Australian teaching hospital

BackgroundPulmonary rehabilitation (PR) is widely recommended for short-term benefits in chronic respiratory diseases, yet long-term outcomes remain uncertain. This retrospective cohort study addresses this gap, comparing 20-year mortality rates between PR participants and matched controls, and hypothesizing that the short-term benefits of PR contribute to improved long-term survival.MethodsThe 20-year mortality of stable chronic respiratory patients who participated in an outpatient PR program was compared with a matched control group based on the type of lung disease. Demographic and clinical variables, and the dates of deaths, were extracted and compared between two groups with two sample t-test and chi-square tests. Kaplan-Meier plots and Cox regression analyses were employed to evaluate survival differences.ResultsBetween 2000 and 2002, 238 individuals enrolled in a pulmonary rehabilitation (PR) program (58% male, mean age ± SD: 69 ± 8 years, mean FEV1% predicted ± SD: 46 ± 21%). An equal number of people with comparable lung disease were selected as controls (88% COPD, 5% ILD). Controls had lower FEV1% predicted values (mean ± SD: 39 ± 17%, P < 0.001), smoked more (mean ± SD: 48 ± 35 pack-years, P = 0.032), and no differences in age, BMI, sex, and Index of Relative Socio-economic Advantage and Disadvantage (IRSAD). Median (IQR) follow-up time was 68 months (34–123), with 371 (78%) deaths. Univariable (HR = 1.71, p < 0.001) and multivariable (HR = 1.64, p < 0.001) Cox regression found higher mortality risk in controls. Subgroup analysis for COPD replicated these findings (HR = 1.70, P < 0.001).DiscussionDespite some methodological limitations, our study suggests that clinically stable patients with chronic respiratory disease who undertake PR may have lower mortality than matched controls.Trial registrationRetrospectively registered.

Comparison of Longitudinal Outcomes in Children with Primary Ciliary Dyskinesia and Cystic Fibrosis.

Primary ciliary dyskinesia (PCD) and cystic fibrosis (CF) are both genetic diseases of mucociliary clearance resulting in progressive lung disease with onset in early life. PCD is often considered to be milder in childhood than CF, based on minimal evidence. Similar to CF, genotype-phenotype associations exist in PCD: pathogenic variants in CCDC39 and CCDC40, causing inner dynein arm/microtubular defects (IDA/MTD) are associated with more severe disease. To compare longitudinal outcomes in matched children with PCD and CF. We hypothesized that children with PCD with IDA/MTD defects would have lower lung function but better nutritional indices than matched children with CF with minimal function genotypes (i.e., those associated with pancreatic insufficiency). Children with PCD enrolled in a prospective, multicenter, observational study were matched with CF patients from the CF Foundation Patient Registry by birth cohort, age, sex, race/ethnicity and year of study visit. The association of disease group overall and by severity class (PCD-IDA/MTD versus all other defects and CF-minimal versus residual function) with longitudinal outcomes up to age 17 was evaluated with cubic spline mixed effects models. Groups included 136 children with PCD (40 IDA/MTD, 96 other) and 476 with CF (446 minimal function, 30 residual function). Below age 14, the PCD group had similar or lower estimated mean FEV1 % predicted compared to CF (e.g., at age 10, -5.4 % predicted lower (95% CI: -7.7, -3.1)). Compared to the CF-minimal function (pancreatic insufficient) group, the PCD-IDA/MTD group had similar BMI; estimated mean FEV1 % predicted was significantly lower by age 10 (mean difference -10.6% (95% CI: -14.7, -6.4), increasing to -15.7% (95% CI: -20.3, -11.2) at age 14. The CF cohort had increased prevalence of Pseudomonas aeruginosa cultured on one or more occasions compared to children with PCD (67% vs 27%, p<0.001); there was no difference in prevalence of P. aeruginosa between children with PCD-IDA/MTD and PCD-other. In childhood, average lung function abnormalities in PCD are not milder than CF, particularly for those with IDA/MTD ciliary defects. New guidelines and treatments to improve outcomes in PCD are urgently needed.

