The European respiratory journal. Supplement最新文献

{"title":"Bilateral hypoglossal nerve stimulation for treatment of adult obstructive sleep apnoea","authors":"P. Eastwood, M. Barnes, S. MacKay, J. Wheatley, D. Hillman, Xuân-Lan Nguyên, R. Lewis, M. Campbell, B. Pételle, J. Walsh, A. Jones, C. Palme, A. Bizon, N. Meslier, C. Bertolus, K. Maddison, L. Laccourreye, G. Raux, K. Denoncin, V. Attali, F. Gagnadoux, S. Launois","doi":"10.1183/13993003.01320-2019","DOIUrl":"https://doi.org/10.1183/13993003.01320-2019","url":null,"abstract":"Background and aim Hypoglossal nerve stimulation (HNS) decreases obstructive sleep apnoea (OSA) severity via genioglossus muscle activation and decreased upper airway collapsibility. This study assessed the safety and effectiveness at 6 months post-implantation of a novel device delivering bilateral HNS via a small implanted electrode activated by a unit worn externally, to treat OSA: the Genio™ system. Methods This prospective, open-label, non-randomised, single-arm treatment study was conducted at eight centres in three countries (Australia, France and the UK). Primary outcomes were incidence of device-related serious adverse events and change in the apnoea–hypopnoea index (AHI). The secondary outcome was the change in the 4% oxygen desaturation index (ODI). Additional outcomes included measures of sleepiness, quality of life, snoring and device use. This trial was registered with ClinicalTrials.gov, number NCT03048604. Results 22 out of 27 implanted participants (63% male, aged 55.9±12.0 years, body mass index (BMI) 27.4±3.0 kg·m−2) completed the protocol. At 6 months BMI was unchanged (p=0.85); AHI decreased from 23.7±12.2 to 12.9±10.1 events·h−1, a mean change of 10.8 events·h−1 (p<0.001); and ODI decreased from 19.1±11.2 to 9.8±6.9 events·h−1, a mean change of 9.3 events·h−1 (p<0.001). Daytime sleepiness (Epworth Sleepiness Scale; p=0.01) and sleep-related quality of life (Functional Outcomes of Sleep Questionnaire-10; p=0.02) both improved significantly. The number of bed partners reporting loud, very intense snoring, or leaving the bedroom due to participant snoring decreased from 96% to 35%. 91% of participants reported device use >5 days per week, and 77% reported use for >5 h per night. No device-related serious adverse events occurred during the 6-month post-implantation period. Conclusions Bilateral HNS using the Genio™ system reduces OSA severity and improves quality of life without device-related complications. The results are comparable with previously published HNS systems despite minimal implanted components and a simple stimulation algorithm. A new method of hypoglossal nerve stimulation to treat sleep apnoea does so bilaterally via an implanted neurostimulator activated externally. Its simplicity and relative non-invasiveness have not compromised its effectiveness relative to older methods. http://bit.ly/2lDCeif","PeriodicalId":77419,"journal":{"name":"The European respiratory journal. Supplement","volume":"17 1","pages":""},"PeriodicalIF":0.0,"publicationDate":"2019-10-10","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"81946629","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}

{"title":"A systematic review of comorbidities and outcomes of adult patients with pleural infection.","authors":"Tamsin N Cargill, Maged Hassan, John P Corcoran, Elinor Harriss, Rachelle Asciak, Rachel M Mercer, David J McCracken, Eihab O Bedawi, Najib M Rahman","doi":"10.1183/13993003.00541-2019","DOIUrl":"10.1183/13993003.00541-2019","url":null,"abstract":"<p><strong>Background: </strong>Pleural infection remains an important cause of mortality. This study aimed to investigate worldwide patterns of pre-existing comorbidities and clinical outcomes of patients with pleural infection.</p><p><strong>Methods: </strong>Studies reporting on adults with pleural infection between 2000 and 2017 were identified from a search of Embase and MEDLINE. Articles reporting exclusively on tuberculous, fungal or post-pneumonectomy infection were excluded. Two reviewers assessed 20 980 records for eligibility.