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Journal club 期刊俱乐部
IF 1 1区 医学
Thorax Pub Date : 2024-10-01 DOI: 10.1136/thorax-2024-222174
Ewan Christopher Mackay
{"title":"Journal club","authors":"Ewan Christopher Mackay","doi":"10.1136/thorax-2024-222174","DOIUrl":"https://doi.org/10.1136/thorax-2024-222174","url":null,"abstract":"Household air pollution has been estimated to be responsible for 3.2 million preventable deaths every year globally. With biomass exposure and environmental pollution linked to exacerbations of airways disease, this health impact disproportionately affects low and middle income countries. Puzzolo et al ( Lancet Resp Med 2024;12(4):281–293) undertook a systematic review and included 116 studies in the subsequent meta-analyses, to compare use of gaseous fuels in the domestic environment with more polluting fuels (wood/charcoal/kerosene) and cleaner fuels (electricity/solar) with no point of use pollution. Use of gas significantly decreased the risk of COPD (OR 0·37, 95%CI 0·23–0·60; p<0·0001), pneumonia (OR 0·54, 0·38–0·77; p=0·0008), deficits in lung function (OR 0·27, 0·17–0·44; p<0·0001), severe respiratory illness or death (OR 0·27, 0·11–0·63; p=0·0024) compared with more polluting fuels. Preterm births (OR 0·66, 0·45–0·97; p=0·033), and low birth weights were similarly reduced (OR 0·70, 0·53–0·93; p=0·015). Risk of asthma did not reach statistical significance. Gas compared with electricity did increase risk of COPD (OR 1·15, 1·06–1·25; p=0·0011) and pneumonia (OR 1·26, 1·03–1·53; p=0·025) but this was not significant in all studies. While having its own health and environmental impacts, switching to gas from more polluting fuels may reduce the burden of health risk in countries without …","PeriodicalId":23284,"journal":{"name":"Thorax","volume":null,"pages":null},"PeriodicalIF":10.0,"publicationDate":"2024-10-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"142236635","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":1,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
引用次数: 0
Harm from tobacco: a common thread 烟草危害:一个共同点
IF 1 1区 医学
Thorax Pub Date : 2024-09-11 DOI: 10.1136/thorax-2024-222191
Sanjay Agrawal
{"title":"Harm from tobacco: a common thread","authors":"Sanjay Agrawal","doi":"10.1136/thorax-2024-222191","DOIUrl":"https://doi.org/10.1136/thorax-2024-222191","url":null,"abstract":"Industry uses market segmentation of products to attract and retain consumers from a variety of groups based on demographics, interests, behavioural factors and common needs. This approach is used across many commercial sectors selling a diverse range of commodities including cleaning, beauty, food, automobile and electronic products. Consumers may be attracted to goods based on a number of characteristics including price, packaging, advertising or ease of use. There may also be a perception among consumers that some products are ‘safer’ to use or consume, perhaps based on frequency of use, appearance, marketing and peer influencers.1 The tobacco industry has been adept at using market segmentation across its tobacco range, for example, evoking emotion and identity by differentiating cigars (tobacco wrapped in tobacco leaf, eg celebration, Winston Churchill), pipe tobacco (thicker tobacco strands, eg, concentration, Sherlock Holmes) and cigarettes (fine ground tobacco, eg, relaxation—music and film celebrities).2 Other examples of market segmentation deployed by the tobacco industry include; filters to imply safety3; menthol flavourings to attract young people and specific ethnic groups …","PeriodicalId":23284,"journal":{"name":"Thorax","volume":null,"pages":null},"PeriodicalIF":10.0,"publicationDate":"2024-09-11","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"142170895","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":1,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
引用次数: 0