Reduction in FEV1 following spinal anesthesia is associated with intraoperative complications: A prospective study.

Although Spinal Anesthesia (SA) remains the technique of choice for many surgeries below the umbilicus, it is associated with multiple intraoperative complications. Sympathetic blockade and Bezold-Jarisch reflex do not fully explain SA-related cardiopulmonary complications. Reduction in FEV1 has been reported as a predictor of sudden cardiac death. This study aimed to determine the association between reduction in FEV1 following SA and adverse intraoperative cardiopulmonary complications. A prospective study of 48 patients of ASA status I and II with no history of primary cardiopulmonary disease scheduled for elective surgery under SA. Spirometry was performed based on ATS/ERS guidelines before induction and 30 min after induction of SA. FEV1% predicted was determined using GLI 2012 equations. Participants were grouped into two (∆FEV1% < 10% and ∆FEV1% ≥ 10%) based on reductions (∆) in FEV1% predicted following SA. Logistic regression analyses were used to examine associations between ∆FEV1% and intraoperative hypoxia, hypotension, bradycardia, and nausea/vomiting, with adjustments for age, gender, and BMI. The mean FEV1% predicted following SA was lower than the mean FEV1% predicted before SA (83.42 vs. 95.31, p = 0.001). In a fully adjusted model, ∆FEV1% ≥ 10% was associated with an increased risk of hypoxia [AOR 13.55; 95% CI, 1.07-171.24, p = 0.044]. The positive associations between ∆FEV1% ≥ 10% and hypotension [2.02 (0.33-12.46), 0.449], bradycardia [1.10 (0.28-4.25), 0.895] and nausea/vomiting [9.74 (0.52-183.94), 0.129] were not statistically significant. Reduction in FEV1% predicted following SA was associated with adverse intraoperative outcomes. FEV1 may play an important role in the association between SA and cardiopulmonary complications.

Plasma Fibrinogen as a Biomarker for Mortality and Hospitalized Exacerbations in People with COPD

Abstract Background Chronic obstructive pulmonary disease (COPD), predominantly prevalent in smokers, is a chronic inflammatory disorder of the airway and lungs. COPD is one of the most prevalent diseases and the third leading cause of death globally. COPD is a common, preventable, treatable disease that characterize by persistent chest symptoms and airflow limitation due to air way or, and alveolar abnormalities. Fibrinogen is a soluble protein that is produced in the liver and released into the bloodstream, with normal ranges of 200-400 mg/dL or 2-4 g/L. COPD and fibrinogen are associated with frequency of hospitalizatied exacerbations of COPD, and related to the severity of the disease and mortality. Objective To explore whether the fibrinogen level affects the mortality and hospitalized excerbations in COPD patients. Methods The present study was conducted upon 50 subjects during the period from August 2022 to January 2023 who were admitted to our chest department at Ain Sham University Hospitals. Results We made our study on 50 smoker patients, spirometric assessment for all patients was done to confirm COPD diagnosis. The mean age was 59.0± 8.83 years. There were 47 (94%) males and 3 (6%) females with male to female ratio was 16.7:1. Fibrinogen test was done during exacerbation with mean fibrinogen level was 3.64± 1.01 g/L. According to cutoff of fibrinogen level, most of excerbated patients had high fibrinogen level (&amp;gt;3.2). The mean FEV1 was 41.06± 18.75 there was a significant negative correlation between fibrinogen level and FEV1 shows that high fibrinogen level was significantly higher in cases with severe and very severe conditions. Most cases (94%) were discharged while three cases (6%) died shows that there was no significant relation between fibrinogen level and mortality. Conclusion Aecopd associated with high fibrinogen level and that the plasma fibrinogen level was increased with decreasing value of FEV1 showing significant negative correlation between plasma fibrinogen level and FEV1% predicated value. this suggests that high plasma fibrinogen level could be a diagnosis of severity of copd and a poor prognostic factor. but no significant relation between fibrinogen level and mortality. the results of the present study, combined with the fact that plasma fibrinogen is a widely and rapidly available, easy to interpret, low-cost biomarker, suggest a possible role for plasma fibrinogen in the identification of copd patients at an increased risk of adverse outcomes.

