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1. Combined approaches, including long-read sequencing, address the diagnostic challenge of HYDIN in primary ciliary dyskinesia

Author

Fleming, Andrew, Galey, Miranda, Briggs, Lizi, Edwards, Matthew, Hogg, Claire, John, Shibu, Wilkinson, Sam, Quinn, Ellie, Rai, Ranjit, Burgoyne, Tom, Rogers, Andy, Patel, Mitali P., Griffin, Paul, Muller, Steven, Carr, Siobhan B., Loebinger, Michael R., Lucas, Jane S., Shah, Anand, Jose, Ricardo, Mitchison, Hannah M., Shoemark, Amelia, Miller, Danny E., and Morris-Rosendahl, Deborah J.

Published
2024
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2. Bronchiectasis and asthma: Data from the European Bronchiectasis Registry (EMBARC)

Author

Polverino, Eva, Dimakou, Katerina, Traversi, Letizia, Bossios, Apostolos, Haworth, Charles S., Loebinger, Michael R., De Soyza, Anthony, Vendrell, Montserrat, Burgel, Pierre-Régis, Mertsch, Pontus, McDonnell, Melissa, Škrgat, Sabina, Maiz Carro, Luis, Sibila, Oriol, van der Eerden, Menno, Kauppi, Paula, Hill, Adam T., Wilson, Robert, Milenkovic, Branislava, Menendez, Rosario, Murris, Marlene, Digalaki, Tonia, Crichton, Megan L., Borecki, Sermin, Obradovic, Dusanka, Nowinski, Adam, Amorim, Adelina, Torres, Antoni, Lorent, Natalie, Welte, Tobias, Blasi, Francesco, Van Braeckel, Eva, Altenburg, Josje, Shoemark, Amelia, Shteinberg, Michal, Boersma, Wim, Elborn, J. Stuart, Aliberti, Stefano, Ringshausen, Felix C., Chalmers, James D., and Goeminne, Pieter C.

Published
2024
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4. Safety and efficacy of the epithelial sodium channel blocker idrevloride in people with primary ciliary dyskinesia (CLEAN-PCD): a multinational, phase 2, randomised, double-blind, placebo-controlled crossover trial

Author

Ringshausen, Felix C, Shapiro, Adam J, Nielsen, Kim G, Mazurek, Henryk, Pifferi, Massimo, Donn, Karl H, van der Eerden, Menno M, Loebinger, Michael R, Zariwala, Maimoona A, Leigh, Margaret W, Knowles, Michael R, Ferkol, Jr, Thomas W, Brown, Trey, Carroll, Mary, Church, Nina, Couluris, Marisa, Davis, Stephanie D, Dell, Sharon D, Di Cicco, Maria E, Di Mango, Angela, Escobar, Hugo, Griffiths, Anne, Haver, Kenan, Hornick, Douglas, Johnson, Christopher, Milla, Carlos E, O'Donnell, Anne, Pink, Isabell, Pogorzelski, Andrzej, Prickett, Michelle, Raby, Benjamin A, Rosenfeld, Margaret, Saba, Thomas G, Sandvik, Rikke Mulvad, Sagel, Scott D, Salathe, Matthias, Simmons, Ashley E, Solomon, George M, Sommerburg, Olaf, Soussi, Najwa, Strausbaugh, Steven D, Sullivan, Kelli M, Werner, Claudius, and Ferkol, Thomas W

Published
2024
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7. Bronchiectasis in Europe: data on disease characteristics from the European Bronchiectasis registry (EMBARC)

Author

Chalmers, James D, Polverino, Eva, Crichton, Megan L, Ringshausen, Felix C, De Soyza, Anthony, Vendrell, Montserrat, Burgel, Pierre Régis, Haworth, Charles S, Loebinger, Michael R, Dimakou, Katerina, Murris, Marlene, Wilson, Robert, Hill, Adam T, Menendez, Rosario, Torres, Antoni, Welte, Tobias, Blasi, Francesco, Altenburg, Josje, Shteinberg, Michal, Boersma, Wim, Elborn, J Stuart, Goeminne, Pieter C, and Aliberti, Stefano

Published
2023
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9. Fluoroquinolones and isoniazid-resistant tuberculosis: implications for the 2018 WHO guidance