</p><p><strong>Results: </strong>211 studies met the inclusion criteria. 134 articles (227 898 patients, mean age 52.8 years) reported comorbidity and/or outcome data. The majority of studies were retrospective observational cohorts (n=104, 78%) and the most common region of reporting was East Asia (n=33, 24%) followed by North America (n=27, 20%). 85 articles (50 756 patients) reported comorbidity. The median (interquartile range (IQR)) percentage prevalence of any comorbidity was 72% (58-83%), with respiratory illness (20%, 16-32%) and cardiac illness (19%, 15-27%) most commonly reported. 125 papers (192 298 patients) reported outcome data. The median (IQR) length of stay was 19 days (13-27 days) and median in-hospital or 30-day mortality was 4% (IQR 1-11%). In regions with high-income economies (n=100, 74%) patients were older (mean 56.5 <i>versus</i> 42.5 years, p<0.0001), but there were no significant differences in prevalence of pre-existing comorbidity nor in length of hospital stay or mortality.</p><p><strong>Conclusion: </strong>Patients with pleural infection have high levels of comorbidity and long hospital stays. Most reported data are from high-income economy settings. Data from lower-income regions is needed to better understand regional trends and enable optimal resource provision going forward.</p>","PeriodicalId":77419,"journal":{"name":"The European respiratory journal. Supplement","volume":"35 1","pages":""},"PeriodicalIF":0.0,"publicationDate":"2019-10-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC6860993/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"88214389","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"IL-1 receptor blockade skews inflammation towards Th2 in a mouse model of systemic sclerosis.","authors":"Anna Birnhuber, Slaven Crnkovic, Valentina Biasin, Leigh M Marsh, Balazs Odler, Anita Sahu-Osen, Elvira Stacher-Priehse, Luka Brcic, Frank Schneider, Nada Cikes, Bahil Ghanim, Walter Klepetko, Winfried Graninger, Yannick Allanore, Robert Eferl, Andrea Olschewski, Horst Olschewski, Grazyna Kwapiszewska","doi":"10.1183/13993003.00154-2019","DOIUrl":"10.1183/13993003.00154-2019","url":null,"abstract":"<p><p>The interleukin (IL)-1 family of cytokines is strongly associated with systemic sclerosis (SSc) and pulmonary involvement, but the molecular mechanisms are poorly understood. The aim of this study was to assess the role of IL-1α and IL-1β in pulmonary vascular and interstitial remodelling in a mouse model of SSc.IL-1α and IL-1β were localised in lungs of SSc patients and in the fos-related antigen-2 (Fra-2) transgenic (TG) mouse model of SSc. Lung function, haemodynamic parameters and pulmonary inflammation were measured in Fra-2 TG mice with or without 8 weeks of treatment with the IL-1 receptor antagonist anakinra (25 mg·kg^-1·day^-1). Direct effects of IL-1 on pulmonary arterial smooth muscle cells (PASMCs) and parenchymal fibroblasts were investigated <i>in vitro</i>Fra-2 TG mice exhibited increased collagen deposition in the lung, restrictive lung function and enhanced muscularisation of the vasculature with concomitant pulmonary hypertension reminiscent of the changes in SSc patients. Immunoreactivity of IL-1α and IL-1β was increased in Fra-2 TG mice and in patients with SSc. IL-1 stimulation reduced collagen expression in PASMCs and parenchymal fibroblasts <i>via</i> distinct signalling pathways. Blocking IL-1 signalling in Fra-2 TG worsened pulmonary fibrosis and restriction, enhanced T-helper cell type 2 (Th2) inflammation, and increased the number of pro-fibrotic, alternatively activated macrophages.Our data suggest that blocking IL-1 signalling as currently investigated in several clinical studies might aggravate pulmonary fibrosis in specific patient subsets due to Th2 skewing of immune responses and formation of alternatively activated pro-fibrogenic macrophages.