Correlation Between Salivary Cortisol Levels and Diurnal Variation in Spirometric Parameters in Apparently Healthy Adults.

This study investigated diurnal variations in spirometric parameters and their correlation with salivary cortisol levels among apparently healthy adults. Forty subjects, aged 18-22, were assessed. Spirometric parameters, including forced vital capacity (FVC), forced expiratory volume in one second (FEV1), forced expiratory flow during 25%-75% of expiration, and peak expiratory flow rate, were measured in the morning, afternoon, and evening. Salivary cortisol levels were also measured at these intervals. Statistical analyses included calculating means, standard deviations, confidence intervals, and Pearson correlation coefficients. The mean FEV1 values were 2.8325 L in the morning, 2.9263 L in the afternoon, and 2.9543 L in the evening, slightly increasing throughout the day but with overlapping confidence intervals, suggesting no statistical significance. The FEV1/FVC ratios displayed a similar pattern. FVC values decreased slightly from morning to evening, but these changes were not statistically significant. Pearson correlation analysis revealed a strong positive correlation between salivary cortisol and FVC (r = 0.829, p = 0.055), a moderate negative correlation with FEV1 (r = -0.649, p = 0.038), and a strong negative correlation with the FEV1/FVC ratio (r = -0.730, p = 0.040). Spirometric parameters exhibited minor diurnal variations, with no statistically significant changes for most measures except for salivary cortisol levels, which significantly decreased throughout the day. Significant correlations were observed between salivary cortisol and certain spirometric parameters.

Correlation of spirometry findings and post-six-minute walk test oxygen desaturation in chronic simple silicosis patients with age, duration of silica exposure, smoking pack years, occupation and mean pulmonary artery pressure.

Silicosis is associated with preventable but irreversible lung damage. Early quantifiable assessment of silicosis workers would promote early interventional steps to reduce health deterioration. The objectives of this study were to correlate spirometry findings and post-six-minute walk test oxygen desaturation (post-6MWT OD) in chronic simple silicosis with age, duration of silica exposure (DSE), smoking pack years (SPY), occupation, and mean pulmonary artery pressure. Based on occupational exposure to silica and radiologic confirmation of chronic simple silicosis, 104 patients (all males) were enrolled and grouped based on SPY (nil, 1-10, 11-20, and >20) and occupation (drillers and dressers). They were further investigated with spirometry, post-6MWT OD, and transthoracic echocardiography (TTE), and findings were statistically analyzed. Abnormal spirometry findings were seen in 62.5% of total cases (65/104), with the highest percentage in the >20 SPY group (84%; 21/25) and drillers (68.3%; 28/41). The post-6MWT OD was seen in 50.96% of cases (53/104) with the highest percentage in the >20 SPY group (56%; 14/25) and drillers (63.4%; 26/41). Normal and restrictive patterns were predominant among <20 SPY groups, while obstructive and mixed patterns were prevalent in the >20 SPY group. Normal, obstructive, and restrictive patterns were predominant among dressers, while mixed patterns were in drillers. Mean age and mean DSE were higher for >20 SPY group and dressers; obstructive and mixed patterns; and patients with post-6MWT OD. Pulmonary hypertension was significantly associated with the presence of abnormal spirometry patterns (69.3%; 45/65) and post-6MWT OD (79.3%; 42/53). Mean forced expiratory volume in one second (FEV1)/forced vital capacity (FVC) was significantly lower for the >20 SPY group. Mean FEV1 had an inverse relation with SPY, and mean FVC was lower for drillers than dressers. Spirometry, post-6MWT OD, and TTE assessment give a complete overview of the cardiopulmonary exercise capacity of chronic silicosis patients and facilitate early interventions with special consideration for workers involved in machine-based mining activity.

