ERJ Open Research最新文献_第6页

Estimates of primary ciliary dyskinesia prevalence: a scoping review 原发性睫状肌运动障碍患病率估算：范围界定审查

ERJ Open Research Pub Date : 2024-04-19 DOI: 10.1183/23120541.00989-2023

Wallace B Wee, D. Gatt, E.L.A. Seidl, Giles Santyr, Teresa To, Sharon D Dell

{"title":"Estimates of primary ciliary dyskinesia prevalence: a scoping review","authors":"Wallace B Wee, D. Gatt, E.L.A. Seidl, Giles Santyr, Teresa To, Sharon D Dell","doi":"10.1183/23120541.00989-2023","DOIUrl":"https://doi.org/10.1183/23120541.00989-2023","url":null,"abstract":"Primary ciliary dyskinesia (PCD) is a rare multi-system genetic disease caused by dysfunctional motile cilia. Despite PCD being the second most common inherited airway disease after cystic fibrosis, PCD continues to be underrecognized globally due to non-specific clinical features, and the lack of a gold standard diagnostic test. Commonly repeated prevalence estimates range from 1-in-10,000 to 1-in-20,000, and based on regional epidemiologic studies with known limitations. The purpose of this scoping review is to appraise the PCD literature, to determine the best available global PCD prevalence estimate and to inform the reader about the potential unmet health services needs in PCD.To systematically review the literature about PCD prevalence estimates.A scoping review was conducted following the PRISMA-ScR methodology. Included studies estimated PCD prevalence and used cohort, clinical or genomic data. Excluded articles were case reports, conference abstracts, review articles, animal studies or non-English articles.Literature review identified unique abstracts. Thirty-four underwent full text review and 8 met the inclusion/exclusion criteria. Seven articles were based on epidemiologic studies of specific geographical regions and provided prevalence estimates that ranged from ∼1 to 44.1-in-100,000. Only one study estimated global prevalence, using two large genomic databases, and calculated it to be ∼13.2-in-100,000 (based on pathogenic variants in 29 disease-causing genes).A population based genomic approach for estimating global prevalence has found that PCD is much more prevalent than previously cited in the literature. This highlights the potential unmet health service needs of people living with PCD.","PeriodicalId":504874,"journal":{"name":"ERJ Open Research","volume":null,"pages":null},"PeriodicalIF":0.0,"publicationDate":"2024-04-19","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"140682738","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

引用次数: 0

Longitudinal change in ultrasound-derived rectus femoris cross-sectional area in COPD 慢性阻塞性肺病患者股直肌横截面积的超声波纵向变化

ERJ Open Research Pub Date : 2024-04-19 DOI: 10.1183/23120541.00123-2024

T.O. Jenkins, S. Patel, George Edwards, C. Nolan, J. Canavan, S. Kon, Sarah Jones, R. Barker, Hannah Littlemore, M. Maddocks, W. D. Man

{"title":"Longitudinal change in ultrasound-derived rectus femoris cross-sectional area in COPD","authors":"T.O. Jenkins, S. Patel, George Edwards, C. Nolan, J. Canavan, S. Kon, Sarah Jones, R. Barker, Hannah Littlemore, M. Maddocks, W. D. Man","doi":"10.1183/23120541.00123-2024","DOIUrl":"https://doi.org/10.1183/23120541.00123-2024","url":null,"abstract":"Skeletal muscle dysfunction is common in chronic obstructive pulmonary disease (COPD). Ultrasound-derived rectus femoris cross-sectional area (RFCSA) is a radiation free, non-invasive measure of muscle bulk that relates to quadriceps strength in people with COPD. However, there are limited longitudinal data for RFCSA, and it is not known whether longitudinal change in RFCSA reflects change in quadricep strength, exercise capacity, lower limb function or muscle mass. We aimed to quantify longitudinal change in ultrasound-derived RFCSA and assess its relationship with change in quadriceps maximal voluntary contraction (QMVC), incremental shuttle walk test (ISWT), five repetition sit-to-stand (5STS) and fat free mass (FFM) over 12 months in people with COPD.We measured ultrasound-derived RFCSA, QMVC, ISWT, 5STS and FFM (measured by bioelectric impedance analysis) at baseline and 12 months in 169 people with stable COPD. Change was correlated using Pearsons or Spearman's coefficients.Baseline characteristics: Mean (sd) age 70 (9.4) years; FEV152.6 (18.8)% predicted. Over the course of 12 months mean (99% confidence interval) RFCSA change was −33.7mm2(−62.6 to −4.9, p=0.003) representing a mean (sd) percentage change of −1.8% (33.5). There was a weak correlation between change in RFCSA and FFM (r=0.205 p=0.009), but not with change in QMVC, ISWT or 5STS.There is a statistically significant decrease in ultrasound-derived RFCSA over 12 months in people with stable COPD, but this decrease does not correlate with change in quadriceps strength, exercise capacity, fat free mass nor lower limb function.","PeriodicalId":504874,"journal":{"name":"ERJ Open Research","volume":null,"pages":null},"PeriodicalIF":0.0,"publicationDate":"2024-04-19","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"140682928","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