Author

Stagg, Helen R, Bothamley, Graham H, Davidson, Jennifer A, Kunst, Heinke, Lalor, Maeve K, Lipman, Marc C, Loutet, Miranda G, Lozewicz, Stefan, Mohiyuddin, Tehreem, Abbara, Aula, Alexander, Eliza, Booth, Helen, Creer, Dean D, Harris, Ross J, Kon, Onn Min, Loebinger, Michael R, McHugh, Timothy D, Milburn, Heather J, Palchaudhuri, Paramita, Phillips, Patrick PJ, Schmok, Erik, Taylor, Lucy, Abubakar, Ibrahim, Baker, Lucy V, Barrett, Jessica C, Burgess, Helen, Cosgrove, Catherine, Dunleavy, Anne, Francis, Marie, Gupta, Urmi, Hamid, Shahid, Haselden, Brigitte M, Holden, Emma, Kahr, Vanessa, Lynn, William, Perrin, Felicity M, Rahman, Ananna, and Soobratty, Mohammad R

Subjects
Biomedical and Clinical Sciences, Clinical Sciences, Tuberculosis, Rare Diseases, Antimicrobial Resistance, Clinical Research, Infectious Diseases, Orphan Drug, Infection, Good Health and Well Being, Adolescent, Adult, Aged, Antitubercular Agents, Drug Therapy, Combination, Duration of Therapy, Ethambutol, Female, Fluoroquinolones, Humans, Isoniazid, Levofloxacin, Logistic Models, London, Male, Middle Aged, Practice Guidelines as Topic, Pyrazinamide, Recurrence, Retrospective Studies, Rifampin, Treatment Failure, Tuberculosis, Multidrug-Resistant, World Health Organization, Young Adult, London INH-R TB study group, Medical and Health Sciences, Respiratory System, Cardiovascular medicine and haematology
Abstract

2018 World Health Organization (WHO) guidelines for the treatment of isoniazid (H)-resistant (Hr) tuberculosis recommend a four-drug regimen: rifampicin (R), ethambutol (E), pyrazinamide (Z) and levofloxacin (Lfx), with or without H ([H]RZE-Lfx). This is used once Hr is known, such that patients complete 6 months of Lfx (≥6[H]RZE-6Lfx). This cohort study assessed the impact of fluoroquinolones (Fq) on treatment effectiveness, accounting for Hr mutations and degree of phenotypic resistance. This was a retrospective cohort study of 626 Hr tuberculosis patients notified in London, 2009-2013. Regimens were described and logistic regression undertaken of the association between regimen and negative regimen-specific outcomes (broadly, death due to tuberculosis, treatment failure or disease recurrence). Of 594 individuals with regimen information, 330 (55.6%) were treated with (H)RfZE (Rf=rifamycins) and 211 (35.5%) with (H)RfZE-Fq. The median overall treatment period was 11.9 months and median Z duration 2.1 months. In a univariable logistic regression model comparing (H)RfZE with and without Fqs, there was no difference in the odds of a negative regimen-specific outcome (baseline (H)RfZE, cluster-specific odds ratio 1.05 (95% CI 0.60-1.82), p=0.87; cluster NHS trust). Results varied minimally in a multivariable model. This odds ratio dropped (0.57, 95% CI 0.14-2.28) when Hr genotype was included, but this analysis lacked power (p=0.42). In a high-income setting, we found a 12-month (H)RfZE regimen with a short Z duration to be similarly effective for Hr tuberculosis with or without a Fq. This regimen may result in fewer adverse events than the WHO recommendations.

Published
2019

12. Criteria and definitions for the radiological and clinical diagnosis of bronchiectasis in adults for use in clinical trials: international consensus recommendations

Author

Aliberti, Stefano, Goeminne, Pieter C, O'Donnell, Anne E, Aksamit, Timothy R, Al-Jahdali, Hamdan, Barker, Alan F, Blasi, Francesco, Boersma, Wim G, Crichton, Megan L, De Soyza, Anthony, Dimakou, Katerina E, Elborn, Stuart J, Feldman, Charles, Tiddens, Harm, Haworth, Charles S, Hill, Adam T, Loebinger, Michael R, Martinez-Garcia, Miguel Angel, Meerburg, Jennifer J, Menendez, Rosario, Morgan, Lucy C, Murris, Marlene S, Polverino, Eva, Ringshausen, Felix C, Shteinberg, Michal, Sverzellati, Nicola, Tino, Gregory, Torres, Antoni, Vandendriessche, Thomas, Vendrell, Montserrat, Welte, Tobias, Wilson, Robert, Wong, Conroy A, and Chalmers, James D