</p>","PeriodicalId":77419,"journal":{"name":"The European respiratory journal. Supplement","volume":"1 1","pages":""},"PeriodicalIF":0.0,"publicationDate":"2019-09-29","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC6860995/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"81006324","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"Sequential screening for lung cancer in a high-risk group: randomised controlled trial","authors":"S. Spiro, P. Shah, R. Rintoul, J. George, S. Janes, M. Callister, M. Novelli, P. Shaw, G. Kocjan, C. Griffiths, M. Falzon, R. Booton, N. Magee, M. Peake, P. Dhillon, K. Sridharan, A. Nicholson, S. Padley, Magali N. Taylor, Asia Ahmed, J. Allen, Y. Ngai, N. Chinyanganya, V. Ashford-Turner, Sarah Lewis, D. Oukrif, P. Rabbitts, N. Counsell, A. Hackshaw","doi":"10.1183/13993003.00581-2019","DOIUrl":"https://doi.org/10.1183/13993003.00581-2019","url":null,"abstract":"Background Low-dose computed tomography (LDCT) screening detects early-stage lung cancer and reduces mortality. We proposed a sequential approach targeted to a high-risk group as a potentially efficient screening strategy. Methods LungSEARCH was a national multicentre randomised trial. Current/ex-smokers with mild/moderate chronic obstructive pulmonary disease (COPD) were allocated (1:1) to have 5 years surveillance or not. Screened participants provided annual sputum samples for cytology and cytometry, and if abnormal were offered annual LDCT and autofluorescence bronchoscopy (AFB). Those with normal sputum provided annual samples. The primary end-point was the percentage of lung cancers diagnosed at stage I/II (nonsmall cell) or limited disease (small cell). Results 1568 participants were randomised during 2007–2011 from 10 UK centres. 85.2% of those screened provided an adequate baseline sputum sample. There were 42 lung cancers among 785 screened individuals and 36 lung cancers among 783 controls. 54.8% (23 out of 42) of screened individuals versus 45.2% (14 out of 31) of controls with known staging were diagnosed with early-stage disease (one-sided p=0.24). Relative risk was 1.21 (95% CI 0.75–1.95) or 0.82 (95% CI 0.52–1.31) for early-stage or advanced cancers, respectively. Overall sensitivity for sputum (in those randomised to surveillance) was low (40.5%) with a cumulative false-positive rate (FPR) of 32.8%. 55% of cancers had normal sputum results throughout. Among sputum-positive individuals who had AFB, sensitivity was 45.5% and cumulative FPR was 39.5%; the corresponding measures for those who had LDCT were 100% and 16.1%, respectively. Conclusions Our sequential strategy, using sputum cytology/cytometry to select high-risk individuals for AFB and LDCT, did not lead to a clear stage shift and did not improve the efficiency of lung cancer screening. While low-dose CT is now preferred for lung cancer screening, our randomised trial of smokers with COPD showed that a proposed sequential policy using sputum testing to select who receives low-dose CT and autofluorescence bronchoscopy was ineffective http://bit.ly/2JZujnx","PeriodicalId":77419,"journal":{"name":"The European respiratory journal. Supplement","volume":"14 3","pages":""},"PeriodicalIF":0.0,"publicationDate":"2019-09-19","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://sci-hub-pdf.com/10.1183/13993003.00581-2019","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"72404433","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}

{"title":"Subpopulations of cells from bronchoalveolar lavage can predict prognosis in sarcoidosis","authors":"P. Darlington, S. Kullberg, A. Eklund, J. Grunewald","doi":"10.1183/13993003.01450-2019","DOIUrl":"https://doi.org/10.1183/13993003.01450-2019","url":null,"abstract":"Sarcoidosis is characterised by an accumulation of CD4+ T-cells in the lungs and an increased bronchoalveolar lavage fluid (BALF) CD4/CD8 ratio (>3.5) [1]. In sarcoidosis, an expansion of BALF CD4+ T-cells expressing the T-cell receptor Vα2.3 has been associated with good prognosis and with specific HLA-alleles, i.e. HLA-DRB1*0301 and HLA-DRB3*0101 (which is often carried together with HLA-DRB1*13). HLA-DRB1*03 and HLA-DRB3*0101 molecules show similarities in the region important for antigen presentation and both may therefore be capable of presenting identical antigens to the lung T-cells [2]. The higher the proportion of a T-cell subset (CD4+Vα2.3+ T-cells) in bronchoalveolar lavage fluid of sarcoidosis patients, the better the prognosis and therefore it may be used as an additional prognostic tool http://bit.ly/2Mta0Cs","PeriodicalId":77419,"journal":{"name":"The European respiratory journal. Supplement","volume":"2 1","pages":""},"PeriodicalIF":0.0,"publicationDate":"2019-08-22","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"82349737","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}