引用次数: 0

Diagnostic accuracy, feasibility, and acceptability of stool-based testing for childhood tuberculosis 儿童结核病粪便检测的诊断准确性、可行性和可接受性

ERJ Open Research Pub Date : 2024-04-11 DOI: 10.1183/23120541.00710-2023

B. Yenew, P. de Haas, Yohannes Babo, G. Diriba, Bihil Sherefdin, A. Bedru, B. Tegegn, T. Gudina, Tadesse Getahun, Saro Abdella, D. Jerene, Eveline Klinkenberg, E. Tiemersma

{"title":"Diagnostic accuracy, feasibility, and acceptability of stool-based testing for childhood tuberculosis","authors":"B. Yenew, P. de Haas, Yohannes Babo, G. Diriba, Bihil Sherefdin, A. Bedru, B. Tegegn, T. Gudina, Tadesse Getahun, Saro Abdella, D. Jerene, Eveline Klinkenberg, E. Tiemersma","doi":"10.1183/23120541.00710-2023","DOIUrl":"https://doi.org/10.1183/23120541.00710-2023","url":null,"abstract":"Childhood tuberculosis (TB) diagnosis remains challenging, partly because children cannot provide sputum. This study evaluated the diagnostic accuracy of the Simple One-Step (SOS) stool method with Xpert MTB/RIF Ultra (Xpert-Ultra) for childhood TB compared to culture and Xpert-Ultra on a respiratory sample (RS) and clinical diagnosis. It also assessed the feasibility and acceptability of stool testing according to laboratory staff, and caregivers’ sample preference.We enrolled children (≤10 years) with presumptive pulmonary tuberculosis in Ethiopia. RS was tested with Xpert-Ultra and culture; stool samples were tested using the SOS stool method with Xpert-Ultra. Laboratory staff and caregivers’ opinions were assessed using standardized questionnaires.Of 898 children enrolled, 792, 832 and 794 were included for assessing the diagnostic accuracy of SOS stool with Xpert-Ultra against culture, RS Xpert-Ultra, and clinical diagnosis, respectively, yielding sensitivity estimates for SOS stool with Xpert-Ultra of 69.1% (95% confidence interval (CI), 56.0–79.7%), 76.8% (95% CI, 64.2–85.9%), and 59.0% (95% CI, 47.9–69.2%), respectively. The specificity was ≥98.8% for all comparisons. The rate of non-determinate test results was 2.8% after one repeat test. According to laboratory staff, stool collection was feasible, acceptable and the SOS stool method was easy to perform. Most caregivers (75%) preferred stool for TB diagnosis over RS.This study shows that SOS stool Xpert-Ultra testing offers a good alternative to RS testing for TB in children who cannot spontaneously produce a sputum sample and would otherwise need to undergo invasive procedures to obtain RS for diagnosis.","PeriodicalId":504874,"journal":{"name":"ERJ Open Research","volume":null,"pages":null},"PeriodicalIF":0.0,"publicationDate":"2024-04-11","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"140715407","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

引用次数: 0

Low TB-treatment initiation after positive TB-LAM results TB-LAM 阳性结果出来后，结核病治疗启动率低

ERJ Open Research Pub Date : 2024-04-11 DOI: 10.1183/23120541.00182-2024

Tinne Gils, Thandanani Madonsela, M. Kamele, Irene Ayakaka, Alastair Van Heerden, Erika Vlieghe, M. Bresser, Tom Decroo, Lutgarde Lynen, Klaus Reither, Shannon Bosman