Published
2022
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16. Airway clearance management in people with bronchiectasis: data from the European Bronchiectasis Registry (EMBARC)

Author

Spinou, Arietta, primary, Hererro-Cortina, Beatriz, additional, Aliberti, Stefano, additional, Goeminne, Pieter C, additional, Polverino, Eva, additional, Dimakou, Katerina, additional, Haworth, Charles S, additional, Loebinger, Michael R, additional, De Soyza, Anthony, additional, Vendrell, Montserrat, additional, Burgel, Pierre Regis, additional, McDonnell, Melissa, additional, Sutharsan, Sivagurunathan, additional, Skrgat, Sabina, additional, Maiz-Carro, Luiz, additional, Sibila, Oriol, additional, Stolz, Daiana, additional, Kauppi, Paula, additional, Bossios, Apostolos, additional, Hill, Adam T, additional, Clifton, Ian, additional, Crichton, Megan L, additional, Walker, Paul, additional, Menendez, Rosario, additional, Borecki, Sermin, additional, Obradovic, Dusanka, additional, Nowinski, Adam, additional, Amorim, Adelina, additional, Torres, Antoni, additional, Lorent, Natalie, additional, Welte, Tobias, additional, Blasi, Francesco, additional, Makek, Mateja Jankovic, additional, Shteinberg, Michal, additional, Boersma, Wim, additional, Elborn, J Stuart, additional, Chalmers, James D, additional, and Ringshausen, Felix C, additional

Published
2024
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18. Bronchiectasis

Author

Stowell, Janet M., primary, José, Ricardo J., additional, Loebinger, Michael R., additional, Boyton, Rosemary J., additional, and Wilson, Robert, additional

Published
2022
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20. Bronchiectasis and asthma:Data from the European Bronchiectasis Registry (EMBARC)

Author

Polverino, Eva, Dimakou, Katerina, Traversi, Letizia, Bossios, Apostolos, Haworth, Charles S., Loebinger, Michael R., De Soyza, Anthony, Vendrell, Montserrat, Burgel, Pierre Régis, Mertsch, Pontus, McDonnell, Melissa, Škrgat, Sabina, Maiz Carro, Luis, Sibila, Oriol, van der Eerden, Menno, Kauppi, Paula, Hill, Adam T., Wilson, Robert, Milenkovic, Branislava, Menendez, Rosario, Murris, Marlene, Digalaki, Tonia, Crichton, Megan L., Borecki, Sermin, Obradovic, Dusanka, Nowinski, Adam, Amorim, Adelina, Torres, Antoni, Lorent, Natalie, Welte, Tobias, Blasi, Francesco, Van Braeckel, Eva, Altenburg, Josje, Shoemark, Amelia, Shteinberg, Michal, Boersma, Wim, Elborn, J. Stuart, Aliberti, Stefano, Ringshausen, Felix C., Chalmers, James D., Goeminne, Pieter C., Polverino, Eva, Dimakou, Katerina, Traversi, Letizia, Bossios, Apostolos, Haworth, Charles S., Loebinger, Michael R., De Soyza, Anthony, Vendrell, Montserrat, Burgel, Pierre Régis, Mertsch, Pontus, McDonnell, Melissa, Škrgat, Sabina, Maiz Carro, Luis, Sibila, Oriol, van der Eerden, Menno, Kauppi, Paula, Hill, Adam T., Wilson, Robert, Milenkovic, Branislava, Menendez, Rosario, Murris, Marlene, Digalaki, Tonia, Crichton, Megan L., Borecki, Sermin, Obradovic, Dusanka, Nowinski, Adam, Amorim, Adelina, Torres, Antoni, Lorent, Natalie, Welte, Tobias, Blasi, Francesco, Van Braeckel, Eva, Altenburg, Josje, Shoemark, Amelia, Shteinberg, Michal, Boersma, Wim, Elborn, J. Stuart, Aliberti, Stefano, Ringshausen, Felix C., Chalmers, James D., and Goeminne, Pieter C.

