ATP binding cassette member A3 (ABCA3): coming of age
Pathogenic variants in the ATP binding cassette member A3 (ABCA3) gene were first described in newborn infants with lethal respiratory failure in 2004.1 Subsequent reports have expanded the clinical spectrum associated with biallelic ABCA3 variants to include infants with the phenotype of childhood interstitial lung disease (chILD), who presented after the newborn period and had prolonged survival, even into adulthood.2–4 A genotype–phenotype correlation has emerged with biallelic null (frameshift or nonsense) ABCA3 variants predicting neonatal onset of respiratory failure and death prior to 1 year of age witho...
Source: Thorax - May 16, 2023 Category: Respiratory Medicine Authors: Casey, A., Nogee, L., Wambach, J. Tags: Thorax Editorial Source Type: research
Do the 'missing millions of COPD patients want to be found?
Finding the ‘missing millions’ of patients with COPD has become a mantra of health policy. In the UK, 1.4 million people have a diagnosis of COPD but it is estimated that 50%–65% of people living with COPD are undiagnosed. This has been made worse by the COVID pandemic when new diagnosis rates fell by over half. In a recent survey of patients with COPD, 22% reported waiting 5 years or more for a diagnosis after noticing symptoms.1 In many cases, they had problems accessing diagnostic testing with spirometry; however, one in three said it was because they had waited a year or more before seeking help. The ...
Source: Thorax - May 16, 2023 Category: Respiratory Medicine Authors: Halpin, D. M. Tags: Thorax Editorial Source Type: research
Do people living with HIV have accelerated lung function decline, despite adequate treatment?
Accelerated lung function decline is relatively common from the late 20s and indicates chronic lung disease in many communities, including those living with HIV.1 2 The factors influencing and mechanisms of lung function decline in people living with HIV (PLWH) or without HIV are complex and multifactorial.2 This includes changes in the lungs and respiratory system, decreased muscle mass, and decreased lung elasticity. As we age, the lungs lose their ability to expand and contract as quickly, which can lead to a decrease in lung capacity and difficulty breathing.3 Other factors, such as smoking, exposure to pollution and c...
Source: Thorax - May 16, 2023 Category: Respiratory Medicine Authors: Alif, S. M., Rahman, M. A. Tags: Thorax Editorial Source Type: research
Journal club
Persistent airflow limitation in asthma: not exclusive to those with severe disease and linked with exacerbations Persistent airflow limitation (PAL) is an asthma phenotype defined as airflow obstruction that remains after the use of a β2-agonist. Past studies on PAL have included small populations and were mostly restricted to severe asthma. Kole et al (Lancet Respir Med 2023;11:55) conducted a post hoc analysis to investigate the determinants and outcomes of PAL in patients with asthma who were included in the Assessment of Small Airways Involvement in Asthma (ATLANTIS) study, a multicentric cohort with 1-year follo...
Source: Thorax - April 13, 2023 Category: Respiratory Medicine Authors: Faria, N. Tags: Thorax Journal club Source Type: research
When is a lung abscess not a lung abscess? Recurrent pneumonia and empyema associated with intrapulmonary cystic teratoma
A 23-year-old woman presented with gradual-onset left chest and back pain. She had at medical history of asthma. She was a never smoker with no significant family history. Her observations and examination were normal. Blood tests performed revealed a raised C reactive protein of 119 mg/L and high white cell count of 12.81x109/L. A chest radiograph showed left hilar consolidation (figure 1A). She was treated with oral clarithromycin and follow-up radiograph showed resolution of the consolidation (figure 1B). One year later, she represented with a 10-day history of dry cough and pleuritic chest pain. A chest radiograph demon...