引用次数: 0

Standards of care and educational gaps in adult cystic fibrosis units: an ERS survey 成人囊性纤维化病房的护理标准和教育差距：ERS 调查

ERJ Open Research Pub Date : 2024-04-11 DOI: 10.1183/23120541.00065-2024

Almudena Felipe Montiel, A. Álvarez Fernández, Mario Culebras Amigo, Francesco Blasi, Andrea Gramegna, Stuart Elborn, Alexander Horsley, Madge Susan, Daiana Stolz, N. Tabin, C. Pannetier, Eva Polverino

{"title":"Standards of care and educational gaps in adult cystic fibrosis units: an ERS survey","authors":"Almudena Felipe Montiel, A. Álvarez Fernández, Mario Culebras Amigo, Francesco Blasi, Andrea Gramegna, Stuart Elborn, Alexander Horsley, Madge Susan, Daiana Stolz, N. Tabin, C. Pannetier, Eva Polverino","doi":"10.1183/23120541.00065-2024","DOIUrl":"https://doi.org/10.1183/23120541.00065-2024","url":null,"abstract":"Significant progress in the field of Cystic Fibrosis (CF) has substantially extended the life expectancy of patients with CF (pwCF). Consequently, the population of adult pwCF has outnumbered paediatric patients in most developed countries. Ageing is a new factor that can contribute to disease complexity and can require adaptation of CF Units. Therefore, the necessity for standardized, specialized, and multidisciplinary care is imperative. Concerns arise regarding the adequacy of current healthcare, therapeutic, and educational offerings.To address these concerns, a multinational survey was conducted to assess the current state of care in specialized multidisciplinary adult and paediatric CF units and identify areas for improvement. Responses were collected from 44 centres providing regular care to CF patients.The survey unveiled considerable disparities in the availability of critical resources, including diagnostic access, supplementary testing, treatment modalities, transplant and transition programs, and healthcare professionals’ training.This study underscores the urgent need to standardize care across these centres in order to minimize disparities in terms of available resources and training with a particular attention to adult pwCF that are becoming more numerous and showing different needs with ageing. The changing landscape of CF in adulthood will require a constant monitoring to ensure a proper adaptation of the current model of care.","PeriodicalId":504874,"journal":{"name":"ERJ Open Research","volume":null,"pages":null},"PeriodicalIF":0.0,"publicationDate":"2024-04-11","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"140714175","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

引用次数: 0

Maternal use of snus as smokeless tobacco in pregnancy and infant lung function 母亲在怀孕期间使用鼻烟作为无烟烟草与婴儿的肺功能

ERJ Open Research Pub Date : 2024-04-11 DOI: 10.1183/23120541.00958-2023

K. Bains, Ina Kreyberg, M. Färdig, B. Granum, H. Gudmundsdóttir, Guttorm Haugen, G. Hedlin, Katarina Hilde, C.M Jonassen, Björn Nordlund, E. Rehbinder, C. Rueegg, H. Skjerven, A. C. Staff, C. Söderhäll, R. Vettukattil, K. C. Carlsen

{"title":"Maternal use of snus as smokeless tobacco in pregnancy and infant lung function","authors":"K. Bains, Ina Kreyberg, M. Färdig, B. Granum, H. Gudmundsdóttir, Guttorm Haugen, G. Hedlin, Katarina Hilde, C.M Jonassen, Björn Nordlund, E. Rehbinder, C. Rueegg, H. Skjerven, A. C. Staff, C. Söderhäll, R. Vettukattil, K. C. Carlsen","doi":"10.1183/23120541.00958-2023","DOIUrl":"https://doi.org/10.1183/23120541.00958-2023","url":null,"abstract":"Smoking in pregnancy has detrimental effects on infant respiratory health, while the effects of other nicotine-containing products on infant lung function are unclear. We aimed to explore if smokeless tobacco such as snus used in pregnancy increased the risk of lower lung function in infancy and if the associations differed by sex.From the Scandinavian population-based Preventing Atopic Dermatitis and ALLergies in Children birth cohort, we included 1163 infants with available tidal flow-volume measurements at three months of age and maternal self-reported use of nicotine-containing products in pregnancy. The risk of a ratio of time to peak tidal expiratory flow to total expiratory time <25th percentile by any nicotine exposure, snus exclusively and cigarette smoking with or without other nicotine-containing products, were explored by regression analyses adjusting for maternal age, education and asthma.Overall 120/1163 (10.3%) infants were exposed to any nicotine in-utero, 71/120 by snus exclusively and 49/120 by smoking, with six also exposed to snus. By pregnancy week six 85.8% of mothers reported stopping nicotine use. The risk of lower lung function was higher in children exposed in-utero to nicotine-containing products with an odds ratio (OR) of 1.63 (95% confidence interval 1.02,2.59) with a similar tendency for snus exclusively OR 1.55 (0.88,2.71) and smoking OR 1.79 (0.84,3.84). Effect estimates were similar after adjusting for covariates. No differences of the effect by sex was observed.Our study suggests that in-utero exposure to not only cigarettes, but also snus, may negatively affect infant lung function.","PeriodicalId":504874,"journal":{"name":"ERJ Open Research","volume":null,"pages":null},"PeriodicalIF":0.0,"publicationDate":"2024-04-11","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"140714299","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