Abstract

Background: Asthma is commonly reported in patients with a diagnosis of bronchiectasis. Objective: The aim of this study was to evaluate whether patients with bronchiectasis and asthma (BE+A) had a different clinical phenotype and different outcomes compared with patients with bronchiectasis without concomitant asthma. Methods: A prospective observational pan-European registry (European Multicentre Bronchiectasis Audit and Research Collaboration) enrolled patients across 28 countries. Adult patients with computed tomography–confirmed bronchiectasis were reviewed at baseline and annual follow-up visits using an electronic case report form. Asthma was diagnosed by the local investigator. Follow-up data were used to explore differences in exacerbation frequency between groups using a negative binomial regression model. Survival analysis used Cox proportional hazards regression. Results: Of 16,963 patients with bronchiectasis included for analysis, 5,267 (31.0%) had investigator-reported asthma. Patients with BE+A were younger, were more likely to be female and never smokers, and had a higher body mass index than patients with bronchiectasis without asthma. BE+A was associated with a higher prevalence of rhinosinusitis and nasal polyps as well as eosinophilia and Aspergillus sensitization. BE+A had similar microbiology but significantly lower severity of disease using the bronchiectasis severity index. Patients with BE+A were at increased risk of exacerbation after adjustment for disease severity and multiple confounders. Inhaled corticosteroid (ICS) use was associated with reduced mortality in patients with BE+A (adjusted hazard ratio 0.78, 95% CI 0.63-0.95) and reduced risk of hospitalization (rate ratio 0.67, 95% CI 0.67-0.86) compared with control subjects without asthma and not receiving ICSs. Conclusions: BE+A was common and was associated with an increased risk of exacerbations and improved outcomes with ICS use. Unexpectedly we identified significantly lower

Published
2024

21. Objective sputum colour assessment and clinical outcomes in bronchiectasis: data from the European Bronchiectasis Registry (EMBARC)

Author

Aliberti, Stefano, Ringshausen, Felix C., Dhar, Raja, Haworth, Charles S., Loebinger, Michael R., Dimakou, Katerina, Crichton, Megan L., De Soyza, Anthony, Vendrell, Montse, Burgel, Pierre-Regis, McDonnell, Melissa, Skrgat, Sabina, Maiz Carro, Luis, de Roux, Andres, Sibila, Oriol, Bossios, Apostolos, van der Eerden, Menno, Kauppi, Paula, Wilson, Robert, Milenkovic, Branislava, Menendez, Rosario, Murris, Marlene, Borekci, Sermin, Munteanu, Oxana, Obradovic, Dusanka, Nowinski, Adam, Amorim, Adelina, Torres, Antoni, Lorent, Natalie, Van Braeckel, Eva, Altenburg, Josje, Shoemark, Amelia, Shteinberg, Michal, Boersma, Wim, Goeminne, Pieter C., Elborn, J. Stuart, Hill, Adam T., Welte, Tobias, Blasi, Francesco, Polverino, Eva, Chalmers, James D., Aliberti, Stefano, Ringshausen, Felix C., Dhar, Raja, Haworth, Charles S., Loebinger, Michael R., Dimakou, Katerina, Crichton, Megan L., De Soyza, Anthony, Vendrell, Montse, Burgel, Pierre-Regis, McDonnell, Melissa, Skrgat, Sabina, Maiz Carro, Luis, de Roux, Andres, Sibila, Oriol, Bossios, Apostolos, van der Eerden, Menno, Kauppi, Paula, Wilson, Robert, Milenkovic, Branislava, Menendez, Rosario, Murris, Marlene, Borekci, Sermin, Munteanu, Oxana, Obradovic, Dusanka, Nowinski, Adam, Amorim, Adelina, Torres, Antoni, Lorent, Natalie, Van Braeckel, Eva, Altenburg, Josje, Shoemark, Amelia, Shteinberg, Michal, Boersma, Wim, Goeminne, Pieter C., Elborn, J. Stuart, Hill, Adam T., Welte, Tobias, Blasi, Francesco, Polverino, Eva, and Chalmers, James D.