{"title":"Unmasking the hidden tuberculosis mortality burden in a large post mortem study in Maputo Central Hospital, Mozambique","authors":"A. García-Basteiro, J. Hurtado, P. Castillo, Fabiola Fernandes, M. Navarro, Lucilia Lovane, Isaac Casas, L. Quintó, Dércio Jordão, Mamudo R Ismail, C. Lorenzoni, C. Carrilho, Ariadna Sanz, N. Rakislova, Á. Mira, M. Álvarez-Martínez, A. Cossa, F. Cobelens, I. Mandomando, J. Vila, Q. Bassat, C. Menéndez, J. Ordi, M. Martínez","doi":"10.1183/13993003.00312-2019","DOIUrl":"https://doi.org/10.1183/13993003.00312-2019","url":null,"abstract":"Sensitive tools are needed to accurately establish the diagnosis of tuberculosis (TB) at death, especially in low-income countries. The objective of this study was to evaluate the burden of TB in a series of patients who died in a tertiary referral hospital in sub-Saharan Africa using an in-house real time PCR (TB-PCR) and the Xpert MTB/RIF Ultra (Xpert Ultra) assay. Complete diagnostic autopsies were performed in a series of 223 deaths (56.5% being HIV-positive), including 54 children, 57 maternal deaths and 112 other adults occurring at the Maputo Central Hospital, Mozambique. TB-PCR was performed in all lung, cerebrospinal fluid and central nervous system samples in HIV-positive patients. All samples positive for TB-PCR or showing histological findings suggestive of TB were analysed with the Xpert Ultra assay. TB was identified as the cause of death in 31 patients: three out of 54 (6%) children, five out of 57 (9%)maternal deaths and 23 out of 112 (21%) other adults. The sensitivity of the main clinical diagnosis to detect TB as the cause of death was 19.4% (95% CI 7.5–37.5) and the specificity was 97.4% (94.0–99.1) compared to autopsy findings. Concomitant TB (TB disease in a patient dying of other causes) was found in 31 additional cases. Xpert Ultra helped to identify 15 cases of concomitant TB. In 18 patients, Mycobacterium tuberculosis DNA was identified by TB-PCR and Xpert Ultra in the absence of histological TB lesions. Overall, 62 (27.8%) cases had TB disease at death and 80 (35.9%) had TB findings. The use of highly sensitive, easy to perform molecular tests in complete diagnostic autopsies may contribute to identifying TB cases at death that would have otherwise been missed. Routine use of these tools in certain diagnostic algorithms for hospitalised patients needs to be considered. Clinical diagnosis showed poor sensitivity for the diagnosis of TB at death. This study shows the usefulness of molecular assays in ascertaining TB diagnosis at death. It questions the information of clinical diagnoses obtained from hospital registries as a reliable tool for TB mortality estimation. http://bit.ly/2KrzTBJ","PeriodicalId":77419,"journal":{"name":"The European respiratory journal. Supplement","volume":"77 1","pages":""},"PeriodicalIF":0.0,"publicationDate":"2019-07-25","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"73910473","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}

{"title":"Accuracy of line probe assays for the diagnosis of pulmonary and multidrug-resistant tuberculosis: a systematic review and meta-analysis","authors":"R. Nathavitharana, P. Cudahy, S. Schumacher, K. Steingart, M. Pai, C. Denkinger","doi":"10.1183/13993003.01075-2016","DOIUrl":"https://doi.org/10.1183/13993003.01075-2016","url":null,"abstract":"Only 25% of multidrug-resistant tuberculosis (MDR-TB) cases are currently diagnosed. Line probe assays (LPAs) enable rapid drug-susceptibility testing for rifampicin (RIF) and isoniazid (INH) resistance and Mycobacterium tuberculosis detection. Genotype MTBDRplusV1 was WHO-endorsed in 2008 but newer LPAs have since been developed. This systematic review evaluated three LPAs: Hain Genotype MTBDRplusV1, MTBDRplusV2 and Nipro NTM+MDRTB. Study quality was assessed with QUADAS-2. Bivariate random-effects meta-analyses were performed for direct and indirect testing. Results for RIF and INH resistance were compared to phenotypic and composite (incorporating sequencing) reference standards. M. tuberculosis detection results were compared to culture. 