引用次数: 0

Environmental, healthcare, and societal impacts of asthma: a UK model-based assessment 哮喘对环境、医疗保健和社会的影响：基于英国模型的评估

ERJ Open Research Pub Date : 2024-04-11 DOI: 10.1183/23120541.00577-2023

M. Orlovic, Dimitrios Tzelis, I. Guerra, Vladislava Bar-Katz, Nicholas Woolley, H. Bray, M. Hanslot, Usmani Omar, A. Madoni

{"title":"Environmental, healthcare, and societal impacts of asthma: a UK model-based assessment","authors":"M. Orlovic, Dimitrios Tzelis, I. Guerra, Vladislava Bar-Katz, Nicholas Woolley, H. Bray, M. Hanslot, Usmani Omar, A. Madoni","doi":"10.1183/23120541.00577-2023","DOIUrl":"https://doi.org/10.1183/23120541.00577-2023","url":null,"abstract":"This study aimed to assess the broader environmental, healthcare, and societal impacts across the entire asthma pathway from diagnosis to treatment, in the United Kingdom (UK).A comprehensive cost-of-Illness framework was developed considering the effects of the full asthma patient pathway, including greenhouse gas (GHG) emissions generated from inhalers, National Health Service (NHS) costs, health-related quality of life (HRQoL), and productivity losses. The model was based on published literature and clinical expert opinion to accurately estimate, in monetary terms, the net present value (NPV) of the asthma pathway impacts for 2022–31.The estimated NPV of the environmental, healthcare, and societal impacts of the asthma pathway was £47 billion over the 2022–31 period in the UK. Loss of disease control was a key contributor to higher GHG emissions and NHS costs. In 2022, a non-severe uncontrolled patient was estimated to incur 22% higher NHS costs than a controlled patient, whilst generating 0.1 tonnes (t) more of CO2equivalent (CO2e) emissions. In the same year, the total direct impacts per severe patient was four times higher than a non-severe controlled patient, with 0.54t CO2e of GHG emissions. Moreover, as much as 77% of the total economic impact was driven from HRQoL worsening and productivity impacts occurring when patients’ symptoms are uncontrolled.Uncontrolled asthma significantly impacts the economy, environment, and patients in the UK. Hence, our results emphasize the need for a holistic approach in controlling asthma and might be carefully considered when developing policies to mitigate the overall burden of the disease.","PeriodicalId":504874,"journal":{"name":"ERJ Open Research","volume":null,"pages":null},"PeriodicalIF":0.0,"publicationDate":"2024-04-11","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"140713925","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

引用次数: 0

Beyond T2-asthma biomarkers: risk stratification for NSAID-Exacerbated Respiratory Disease 超越 T2-哮喘生物标志物：非甾体抗炎药致呼吸道疾病的风险分层

ERJ Open Research Pub Date : 2024-04-11 DOI: 10.1183/23120541.00909-2023

Jacqueline Pérez-Pazos, A. García-Sánchez, Miguel Estravís, Emma Moreno-Jimenez, Natalia Morgado, Manuel Gómez-García, Jacinto Ramos-González, M. Gil-Melcón, C. Martín-García, F. Muñoz-Bellido, C. Sanz, M. Isidoro-García, Ignacio Dávila