Abstract

Background A validated 4-point sputum colour chart can be used to objectively evaluate the levels of airway inflammation in bronchiectasis patients. In the European Bronchiectasis Registry (EMBARC), we tested whether sputum colour would be associated with disease severity and clinical outcomes. Methods We used a prospective, observational registry of adults with bronchiectasis conducted in 31 countries. Patients who did not produce spontaneous sputum were excluded from the analysis. The Murray sputum colour chart was used at baseline and at follow-up visits. Key outcomes were frequency of exacerbations, hospitalisations for severe exacerbations and mortality during up to 5-year follow-up. Results 13 484 patients were included in the analysis. More purulent sputum was associated with lower forced expiratory volume in 1 s (FEV 1), worse quality of life, greater bacterial infection and a higher bronchiectasis severity index. Sputum colour was strongly associated with the risk of future exacerbations during follow-up. Compared to patients with mucoid sputum (reference group), patients with mucopurulent sputum experienced significantly more exacerbations (incident rate ratio (IRR) 1.29, 95% CI 1.22–1.38; p<0.0001), while the rates were even higher for patients with purulent (IRR 1.55, 95% CI 1.44–1.67; p<0.0001) and severely purulent sputum (IRR 1.91, 95% CI 1.52–2.39; p<0.0001). Hospitalisations for severe exacerbations were also associated with increasing sputum colour with rate ratios, compared to patients with mucoid sputum, of 1.41 (95% CI 1.29–1.56; p<0.0001), 1.98 (95% CI 1.77–2.21; p<0.0001) and 3.05 (95% CI 2.25–4.14; p<0.0001) for mucopurulent, purulent and severely purulent sputum, respectively. Mortality was significantly increased with increasing sputum purulence, hazard ratio 1.12 (95% CI 1.01–1.24; p=0.027), for each increment in sputum purulence. Conclusion Sputum colour is a simple marker of disease severity and future ris

Published
2024

23. Structural Lung Disease and Clinical Phenotype in Bronchiectasis Patients: The EMBARC CT Study.

Author

Pieters, Angelina L. P., van der Veer, Tjeerd, Meerburg, Jennifer J., Andrinopoulou, Eleni-Rosalina, van der Eerden, Menno M., Ciet, Pierluigi, Aliberti, Stefano, Burgel, Pierre-Regis, Crichton, Megan L., Shoemark, Amelia, Goeminne, Pieter C., Shteinberg, Michal, Loebinger, Michael R., Haworth, Charles S., Blasi, Francesco, Tiddens, Harm A. W. M., Caudri, Daan, and Chalmers, James D.

Subjects
CILIARY motility disorders, LUNG diseases, ATELECTASIS, BRONCHIECTASIS, DYSKINESIAS, CHRONIC obstructive pulmonary disease, MYCOBACTERIAL diseases
Abstract

Rationale: Chest computed tomography (CT) scans are essential to diagnose and monitor bronchiectasis (BE). To date, few quantitative data are available about the nature and extent of structural lung abnormalities (SLAs) on CT scans of patients with BE. Objectives: To investigate SLAs on CT scans of patients with BE and the relationship of SLAs to clinical features using the EMBARC (European Multicenter Bronchiectasis Audit and Research Collaboration) registry. Methods: CT scans from patients with BE included in the EMBARC registry were analyzed using the validated Bronchiectasis Scoring Technique for CT (BEST-CT). The subscores of this instrument are expressed as percentages of total lung volume. The items scored are atelectasis/consolidation, BE with and without mucus plugging (MP), airway wall thickening, MP, ground-glass opacities, bullae, airways, and parenchyma. Four composite scores were calculated: total BE (i.e., BE with and without MP), total MP (i.e., BE with MP plus MP alone), total inflammatory changes (i.e., atelectasis/consolidation plus total MP plus ground-glass opacities), and total disease (i.e., all items but airways and parenchyma). Measurements and Main Results: CT scans of 524 patients with BE were analyzed. Mean subscores were 4.6 (range, 2.3–7.7) for total BE, 4.2 (1.2–8.1) for total MP, 8.3 (3.5–16.7) for total inflammatory changes, and 14.9 (9.1–25.9) for total disease. BE associated with primary ciliary dyskinesia was associated with more SLAs, whereas chronic obstructive pulmonary disease was associated with fewer SLAs. Lower FEV1, longer disease duration, Pseudomonas aeruginosa and nontuberculous mycobacterial infections, and severe exacerbations were all independently associated with worse SLAs. Conclusions: The type and extent of SLAs in patients with BE are highly heterogeneous. Strong relationships between radiological disease and clinical features suggest that CT analysis may be a useful tool for clinical phenotyping. [ABSTRACT FROM AUTHOR]

Published
2024
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24. The Association between Bronchiectasis and Chronic Obstructive Pulmonary Disease: Data from the European Bronchiectasis Registry (EMBARC).