74 unique studies were included. For RIF resistance (21 225 samples), pooled sensitivity and specificity (with 95% confidence intervals) were 96.7% (95.6–97.5%) and 98.8% (98.2–99.2%). For INH resistance (20 954 samples), pooled sensitivity and specificity were 90.2% (88.2–91.9%) and 99.2% (98.7–99.5%). Results were similar for direct and indirect testing and across LPAs. Using a composite reference standard, specificity increased marginally. For M. tuberculosis detection (3451 samples), pooled sensitivity was 94% (89.4–99.4%) for smear-positive specimens and 44% (20.2–71.7%) for smear-negative specimens. In patients with pulmonary TB, LPAs have high sensitivity and specificity for RIF resistance and high specificity and good sensitivity for INH resistance. This meta-analysis provides evidence for policy and practice. Line probe assays have high accuracy for detection of RIF resistance and INH resistance http://ow.ly/USX5305tqFV","PeriodicalId":77419,"journal":{"name":"The European respiratory journal. Supplement","volume":"2 1","pages":""},"PeriodicalIF":0.0,"publicationDate":"2017-01-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"77822148","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}

{"title":"A randomised controlled trial of tiotropium in adolescents with severe symptomatic asthma","authors":"E. Hamelmann, J. Bernstein, M. Vandewalker, P. Moroni-Zentgraf, D. Verri, A. Unseld, M. Engel, A. Boner","doi":"10.1183/13993003.01100-2016","DOIUrl":"https://doi.org/10.1183/13993003.01100-2016","url":null,"abstract":"We present results from the first phase III trial of once-daily tiotropium add-on to inhaled corticosteroids (ICS) plus one or more controller therapies in adolescents with severe symptomatic asthma. In this double-blind, parallel-group trial (NCT01277523), 392 patients aged 12–17 years were randomised to receive once-daily tiotropium 5 µg or 2.5 µg, or placebo, as an add-on to ICS plus other controller therapies over 12 weeks. The primary and key secondary end-points were change from baseline (response) in peak forced expiratory volume in 1 s (FEV1) within 3 h post-dosing (FEV1(0–3h)) and trough FEV1, respectively, after 12 weeks of treatment. Tiotropium 5 µg provided numerical improvements in peak FEV1(0–3h) response, compared with placebo (90 mL; p=0.104), and significant improvements were observed with tiotropium 2.5 µg (111 mL; p=0.046). Numerical improvements in trough FEV1 response and asthma control were observed with both tiotropium doses, compared with placebo. The safety and tolerability of tiotropium were comparable with those of placebo. Once-daily tiotropium Respimat add-on to ICS plus one or more controller therapies in adolescents with severe symptomatic asthma was well tolerated. The primary end-point of efficacy was not met, although positive trends for improvements in lung function and asthma control were observed. Tiotropium add-on therapy provided numerical improvements in outcomes in adolescents with asthma http://ow.ly/eL8g304a9XV","PeriodicalId":77419,"journal":{"name":"The European respiratory journal. Supplement","volume":"18 1","pages":""},"PeriodicalIF":0.0,"publicationDate":"2017-01-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"81438273","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}