Pulmonary fibrosis and lung cancer: an analysis of the Clinical Practice Research Datalink linked to the National Cancer Registration Dataset 肺纤维化与肺癌:与全国癌症登记数据集链接的临床实践研究数据链分析
IF 1 1区 医学
Thorax Pub Date : 2024-09-10 DOI: 10.1136/thorax-2024-221865
Francesca Gonnelli, Jaspreet Kaur, Martina Bonifazi, David Baldwin, Emma O’Dowd, Richard Hubbard
{"title":"Pulmonary fibrosis and lung cancer: an analysis of the Clinical Practice Research Datalink linked to the National Cancer Registration Dataset","authors":"Francesca Gonnelli, Jaspreet Kaur, Martina Bonifazi, David Baldwin, Emma O’Dowd, Richard Hubbard","doi":"10.1136/thorax-2024-221865","DOIUrl":"https://doi.org/10.1136/thorax-2024-221865","url":null,"abstract":"We quantified the proportion of diagnoses of pulmonary fibrosis (PF) among 25 136 people with lung cancer and 250 583 matched controls and compared the natural history of lung cancer in people with and without PF. Diagnoses of PF were more common in people with lung cancer than those without (1.5% vs 0.8%, OR 1.97; 95% CI 1.77 to 2.21). Within people with PF, squamous cell carcinoma was more (22.9% vs 19.1%), and adenocarcinoma was less common (18.0% vs 21.3%). People with PF were less likely to have stage 4 disease at diagnosis (OR 0.43, 95% CI 0.28 to 0.65) but their survival was worse. Data may be obtained from a third party and are not publicly available. Data are available through CPRD and NCRAS.","PeriodicalId":23284,"journal":{"name":"Thorax","volume":null,"pages":null},"PeriodicalIF":10.0,"publicationDate":"2024-09-10","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"142166278","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":1,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
引用次数: 0
Journal club 期刊俱乐部
IF 1 1区 医学
Thorax Pub Date : 2024-09-01 DOI: 10.1136/thorax-2024-222173
Marta Duarte-Silva
{"title":"Journal club","authors":"Marta Duarte-Silva","doi":"10.1136/thorax-2024-222173","DOIUrl":"https://doi.org/10.1136/thorax-2024-222173","url":null,"abstract":"Chronic obstructive pulmonary disease (COPD) is characterised by reduced lung function and frequent exacerbations, which can worsen symptoms, increasing the risk of further health decline and mortality. Although COPD has long been recognised as involving an amplified innate immune response, recent findings suggest that type two inflammation, present in 20–40% of COPD patients, could be a therapeutic target. The BOREAS trial ( N Engl J Med 2023;389:205–14) investigated the efficacy and safety of dupilumab, a monoclonal antibody that inhibits interleukin-4 and interleukin-13, in reducing exacerbations and improving lung function in COPD patients with type two inflammation. The phase 3, multicenter, double-blind, placebo-controlled trial enrolled 939 COPD patients with blood eosinophil count of at least 300 per microliter, and a high exacerbation risk despite standard triple therapy. Participants were randomised to receive either 300 mg of dupilumab or placebo biweekly for 52 weeks, alongside their standard inhaled therapies. The primary endpoint was the annualised rate of moderate or severe COPD exacerbations. Dupilumab significantly reduced the annualised rate of moderate or severe exacerbations compared with placebo (0.78 vs 1.10; rate ratio, 0.70; 95% CI, 0.58 to 0.86; p<0.001). Additionally, patients treated with dupilumab showed greater improvements in …","PeriodicalId":23284,"journal":{"name":"Thorax","volume":null,"pages":null},"PeriodicalIF":10.0,"publicationDate":"2024-09-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"142007529","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":1,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
引用次数: 0
Consolidation with pseudocavitation 伪气蚀加固
IF 1 1区 医学
Thorax Pub Date : 2024-08-30 DOI: 10.1136/thorax-2024-222040
Tanzil Rahaman, Kavitha Venkatnarayan, Chitra Veluthat, Priya Ramachandran