{"title":"Beyond T2-asthma biomarkers: risk stratification for NSAID-Exacerbated Respiratory Disease","authors":"Jacqueline Pérez-Pazos, A. García-Sánchez, Miguel Estravís, Emma Moreno-Jimenez, Natalia Morgado, Manuel Gómez-García, Jacinto Ramos-González, M. Gil-Melcón, C. Martín-García, F. Muñoz-Bellido, C. Sanz, M. Isidoro-García, Ignacio Dávila","doi":"10.1183/23120541.00909-2023","DOIUrl":"https://doi.org/10.1183/23120541.00909-2023","url":null,"abstract":"Type 2 (T2)-asthma is often associated with chronic rhinosinusitis with nasal polyposis (CRSwNP). Additionally, non-steroidal anti-inflammatory drug intolerance leads to NSAID-exacerbated respiratory disease (N-ERD). Previous transcriptomic data in non-CRSwNP T2-asthma patients showed differentially expressed genes (DEGs). Of them, we focused onALOX15,CLC,CYSLTR2,HRH4, andSMPD3to investigate their role in T2-asthma patients. The study included 100 healthy controls (HCs) and 103T2-asthma patients, divided into asthmatics (54), asthmatics with CRSwNP (30), and N-ERD (19). Quantitative PCR analysis was performed on blood-derived RNA samples first to validate the five DEGs. The data were further analyzed to find potential associations and biomarkers. Patients, regardless of stratification, exhibited significantly higher gene expressions than HCs. The patterns of association revealed thatALOX15was exclusively present in the non-comorbidity group,SMPD3andCLCin the comorbidity groups, andHRH4in all patient groups.ALOX15, CYSLTR2, andSMPD3expression showed potential as biomarkers to confirm the diagnosis of T2-asthma using peripheral blood eosinophils (PBE) as the initial criterion. PBE combined with gene expressions, especiallySMPD3, may improve the diagnosis.CLCandCYSLTR2expressions play a specific role in discriminating N-ERD. We validated the transcriptomic data of five DEGs in T2-asthma. Different patterns of association were identified in patient stratification, suggesting different molecular mechanisms underlying the spectrum of T2-asthma. Potential biomarkers were also found and used to design an algorithm with practical diagnostic utility for T2-asthma, including risk stratification for N-ERD.","PeriodicalId":504874,"journal":{"name":"ERJ Open Research","volume":null,"pages":null},"PeriodicalIF":0.0,"publicationDate":"2024-04-11","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"140712672","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

引用次数: 0

Unmet need in PH-ILD: A clinician survey of real-world management of PH-ILD in Europe 未满足的 PH-ILD 需求：欧洲 PH-ILD 实际管理情况临床医生调查

ERJ Open Research Pub Date : 2024-04-11 DOI: 10.1183/23120541.00039-2024

D. Montani, José M. Cifrián, Raquel Rojo, Hilario Nunes, Federica Meloni, Stefano Ghio, John Cannon, Andreas Günther, Héctor Gálvez García, Miriam F. Delgado, Gabriela B. Jeanneret, Luke S Howard

{"title":"Unmet need in PH-ILD: A clinician survey of real-world management of PH-ILD in Europe","authors":"D. Montani, José M. Cifrián, Raquel Rojo, Hilario Nunes, Federica Meloni, Stefano Ghio, John Cannon, Andreas Günther, Héctor Gálvez García, Miriam F. Delgado, Gabriela B. Jeanneret, Luke S Howard","doi":"10.1183/23120541.00039-2024","DOIUrl":"https://doi.org/10.1183/23120541.00039-2024","url":null,"abstract":"With no approved therapies for pulmonary hypertension (PH) associated with interstitial lung disease (PH-ILD) in Europe, we surveyed clinician perceptions on PH-ILD management and unmet need to understand current real-world practices.: An online clinician survey on PH-ILD management was conducted in France, Germany, Italy, Spain, and the UK.: Fifty-five clinicians (78% pulmonologists) each managing a median 20 PH-ILD patients (interquartile range [IQR]=10–50), participated. Upon PH suspicion, clinicians referred a median 50% (IQR=20–73%) of patients for echocardiography alone and 35% (IQR=20–78%) for echocardiography, followed by right heart catheterisation.Upon diagnosis, a median 20% (IQR=9–30%), 40% (IQR=20–50%) and 35% (IQR=20–55%) of patients fell under the pulmonary arterial pressure ranges of 21–24mmHg, 25–34mmHg and >35mmHg respectively. Fifty percent of patients received off-label treatment for their PH and, of those, off-label phosphodiesterase-5 inhibitor (PDE-5i), endothelin receptor antagonist (ERA) and prostacyclin analogues were prescribed first-line by 78%, 9% and 7% of clinicians, respectively. Upon PDE-5i non-response, 35% of clinicians proceed with an ERA, 35% with no further therapy. Fifty-five percent of clinicians used dual therapy. Yearly median inpatient admissions and emergency visits were 2.0 (IQR=1.3–2.9) and 1.5 (IQR=1.0–2.0) respectively (n=31 responses). Most clinicians (69%) highlighted lack of efficacy or evidence for current therapies as a key gap in PH-ILD management.This study gives insight into real-world European PH-ILD diagnosis and management. With significant use of off-label treatment, there is a large unmet need due to lack of approved therapies. Despite updated guidelines, more evidence is needed to standardise PH-ILD management.","PeriodicalId":504874,"journal":{"name":"ERJ Open Research","volume":null,"pages":null},"PeriodicalIF":0.0,"publicationDate":"2024-04-11","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"140714477","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