{"title":"The international primary ciliary dyskinesia cohort (iPCD Cohort): methods and first results","authors":"M. Goutaki, Elisabeth Maurer, F. Halbeisen, I. Amirav, A. Barbato, L. Behan, M. Boon, C. Casaulta, A. Clément, S. Crowley, E. Haarman, C. Hogg, B. Karadag, C. Koerner-Rettberg, M. Leigh, M. Loebinger, H. Mazurek, L. Morgan, K. Nielsen, H. Omran, N. Schwerk, S. Scigliano, C. Werner, P. Yiallouros, Z. Zivković, J. Lucas, C. Kuehni","doi":"10.1183/13993003.01181-2016","DOIUrl":"https://doi.org/10.1183/13993003.01181-2016","url":null,"abstract":"Data on primary ciliary dyskinesia (PCD) epidemiology is scarce and published studies are characterised by low numbers. In the framework of the European Union project BESTCILIA we aimed to combine all available datasets in a retrospective international PCD cohort (iPCD Cohort). We identified eligible datasets by performing a systematic review of published studies containing clinical information on PCD, and by contacting members of past and current European Respiratory Society Task Forces on PCD. We compared the contents of the datasets, clarified definitions and pooled them in a standardised format. As of April 2016 the iPCD Cohort includes data on 3013 patients from 18 countries. It includes data on diagnostic evaluations, symptoms, lung function, growth and treatments. Longitudinal data are currently available for 542 patients. The extent of clinical details per patient varies between centres. More than 50% of patients have a definite PCD diagnosis based on recent guidelines. Children aged 10–19 years are the largest age group, followed by younger children (≤9 years) and young adults (20–29 years). This is the largest observational PCD dataset available to date. It will allow us to answer pertinent questions on clinical phenotype, disease severity, prognosis and effect of treatments, and to investigate genotype–phenotype correlations. The iPCD Cohort offers a unique opportunity to study PCD in an international retrospective cohort of >3000 patients http://ow.ly/rn0m304Jgsu","PeriodicalId":77419,"journal":{"name":"The European respiratory journal. Supplement","volume":"104 1","pages":""},"PeriodicalIF":0.0,"publicationDate":"2017-01-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"75926379","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}

{"title":"Mandatory tuberculosis case notification in high tuberculosis-incidence countries: policy and practice.","authors":"Mukund Uplekar, Sachin Atre, William A Wells, Diana Weil, Rafael Lopez, Giovanni Battista Migliori, Mario Raviglione","doi":"10.1183/13993003.00956-2016","DOIUrl":"10.1183/13993003.00956-2016","url":null,"abstract":"<p><p>Mandatory tuberculosis (TB) notification is an important policy under the End TB Strategy, but little is known about its enforcement especially in high TB incidence countries. We undertook a literature search for selected high-incidence countries, followed by a questionnaire-based survey among key informants in countries with high-, intermediate- and low-TB incidence. Published literature on TB notification in high-incidence countries was limited, but it did illustrate some of the current barriers to notification and the importance of electronic systems to facilitate reporting by private providers. Required survey data were successfully gathered from 40 out of 54 countries contacted. TB is notifiable in 11 out of 15 high-incidence countries, all 16 intermediate-incidence countries, and all nine low-incidence countries contacted. TB case notification by public sector facilities is generally systematised, but few high-incidence countries had systems and tools to facilitate notification from private care providers. In the context of the new End TB Strategy aimed at eventual TB elimination, all countries should have TB on their national list of notifiable diseases. Enhancing the ease of notification by private providers is essential for effective implementation. To that effect, investing in strengthening disease surveillance systems and introducing digital tools to simplify notification are logical ways forward.</p>","PeriodicalId":77419,"journal":{"name":"The European respiratory journal. Supplement","volume":"59 1","pages":"1571-1581"},"PeriodicalIF":0.0,"publicationDate":"2016-12-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC5898941/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"84614742","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}