{"title":"Consolidation with pseudocavitation","authors":"Tanzil Rahaman, Kavitha Venkatnarayan, Chitra Veluthat, Priya Ramachandran","doi":"10.1136/thorax-2024-222040","DOIUrl":"https://doi.org/10.1136/thorax-2024-222040","url":null,"abstract":"A 48-year-old man, non-smoker, with no known comorbidities, presented with shortness of breath, cough with mucoid expectoration, loss of appetite and loss of weight for 6 weeks. There was no history of fever, hemoptysis or wheezing. He had received multiple courses of antibiotics from elsewhere before presenting to our centre with no relief of symptoms. On examination, crepitations were audible in the right infrascapular region. Examination of other organ systems and lab investigations were unremarkable. Chest radiograph showed a right lower zone consolidation. CT of the thorax showed right lower lobe consolidation with ground glass opacities with evidence of pseudocavitation. There were multiple nodules with pseudocavity in the other lobes and opposite lung as well (figure 1). With a clinical and radiological suspicion of …","PeriodicalId":23284,"journal":{"name":"Thorax","volume":null,"pages":null},"PeriodicalIF":10.0,"publicationDate":"2024-08-30","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"142100859","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":1,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
引用次数: 0
Impact of TAS2R38 polymorphisms on nasal nitric oxide and Pseudomonas infections in primary ciliary dyskinesia: relation to genotype TAS2R38多态性对原发性睫状肌运动障碍患者鼻腔一氧化氮和假单胞菌感染的影响:与基因型的关系
IF 1 1区 医学
Thorax Pub Date : 2024-08-24 DOI: 10.1136/thorax-2024-221396
Massimo Pifferi, Attilio Boner, Debora Maj, Angela Michelucci, Gabriele Donzelli, Angela M Cangiotti, Raffaella Guazzo, Giulia Bertolucci, Veronica Bertini, Chiara Doccioli, Michele Piazza, Angelo Valetto, Maria Adelaide Caligo, Diego Peroni, Andrew Bush
{"title":"Impact of TAS2R38 polymorphisms on nasal nitric oxide and Pseudomonas infections in primary ciliary dyskinesia: relation to genotype","authors":"Massimo Pifferi, Attilio Boner, Debora Maj, Angela Michelucci, Gabriele Donzelli, Angela M Cangiotti, Raffaella Guazzo, Giulia Bertolucci, Veronica Bertini, Chiara Doccioli, Michele Piazza, Angelo Valetto, Maria Adelaide Caligo, Diego Peroni, Andrew Bush","doi":"10.1136/thorax-2024-221396","DOIUrl":"https://doi.org/10.1136/thorax-2024-221396","url":null,"abstract":"Objective Primary ciliary dyskinesia (PCD) severity has been related to genotype and levels of nasal nitric oxide (nNO). The most common TAS2R38 haplotypes (PAV/PAV, PAV/AVI, AVI/AVI) encoding the bitter taste receptor can affect nNO levels and thus could play a role in the susceptibility to respiratory infections. We assessed the impact of these polymorphisms on nNO production and Pseudomonas aeruginosa ( P.a .) infections in different PCD genotypes. Methods Prospective, longitudinal, single-centre study in patients with PCD with known genotype and one of three TAS2R38 haplotypes evaluated for up to 10 years. We related nNO values to TAS2R38 haplotypes in all patients, and in the three most frequent genotypes ( CCDC39/CCDC40 , DNAH5 , DNAH11 ). In the genetic group(s) with different mean trends of nNO in relation to the polymorphism, we evaluated longitudinal lung function as a clinical outcome measure. We also studied any associations between the prevalence of chronic P.a . infection and PAV alleles. Linear mixed-effects models were used to evaluate longitudinal associations. Results 119 patients with PCD underwent 1116 study visits. Only in the DNAH11 mutations group was there a mean trend of nNO production which was significantly higher in PAV/PAV than AVI/AVI haplotype (p=0.033), with a better trend in spirometric and plethysmographic parameters. In patients with DNAH11 mutations the PAV allele was also associated with a significantly reduced prevalence of chronic P.a . infection. Conclusion TAS2R38 may be a modifier gene for PCD severity, but only in mild phenotype disease. Further study of TAS2R38 polymorphisms might enable new management strategies to prevent chronic P.a . infections. Data are available upon reasonable request. De-identified participant data are available from the corresponding author upon reasonable request, subject to the terms of Ethics Committee approval.","PeriodicalId":23284,"journal":{"name":"Thorax","volume":null,"pages":null},"PeriodicalIF":10.0,"publicationDate":"2024-08-24","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"142050632","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":1,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