引用次数: 0

Genomic Epidemiology ofMycobacterium abscessusat an Adult Cystic Fibrosis Program Reveals Low Potential for Healthcare-Associated Transmission 成人囊性纤维化项目中脓肿分枝杆菌的基因组流行病学研究显示，医疗保健相关传播的可能性较低

ERJ Open Research Pub Date : 2024-04-11 DOI: 10.1183/23120541.00165-2024

J.E. Gross, James D. Finklea, S. Caceres, K. Poch, N. Hasan, Fan Jia, L. Epperson, Ettie M. Lipner, C. Vang, J.R. Honda, Matthew Strand, Vinicius Calado Nogueira de Moura, Charles L. Daley, M. Strong, Jerry A. Nick

{"title":"Genomic Epidemiology ofMycobacterium abscessusat an Adult Cystic Fibrosis Program Reveals Low Potential for Healthcare-Associated Transmission","authors":"J.E. Gross, James D. Finklea, S. Caceres, K. Poch, N. Hasan, Fan Jia, L. Epperson, Ettie M. Lipner, C. Vang, J.R. Honda, Matthew Strand, Vinicius Calado Nogueira de Moura, Charles L. Daley, M. Strong, Jerry A. Nick","doi":"10.1183/23120541.00165-2024","DOIUrl":"https://doi.org/10.1183/23120541.00165-2024","url":null,"abstract":"Nontuberculous mycobacteria (NTM) has been reported to be transmitted between people with cystic fibrosis (pwCF) attending Cystic Fibrosis (CF) Centers. A suspectedMycobacterium abscessusoutbreak was investigated at the University of Texas Southwestern (UTSW) Adult CF Program using a combination of pathogen genomic sequencing and epidemiologic methods.Apply the Healthcare-associated Links in Transmission of NTM (HALT NTM) study to investigate the occurrence of potential healthcare-associated transmission and/or acquisition of NTM among pwCF infected with genetically similar NTM isolates.Whole genome sequencing (WGS) of respiratoryM. abscessusisolates from 50 pwCF receiving care at UTSW was performed to identify genetically similar isolates. Epidemiological investigation, comparison of respiratory and environmental isolates, and home residence watershed mapping were studied.WGS analysis demonstrated seven clusters of genetically similarM. abscessus(four ssp. abscessusand three ssp.massiliense). Epidemiologic investigation revealed potential opportunities for healthcare-associated transmission within three of these clusters. Healthcare environmental sampling did not recoverM. abscessus, but did recover four human disease-causing species of NTM. No subjects having clustered infections lived in the same home residence watershed. Some subjects were infected with more than oneM. abscessusgenotype, both within and outside of the dominant circulating clones.Healthcare-associated patient-to-patient transmission ofM. abscessusappears rare at this Center. However, polyclonal infections ofM. abscessusspecies and subspecies, not originating from the endemic hospital environment, suggests multiple shared modes of acquisition outside the healthcare setting.","PeriodicalId":504874,"journal":{"name":"ERJ Open Research","volume":null,"pages":null},"PeriodicalIF":0.0,"publicationDate":"2024-04-11","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"140714430","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

引用次数: 0