Impact of the expanded label for elexacaftor/tezacaftor/ivacaftor in people with cystic fibrosis with no F508del variant in the USA.

Elexacaftor/tezacaftor/ivacaftor (ETI), which is approved for people with cystic fibrosis (pwCF) with a F508del variant, was further approved based on in vitro data in the USA for those carrying at least one of 177 rare CFTR (cystic fibrosis transmembrane conductance regulator) variants. PwCF, aged ≥6 years, carrying no F508del variant but with at least one of these 177 rare variants, were identified within the US Cystic Fibrosis Foundation Patient Registry (CFFPR) between 2020 and 2022. The evolution of forced expiratory volume in 1 s (FEV1) percentage predicted and rates of pulmonary exacerbations were analysed over the first year following ETI initiation, using a linear regression with generalised estimating equations and a negative binomial model, respectively. A total of 1791 individuals aged ≥6 years with rare CFTR variants were eligible for ETI, corresponding to 5.2% of CFFPR participants. 815 individuals (45.5%), of which 57.9% were already treated with another CFTR modulator, initiated ETI within the first 2 years following approval. Individuals with more severe respiratory disease were more likely to initiate ETI, whereas those previously treated with another CFTR modulator or those with no private insurance coverage had less ETI initiation. ETI initiation was associated with an increase in mean FEV1 % pred by +3.39 (95% CI 2.14-4.64) and a decrease in the rates of pulmonary exacerbations (adjusted rate ratio 0.55, 95% CI 0.38-0.79). These effects were greater in individuals naïve of previous CFTR modulators. Extension of the ETI label to rare CFTR variants is associated with meaningful improvements in lung function and a marked reduction in pulmonary exacerbations.

Diabetic Lung: An Entity Complicated by Coronavirus Disease 2019 Pneumonia

ABSTRACT Background: Diabetes is an important public health problem and considering the extent of the aftermath following coronavirus disease 2019 (COVID-19) pandemic, this study aimed to evaluate the effect of each disease on lung function individually and also to assess how both the disease entities together in combination affect the lungs. Methods: This observational study included adult patients below the age of 60 years with complaints of fatigue. Included patients were assessed for their basic demographics and history of COVID-19 pneumonia. For those diagnosed with diabetes mellitus (DM), the duration of DM and HbA1C were noted. For all included patients, spirometry was performed. Results were statistically analyzed. Results: Comparing the spirometry measures of DM patients with and without a history of COVID-19 pneumonia, the mean forced expiratory volume in 1 s (FEV1), forced vital capacity (FVC), FEV1/FVC, and forced expiratory flow (FEF) 25%–75% for DM patients with COVID-19 pneumonia history was significantly lower than DM patients without a history of COVID-19 pneumonia (P &lt; 0.0001). Comparing the spirometry measures of patients (DM vs. non-DM) with a history of COVID-19 pneumonia, the mean FEV1, FVC, FEV1/FVC, and FEF 25%–75% for DM patients with COVID-19 pneumonia history was significantly lower than non-DM patients (P &lt; 0.0001). Conclusion: Considering the high burden of the diabetic population and the extent of the pandemic’s aftermath, clinicians should be aware of this and aim to optimize control of blood glucose levels in uncontrolled diabetics with a history of COVID-19 to prevent the deterioration of lung function.

Health State Utilities Associated With Treatment Burden in Cystic Fibrosis: A Patient Valuation Study