引用次数: 0
Atypical radiological manifestation of sarcoidosis presenting with an anterior mediastinal mass. 肉样瘤病的非典型放射学表现,表现为纵隔前部肿块。
IF 9 1区 医学
Thorax Pub Date : 2024-08-22 DOI: 10.1136/thorax-2024-221604
Wan-Ting Tao, Hao-Yu Huang, Wen-Chiuan Tsai, Kai-Hsiung Ko
{"title":"Atypical radiological manifestation of sarcoidosis presenting with an anterior mediastinal mass.","authors":"Wan-Ting Tao, Hao-Yu Huang, Wen-Chiuan Tsai, Kai-Hsiung Ko","doi":"10.1136/thorax-2024-221604","DOIUrl":"https://doi.org/10.1136/thorax-2024-221604","url":null,"abstract":"","PeriodicalId":23284,"journal":{"name":"Thorax","volume":null,"pages":null},"PeriodicalIF":9.0,"publicationDate":"2024-08-22","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"142037120","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":1,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
引用次数: 0
Consensus palliative care referral criteria for people with chronic obstructive pulmonary disease. 慢性阻塞性肺病患者姑息关怀转诊标准共识。
IF 9 1区 医学
Thorax Pub Date : 2024-08-22 DOI: 10.1136/thorax-2024-221721
Jennifer Philip, Yuchieh Kathryn Chang, Anna Collins, Natasha Smallwood, Donald Richard Sullivan, Barbara P Yawn, Richard Mularski, Magnus Ekström, Ian A Yang, Christine F McDonald, Masanori Mori, Pedro Perez-Cruz, David M G Halpin, Shao-Yi Cheng, David Hui
{"title":"Consensus palliative care referral criteria for people with chronic obstructive pulmonary disease.","authors":"Jennifer Philip, Yuchieh Kathryn Chang, Anna Collins, Natasha Smallwood, Donald Richard Sullivan, Barbara P Yawn, Richard Mularski, Magnus Ekström, Ian A Yang, Christine F McDonald, Masanori Mori, Pedro Perez-Cruz, David M G Halpin, Shao-Yi Cheng, David Hui","doi":"10.1136/thorax-2024-221721","DOIUrl":"https://doi.org/10.1136/thorax-2024-221721","url":null,"abstract":"<p><strong>Objective: </strong>People with advanced chronic obstructive pulmonary disease (COPD) have substantial palliative care needs, but uncertainty exists around appropriate identification of patients for palliative care referral.We conducted a Delphi study of international experts to identify consensus referral criteria for specialist outpatient palliative care for people with COPD.</p><p><strong>Methods: </strong>Clinicians in the fields of respiratory medicine, palliative and primary care from five continents with expertise in respiratory medicine and palliative care rated 81 criteria over three Delphi rounds. Consensus was defined a priori as ≥70% agreement. A criterion was considered 'major' if experts endorsed meeting that criterion alone justified palliative care referral.</p><p><strong>Results: </strong>Response rates from the 57 panellists were 86% (49), 84% (48) and 91% (52) over first, second and third rounds, respectively. Panellists reached consensus on 17 major criteria for specialist outpatient palliative care referral, categorised under: (1) 'Health service use and need for advanced respiratory therapies' (six criteria, eg, need for home non-invasive ventilation); (2) 'Presence of symptoms, psychosocial and decision-making needs' (eight criteria, eg, severe (7-10 on a 10 point scale) chronic breathlessness); and (3) 'Prognostic estimate and performance status' (three criteria, eg, physician-estimated life expectancy of 6 months or less).</p><p><strong>Conclusions: </strong>International experts evaluated 81 potential referral criteria, reaching consensus on 17 major criteria for referral to specialist outpatient palliative care for people with COPD. Evaluation of the feasibility of these criteria in practice is required to improve standardised palliative care delivery for people with COPD.</p>","PeriodicalId":23284,"journal":{"name":"Thorax","volume":null,"pages":null},"PeriodicalIF":9.0,"publicationDate":"2024-08-22","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"142037121","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":1,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