Source: Thorax - April 13, 2023 Category: Respiratory Medicine Authors: Penman, C., Dixon, G., Daly, R., Low, A. Tags: Images in Thorax, Thorax Source Type: research
Cost-effectiveness of home non-invasive ventilation in patients with persistent hypercapnia after an acute exacerbation of COPD in the UK
Home non-invasive mechanical ventilation (HMV) with home oxygen therapy (HOT) in patients with persistent hypercapnia following an acute exacerbation of chronic obstructive pulmonary disease delays hospital readmission. The economic impact of this treatment is unknown. We evaluated the cost-effectiveness of HMV in the UK healthcare system using data from a previously published efficacy trial. Quality-adjusted life-years (QALYs) were computed from EQ-5D-5L. Accounting for all direct patient costs HOT-HMV was £512 (95%CI £36 to £990) more expensive per patient per year than HOT-alone. This small increase in...
Source: Thorax - April 13, 2023 Category: Respiratory Medicine Authors: Murphy, P. B., Brueggenjuergen, B., Reinhold, T., Gu, Q., Fusfeld, L., Criner, G., Goss, T. F., Hart, N. Tags: Open access, Thorax Brief communication Source Type: research
Ultra-low-dose CT versus chest X-ray for patients suspected of pulmonary disease at the emergency department: a multicentre randomised clinical trial
Conclusions
Short-term functional health was comparable between ULDCT and CXR, as were hospital admissions and LOS, but more incidental findings were found in the ULDCT group. Our trial does not support routine use of ULDCT in the work-up of patients suspected of non-traumatic pulmonary disease at the emergency department.
Trial registration number
NTR6163. (Source: Thorax)
Source: Thorax - April 13, 2023 Category: Respiratory Medicine Authors: van den Berk, I. A. H., Kanglie, M. M. N. P., van Engelen, T. S. R., Altenburg, J., Annema, J. T., Beenen, L. F. M., Boerrigter, B., Bomers, M. K., Bresser, P., Eryigit, E., Groenink, M., Hochheimer, S. M. R., Holleman, F., Kooter, J. A. J., van Loon, R. Tags: Open access, Thorax Respiratory infection Source Type: research
Placental mesenchymal stem cells boost M2 alveolar over M1 bone marrow macrophages via IL-1{beta} in Klebsiella-mediated acute respiratory distress syndrome
Conclusions
Human PMSC treatment preferentially rescued resident M2 AMs over recruited M1 BMMs with overall M2 polarisation to improve KP-related ARDS survival. (Source: Thorax)
Source: Thorax - April 13, 2023 Category: Respiratory Medicine Authors: Wang, L.-T., Yen, B. L., Wang, H.-H., Chao, Y.-Y., Lee, W., Huang, L.-Y., Chiu, S.-K., Siu, L. K., Liu, K.-J., Sytwu, H.-K., Yen, M.-L. Tags: Open access, Thorax Respiratory infection Source Type: research
Mendelian randomisation of eosinophils and other cell types in relation to lung function and disease
Conclusions
Broad consistency across MR methods may suggest causation by eosinophils (although of uncertain magnitude), yet heterogeneity necessitates caution: other important mechanisms may be responsible for the impairment of respiratory health by these eosinophil-raising variants. These results could suggest that anti-IL5 agents (designed to lower eosinophils) may be valuable in treating other respiratory conditions, including people with overlapping features of asthma and COPD. (Source: Thorax)
Source: Thorax - April 13, 2023 Category: Respiratory Medicine Authors: Guyatt, A., John, C., Williams, A. T., Shrine, N., Reeve, N. F., SpiroMeta consortium, Sayers, I., Hall, I., Wain, L. V., Sheehan, N., Dudbridge, F., Tobin, M. D., Cook, Deary, Enroth, Ewert, Gieger, Gyllensten, Harris, Hayward, Homuth, Hui, Imboden, Jame Tags: Open access, Thorax Respiratory epidemiology Source Type: research
Optimising breathlessness triggered services for older people with advanced diseases: a multicentre economic study (OPTBreathe)
Conclusion