BACKGROUNDWhile recent advancements in the treatment of cystic fibrosis (CF) have improved survival, reducing high levels of treatment burden remains a priority issue for many people with CF (pwCF). However, economic evaluations of novel interventions may fail to capture their impact on treatment burden due to a lack of suitable outcome measures. This study aimed to estimate health state utilities (HSUs), for changes in treatment burden associated with different CF treatments. RESEARCH QUESTIONWhat value do pwCF place on changes in treatment burden associated with intravenous antibiotic treatment of pulmonary exacerbations, inhaled medicines, and physiotherapy? STUDY DESIGNAND METHODS: Adults attending a specialist CF center were invited to participate in a web-based time trade-off interview. Participants valued their own health, and five health state vignettes describing varying levels of intensity of physiotherapy, inhaled, and IV antibiotic treatment. HSUs for additional instances of each treatment type were estimated using mixed effect linear regression models. RESULTSFifty one people with CF completed the interview (median age, 30 years; range, 19-66); 53% were female; mean FEV(1) % predicted, 65% [SD, 20%]).Mean utility scores for own health were very similar between the EQ-5D index value (0.81, SD 0.20) and the TTO value (0.82, SD 0.20), however limited concordance was observed at the individual level. Adjusted utility decrements associated with treatment burden were -0.037 (SE 0.008) for an additional annual IV antibiotic treatment, -0.029 (SE 0.014) for an additional daily physiotherapy session, and -0.019 (SE 0.013) for an additional daily inhaled medicine. INTERPRETATIONIncreasing treatment burden was associated with decreasing HSU values. The utility decrements associated with treatment burden changes suggest meaningful differences in health-related quality of life (HRQoL) for pwCF. These findings align with existing literature on the impact of treatment burden on HRQoL, and highlight the importance of considering treatment burden in economic evaluations of interventions in CF.

Bronchodilator Response in Post-COVID-19 Patients Undergoing Pulmonary Rehabilitation

Introduction: SARS-CoV-2 infections can result in a broad spectrum of symptoms from mild to life-threatening. Long-term consequences on lung function are not well understood yet. Methods: In our study, we have examined 134 post-COVID patients (aged 54.83 ± 14.4 years) with dyspnea on exertion as a leading symptom 6 weeks to 24 months after a SARS-CoV-2 infection for bronchodilator responsiveness during their stay in our pulmonary rehabilitation clinic. Results: Prior to bronchial dilation, 6 out of 134 patients (4.47%) presented an FEV1/FVC ratio below lower limit of normal (Z-score = −1.645) indicative of an obstructive airway disease. Following inhalation of a β2-adrenergic agonist we measured a mean FEV1 increase of 181.5 mL in our cohort, which was significantly elevated compared to a historical control group (ΔFEV1 = 118 mL). 28.7% of the patients showed an increase greater than 200 mL and 12% displayed a significant bronchodilation response (>200 mL ΔFEV1 and >12% FEV1 increase). Interestingly, no significant difference in bronchial dilation effect was observed when comparing patients hospitalized and those non-hospitalized during the course of their SARS-CoV-2 infection. Conclusion: Our data provide evidence for increased prevalence of obstructive ventilatory defects and increased bronchodilator responsiveness in patients with persisting symptoms after COVID-19. Depending on the extent of this complication, post-COVID patients may benefit from an adapted β2-inhalation therapy including subsequent reevaluation.

Longitudinal lung function trajectories in response to azithromycin therapy for chronic lung disease in children with HIV infection: a secondary analysis of the BREATHE trial