引用次数: 0
Indirect impact of childhood 13-valent pneumococcal conjugate vaccine (PCV13) in Canadian older adults: a Canadian Immunization Research Network (CIRN) retrospective observational study. 儿童 13 价肺炎球菌结合疫苗 (PCV13) 对加拿大老年人的间接影响:加拿大免疫研究网络 (CIRN) 的一项回顾性观察研究。
IF 9 1区 医学
Thorax Pub Date : 2024-08-19 DOI: 10.1136/thorax-2023-220377
Sharifa Nasreen, Jun Wang, Fawziah Marra, Jeffrey C Kwong, Allison McGeer, Manish Sadarangani, Sarah E Wilson, Shaza A Fadel
{"title":"Indirect impact of childhood 13-valent pneumococcal conjugate vaccine (PCV13) in Canadian older adults: a Canadian Immunization Research Network (CIRN) retrospective observational study.","authors":"Sharifa Nasreen, Jun Wang, Fawziah Marra, Jeffrey C Kwong, Allison McGeer, Manish Sadarangani, Sarah E Wilson, Shaza A Fadel","doi":"10.1136/thorax-2023-220377","DOIUrl":"10.1136/thorax-2023-220377","url":null,"abstract":"<p><strong>Background: </strong>13-valent pneumococcal conjugate vaccine (PCV13) has been part of publicly funded childhood immunisation programmes in Ontario and British Columbia (BC) since 2010. We assessed the indirect impact of infant PCV13 programmes on invasive pneumococcal disease (IPD) and all-cause pneumonia hospitalisation in older adults (aged ≥65 years) using a retrospective observational study.</p><p><strong>Methods: </strong>We extracted monthly IPD and all-cause pneumonia cases from laboratory and health administrative databases between January 2005 and December 2018. Using a quasi-experimental difference-in-differences design, we calculated the ratio of risk ratios (RRRs) using incidence rates of IPD or all-cause pneumonia cases before (pre-PCV13 period) and after (PCV13 period) 2010 with rates of fractures as controls.</p><p><strong>Results: </strong>The rates of all IPD or PCV serotype-specific IPD for older adults in both Ontario and BC did not change in 8 years after childhood PCV13 programme implementation. All-cause pneumonia increased in Ontario (RRR 1.38, 95% CI 1.11 to 1.71) but remained unchanged in BC.</p><p><strong>Conclusions: </strong>Indirect community protection of older adults from hospitalisation with pneumococcal disease stalled despite maturation of childhood PCV13 vaccination programmes in two Canadian provinces.</p>","PeriodicalId":23284,"journal":{"name":"Thorax","volume":null,"pages":null},"PeriodicalIF":9.0,"publicationDate":"2024-08-19","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC11347212/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"139742100","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":1,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
引用次数: 0
Epidemiology of childhood interstitial lung disease in France: the RespiRare cohort. 法国儿童间质性肺病流行病学:RespiRare 队列。
IF 9 1区 医学
Thorax Pub Date : 2024-08-19 DOI: 10.1136/thorax-2023-221325
Camille Fletcher, Alice Hadchouel, Caroline Thumerelle, Julie Mazenq, Manon Fleury, Harriet Corvol, Nouha Jedidi, Myriam Benhamida, Katia Bessaci, Tiphaine Bilhouee, Raphael Borie, Jacques Brouard, Aurélie Cantais, Annick Clement, Laurianne Coutier, Camille Cisterne, Pierrick Cros, Marie-Laure Dalphin, Christophe Delacourt, Eric Deneuville, Jean-Christophe Dubus, Carole Egron, Ralph Epaud, Michael Fayon, Aude Forgeron, Elsa Gachelin, François Galode, Isabelle Gertini, Lisa Giovannini-Chami, Pierre Gourdan, Tamazoust Guiddir, Audrey Herzog, Véronique Houdouin, Églantine Hullo, Pierre-Henri Jarreau, Guillame Labbé, Géraldine Labouret, Alice Ladaurade, Laurence Le Clainche Viala, Christophe Marguet, Alexandra Masson-Rouchaud, Caroline Perisson, Cinthia Rames, Philippe Reix, Marie-Catherine Renoux, Léa Roditis, Cyril Schweitzer, Aurélie Tatopoulos, Pascale Trioche-Eberschweiler, Françoise Troussier, Clémentine Vigier, Laurence Weiss, Marie Legendre, Camille Louvrier, Alix de Becdelievre, Aurore Coulomb, Chiara Sileo, Hubert Ducou le Pointe, Laureline Berteloot, Céline Delestrain, Nadia Nathan