Breathlessness services are cost-effective compared with usual care for health and social care, giving cost savings and better quality of life. Uptake of breathlessness services is higher when service attributes are individually tailored. (Source: Thorax)
Source: Thorax - April 13, 2023 Category: Respiratory Medicine Authors: Yi, D., Reilly, C. C., Wei, G., Higginson, I. J., on behalf of OPTBreathe collaborators, Baxter, Birring, Patel Tags: Open access, Thorax Rehabilitation Source Type: research
Bronchopulmonary dysplasia and expiratory airflow at 8 years in children born extremely preterm in the post-surfactant era
Conclusions
In children born EP, moderate–severe BPD, but not mild BPD was independently associated with reduced expiratory airflow at 8 years. (Source: Thorax)
Source: Thorax - April 13, 2023 Category: Respiratory Medicine Authors: Doyle, L. W., Ranganathan, S., Cheong, J., The Victorian Infant Collaborative Study Group, Anderson, Burnett, Charlton, Davis, Duff, Lee, McDonald, Opie, Williamson Tags: Thorax Paediatric lung disease Source Type: research
Minimal important difference in childhood interstitial lung diseases
Conclusions
For the first time, we provide estimates of MIDs for vital signs and HrQoL scores in a large cohort of chILD using different methods. (Source: Thorax)
Source: Thorax - April 13, 2023 Category: Respiratory Medicine Authors: Griese, M., Schwerk, N., Carlens, J., Wetzke, M., Emiralioglu, N., Kiper, N., Lange, J., Krenke, K., chILD collaborators, Seidl, E. Tags: Open access, Thorax Paediatric lung disease Source Type: research
Impact of time-to-treatment on survival for advanced non-small cell lung cancer patients in the Netherlands: a nationwide observational cohort study
Conclusion
Although in general it is important to start treatment as early as possible, our study finds no evidence that a more rapid start of treatment improves outcomes in advanced stage NSCLC patients. The benefit of urgent treatment is probably confounded by unmeasured patient and tumour characteristics and, clinical urgency dictating timelines of treatment. Time-to-treatment and its impact should be continuously evaluated as therapeutic strategies continue to evolve and improve. (Source: Thorax)
Source: Thorax - April 13, 2023 Category: Respiratory Medicine Authors: Klarenbeek, S. E., Aarts, M. J., van den Heuvel, M. M., Prokop, M., Tummers, M., Schuurbiers, O. C. J. Tags: Editor's choice, Thorax Lung cancer Source Type: research
Short-term exposure to ambient air pollution and individual emergency department visits for COVID-19: a case-crossover study in Canada
Conclusions
We found associations between short-term exposure to ambient air pollutants and COVID-19 ED visits. Exposure to air pollution may also lead to more severe COVID-19 disease. (Source: Thorax)
Source: Thorax - April 13, 2023 Category: Respiratory Medicine Authors: Lavigne, E., Ryti, N., Gasparrini, A., Sera, F., Weichenthal, S., Chen, H., To, T., Evans, G. J., Sun, L., Dheri, A., Lemogo, L., Kotchi, S. O., Stieb, D. Tags: Open access, Thorax Environmental exposure Source Type: research
Budesonide/formoterol maintenance and reliever therapy versus fluticasone/salmeterol fixed-dose treatment in patients with COPD
Conclusions
This first study of MART in COPD found that budesonide/formoterol MART might be similarly effective to fluticasone/salmeterol fixed-dose therapy in moderate to severe patients with COPD, at a lower daily ICS dosage. Further evidence is needed about long-term safety. (Source: Thorax)
Source: Thorax - April 13, 2023 Category: Respiratory Medicine Authors: Muiser, S., Imkamp, K., Seigers, D., Halbersma, N. J., Vonk, J. M., Luijk, B. H. D., Braunstahl, G.-J., van den Berg, J.-W., Kroesen, B.-J., Kocks, J. W. H., Heijink, I. H., Reddel, H. K., Kerstjens, H. A. M., van den Berge, M. Tags: Thorax Chronic obstructive pulmonary disease Source Type: research