BackgroundChronic lung disease (CLD) is common among children with HIV (CWH) including in those taking antiretroviral therapy (ART). Azithromycin has both antimicrobial and anti-inflammatory effects and has been effective in improving lung function in a variety of lung diseases. We investigated lung function trajectories among CWH with CLD on ART enrolled in a randomized controlled trial of adjuvant azithromycin. We also investigated factors that modified the effect of azithromycin on lung function.MethodsThe study used data from a double-blinded placebo-controlled trial conducted in Malawi and Zimbabwe of 48 weeks on azithromycin (BREATHE: ClinicalTrials.gov NCT02426112) among CWH aged 6 to 19 years taking ART for at least six months who had a forced expiratory volume in one second (FEV1) z-score <-1.0. Participants had a further follow-up period of 24 weeks after intervention cessation. FEV1, forced vital capacity (FVC) and FEV1/FVC were measured at baseline, 24, 48 and 72-weeks and z-scores values calculated. Generalized estimating equations (GEE) models were used to determine the mean effect of azithromycin on lung-function z-scores at each follow-up time point.ResultsOverall, 347 adolescents (51% male, median age 15 years) were randomized to azithromycin or placebo. The median duration on ART was 6.2 (interquartile range: 3.8–8.6) years and 56.2% had an HIV viral load < 1000copies/ml at baseline. At baseline, the mean FEV1 z-score was − 2.0 (0.7) with 44.7% (n = 155) having an FEV1 z-score <-2, and 10.1% had microbiological evidence of azithromycin resistance. In both trial arms, FEV1 and FVC z-scores improved by 24 weeks but appeared to decline thereafter. The adjusted overall mean difference in FEV1 z-score between the azithromycin and placebo arms was 0.004 [-0.08, 0.09] suggesting no azithromycin effect and this was similar for other lung function parameters. There was no evidence of interaction between azithromycin effect and baseline age, lung function, azithromycin resistance or HIV viral load.ConclusionThere was no observed azithromycin effect on lung function z-scores at any time point suggesting no therapeutic effect on lung function.Trial registrationClinicalTrials.gov NCT02426112. First registered on 24/04/2015.

Is Spirometry a Sufficient Test for Assessing Respiratory Function after Lung Resection?

The prediction of postoperative functional status in non-small cell lung cancer patients based on preoperative assessment of physical and respiratory capacity is inadequate based on recent RCTs. Prospectively collected spirometry data and the six-minute walk test results of 57 patients treated with lobectomy for non-small cell lung cancer were analyzed. The tests were performed before surgery, and 30 and 90 days after lobectomy. All patients underwent a respiratory functional and physical capacity assessment. All 57 patients underwent lobectomy. Before surgery, mean FEV1 was 2.4 ± 0.7 L, corresponding to %FEV1 of 88.3 ± 17.3%. The mean absolute and expected 6MWT distance was 548 ± 74.6 m and 108.9 ± 14.5%, respectively. At the first postoperative evaluation 30 days after surgery, FEV1 and %FEV1 decreased significantly by an average of 0.5 ± 0.3 L and 15.1 ± 10.7%, while 6MWT and expected 6MWT decreased minimally by an average of 1.0 m and 0.8%, respectively. Three months after lobectomy, FEV1 and %FEV1, compared with the initial assessment, decreased by an average of 0.3 ± 0.3 l and 7.8 ± 10.0%, while 6MWT and its expected score increased to 564.6 ± 84.6 m and 112.8 ± 15.8%, respectively. After lobectomy, FEV1 decreased slightly and less than expected, while 6MWT increased proportionally compared to the preoperative evaluation.

Association of bronchial disease on CT imaging and clinical definitions of chronic bronchitis in a single-center COPD phenotyping study

IntroductionChronic Bronchitis (CB) represents a phenotype of chronic obstructive pulmonary disease (COPD). While several definitions have been used for diagnosis, the relationship between clinical definitions and radiologic assessment of bronchial disease (BD) has not been well studied. The aim of this study was to evaluate the relationship between three clinical definitions of CB and radiographic findings of BD in spirometry-defined COPD patients. MethodsA cross-sectional analysis was performed from a COPD phenotyping study. It was a prospective observational cohort. Participants had spirometry-defined COPD and available chest CT imaging. Comparison between CB definitions, Medical Research Council (CBMRC), St. George's Respiratory Questionnaire (CBSGRQ), COPD Assessment Test (CBCAT) and CT findings were performed using Cohen's Kappa, univariate and multivariate logistic regressions. ResultsOf 112 participants, 83 met inclusion criteria. Demographics included age of 70.1 ± 7.0 years old, predominantly male (59.0 %), 45.8 ± 30.8 pack-year history, 21.7 % actively smoking, and mean FEV1 61.5 ± 21.1 %. With MRC, SGRQ and CAT definitions, 22.9 %, 36.6 % and 28.0 % had CB, respectively. BD was more often present in CB compared to non-CB patients; however, it did not have a statistically significant relationship between any of the CB definitions. CBSGRQ had better agreement with radiographically assessed BD compared to the other two definitions. ConclusionIdentification of BD on CT was associated with the diagnoses of CB. However, agreement between imaging and definitions were not significant, suggesting radiologic findings of BD and criteria defining CB may not identify the same COPD phenotype. Research to standardize imaging and clinical methods is needed for more objective identification of COPD phenotypes.