{"title":"Epidemiology of childhood interstitial lung disease in France: the RespiRare cohort.","authors":"Camille Fletcher, Alice Hadchouel, Caroline Thumerelle, Julie Mazenq, Manon Fleury, Harriet Corvol, Nouha Jedidi, Myriam Benhamida, Katia Bessaci, Tiphaine Bilhouee, Raphael Borie, Jacques Brouard, Aurélie Cantais, Annick Clement, Laurianne Coutier, Camille Cisterne, Pierrick Cros, Marie-Laure Dalphin, Christophe Delacourt, Eric Deneuville, Jean-Christophe Dubus, Carole Egron, Ralph Epaud, Michael Fayon, Aude Forgeron, Elsa Gachelin, François Galode, Isabelle Gertini, Lisa Giovannini-Chami, Pierre Gourdan, Tamazoust Guiddir, Audrey Herzog, Véronique Houdouin, Églantine Hullo, Pierre-Henri Jarreau, Guillame Labbé, Géraldine Labouret, Alice Ladaurade, Laurence Le Clainche Viala, Christophe Marguet, Alexandra Masson-Rouchaud, Caroline Perisson, Cinthia Rames, Philippe Reix, Marie-Catherine Renoux, Léa Roditis, Cyril Schweitzer, Aurélie Tatopoulos, Pascale Trioche-Eberschweiler, Françoise Troussier, Clémentine Vigier, Laurence Weiss, Marie Legendre, Camille Louvrier, Alix de Becdelievre, Aurore Coulomb, Chiara Sileo, Hubert Ducou le Pointe, Laureline Berteloot, Céline Delestrain, Nadia Nathan","doi":"10.1136/thorax-2023-221325","DOIUrl":"10.1136/thorax-2023-221325","url":null,"abstract":"<p><strong>Introduction: </strong>Interstitial lung disease in children (chILD) are rare and mostly severe lung diseases. Very few epidemiological data are available in limited series of patients. The aim of this study was to assess the prevalence and incidence of chILD in France.</p><p><strong>Methods: </strong>We performed within the RespiRare network a multicentre retrospective observational study in patients with chILD from 2000 to 2022 and a prospective evaluation of chILD's incidence between February 2022 and 2023.</p><p><strong>Results: </strong>chILD was reported in 790 patients in 42 centres. The estimated 2022 prevalence in France was 44 /million children (95% CI 40.76 to 47.46) and the computed incidence was 4.4 /million children (95% CI 3.44 to 5.56). The median age at diagnosis was 3 months with 16.9% of familial forms. Lung biopsy and genetic analyses were performed in 23.4% and 76.9%, respectively. The most frequent chILD aetiologies in the <2 years group were surfactant metabolism disorders (16.3%) and neuroendocrine cell hyperplasia of infancy (11.8%), and in the 2-18 years group diffuse alveolar haemorrhage (12.2%), connective tissue diseases (11.4%), hypersensitivity pneumonitis (8.8%) and sarcoidosis (8.8%). The management included mainly oxygen therapy (52%), corticosteroid pulses (56%), oral corticosteroids (44%), azithromycin (27.2%), enteral nutrition (26.9%), immunosuppressants (20.3%) and hydroxychloroquine (15.9%). The 5-year survival rate was 57.3% for the patients diagnosed before 2 years and 86% between 2 and 18 years.</p><p><strong>Conclusion: </strong>This large and systematic epidemiological study confirms a higher incidence and prevalence of chILD than previously described. In order to develop international studies, efforts are still needed to optimise the case collection and to harmonise diagnostic and management practices.</p>","PeriodicalId":23284,"journal":{"name":"Thorax","volume":null,"pages":null},"PeriodicalIF":9.0,"publicationDate":"2024-08-19","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"141535396","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":1,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
引用次数: 0
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