Long-term lung function follow-up of preterm infants less than 32 weeks of gestational age.

Preterm infants, particularly those with bronchopulmonary dysplasia (BPD), are at risk of lung development problems. Over the last decades, lung protective strategies have been used, decreasing the risk of chronic lung disease. To evaluate the pulmonary function test (PFT) of preterm infants born after the introduction of lung protective strategies and to assess perinatal determinants of impaired lung function in this population. A prospective, observational, single-center study was conducted in the neonatal unit of a high-complexity hospital. The study included newborns withless than 32 weeks gestational age born between 2012 and 2014, who were followed up until they reach school age. For the main outcome, two groups were stablished: no BPD or grade 1 BPD (no BPD/1) and grade 2 or 3 BPD (BPD 2/3). Out of 327 patients, 116 were included. BPD was diagnosed in 49.1% (47), with 50.9% (29) classified as grade 1, 35.1% (20) as grade 2, and 14.0% (8) as grade 3. Mean age at PFT was 8.59 years (SD 0.90). Mean FEV1% was 95.36% (SD 13.21) and FEV1 z-score -0.36 (SD 1.12); FVC% 97.53% (SD 12.59) and FVC z-score -0.20 (SD 1.06); FEV1/FVC ratio 85.84% (SD 8.34) and z-score -0.24 (SD 1.34). When comparing patients with no BPD/1 and BPD 2/3, we observed differences in all pulmonary function parameters, which persisted after adjusting for gestational age. No differences in PFT were observed between patients without BPD and those with grade 1 BPD. Most patients (76.7%, 89) had normal spirometry pattern, with obstructive pattern observed in 12.9% (15), restrictive pattern in 9.5% (11), and mixed pattern in 0.9% (1) of patients. Preterm infants with BPD 2/3 showed a decrease in all pulmonary function parameters compared to preterm infants with no BPD/1; an effect that was independent of gestational age. Among patients with BPD who had an altered PFT pattern, the most common pattern was obstructive, followed by restrictive and then, mixed.

Clinical-Epidemiological Profile and Distribution of Interstitial Lung Diseases: A Tertiary Care Centre Experience from North India

Background: The Interstitial Lung Disease (ILD) India registry, which included more than 1000 patients across 27 centers of the country, reported Hypersensitivity Pneumonitis (HP) to be the most common ILD in India, followed by Connective Tissue Disease-related ILD (CTD-ILD) and Idiopathic Pulmonary Fibrosis (IPF). This study was carried out to evaluate whether the spectrum of ILDs at our center conforms to the national data. Aims: To evaluate the clinical and epidemiological profile, distribution of various ILDs, and any association of the type of ILD with clinical parameters. Materials and methods: A prospective, observational study was carried out at a tertiary care center in North India, where patients having symptoms and radiological features suggestive of ILD were evaluated for clinical, radiological features and spirometric indices. Results: A total of 83 patients with ILD were evaluated. The mean age was 53.76 ± 13.07 years. The male-to-female ratio was 0.97. The mean FEV1 was 1.41 ± 0.38 L, mean FVC was 1.57 ± 0.43 L and the mean FEV1/FVC was 90.26 ± 8.99%. The mean 6 MWD was 270.85 ± 110.52 m. Reticulation and honeycombing were the most common radiological features. IPF was the most common ILD (28.9%). Conclusion: IPF was the most common ILD at our center. A statistically significant association was found between type of ILD and age, gender, past ATT intake, HTN, clubbing, and FEV1/FVC%.