Author

Polverino, Eva, De Soyza, Anthony, Dimakou, Katerina, Traversi, Letizia, Bossios, Apostolos, Crichton, Megan L., Ringshausen, Felix C., Vendrell, Montserrat, Burgel, Pierre-Régis, Haworth, Charles S., Loebinger, Michael R., Lorent, Natalie, Pink, Isabell, McDonnell, Melissa, Skrgat, Sabina, Carro, Luis M., Sibila, Oriol, van der Eerden, Menno, Kauppi, Paula, and Shoemark, Amelia

Subjects
BRONCHIECTASIS, CHRONIC obstructive pulmonary disease
Abstract

Rationale: COPD and bronchiectasis are commonly reported together. Studies report varying impacts of co-diagnosis on outcomes, which may be related to different definitions of disease used across studies. Objectives: To investigate the prevalence of chronic obstructive pulmonary disease (COPD) associated with bronchiectasis and its relationship with clinical outcomes. We further investigated the impact of implementing the standardized ROSE criteria (radiological bronchiectasis [R], obstruction [FEV1/FVC ratio <0.7; O], symptoms [S], and exposure [⩾10 pack-years of smoking; E]), an objective definition of the association of bronchiectasis with COPD. Methods: Analysis of the EMBARC (European Bronchiectasis Registry), a prospective observational study of patients with computed tomography–confirmed bronchiectasis from 28 countries. The ROSE criteria were used to objectively define the association of bronchiectasis with COPD. Key outcomes during a maximum of 5 years of follow-up were exacerbations, hospitalization, and mortality. Measurements and Main Results: A total of 16,730 patients with bronchiectasis were included; 4,336 had a clinician-assigned codiagnosis of COPD, and these patients had more exacerbations, worse quality of life, and higher severity scores. We observed marked overdiagnosis of COPD: 22.2% of patients with a diagnosis of COPD did not have airflow obstruction and 31.9% did not have a history of ⩾10 pack-years of smoking. Therefore, 2,157 patients (55.4%) met the ROSE criteria for COPD. Compared with patients without COPD, patients who met the ROSE criteria had increased risks of exacerbations and exacerbations resulting in hospitalization during follow-up (incidence rate ratio, 1.25; 95% confidence interval, 1.15–1.35; vs. incidence rate ratio, 1.69; 95% confidence interval, 1.51–1.90, respectively). Conclusions: The label of COPD is often applied to patients with bronchiectasis who do not have objective evidence of airflow obstruction or a smoking history. Patients with a clinical label of COPD have worse clinical outcomes. [ABSTRACT FROM AUTHOR]

Published
2024
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26. NTM in Bronchiectasis

Author

Loebinger, Michael R., Aksamit, Timothy, Chalmers, James, editor, Polverino, Eva, editor, and Aliberti, Stefano, editor

Published
2018
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27. LUNG FUNCTION IN PATIENTS WITH NON-CYSTIC FIBROSIS BRONCHIECTASIS BY PRESPECIFIED SUBGROUPS IN THE PHASE 3, RANDOMIZED, DOUBLE-BLIND, PLACEBO-CONTROLLED ASPEN TRIAL

Author

AKSAMIT, TIMOTHY R., CHALMERS, JAMES D., BURGEL, PIERRE-RÉGIS, DALEY, CHARLES L., DE SOYZA, ANTHONY, HAWORTH, CHARLES S., MAUGER, DAVID, LOEBINGER, MICHAEL R., HASEGAWA, NAOKI, MORIMOTO, KOZO, RINGSHAUSEN, FELIX C., MANGE, KEVIN, TEPER, ARIEL, FERNANDEZ, CARLOS, FAN, CHUNPENG, ZHANG, XIANGMIN, and METERSKY, MARK L.

Published
2024
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28. ANNUALIZED EXACERBATION RATE IN PATIENTS WITH NON-CYSTIC FIBROSIS BRONCHIECTASIS BY PRESPECIFIED SUBGROUPS IN THE PH3, RANDOMIZED, DOUBLE-BLIND, PLACEBO-CONTROLLED ASPEN TRIAL

Author

METERSKY, MARK L., BURGEL, PIERRE-RÉGIS, DALEY, CHARLES L., SOYZA, ANTHONYDE, HAWORTH, CHARLES S., MAUGER, DAVID, LOEBINGER, MICHAEL R., AKSAMIT, TIMOTHY R., HASEGAWA, NAOKI, MORIMOTO, KOZO, RINGSHAUSEN, FELIX C., MANGE, KEVIN, TEPER, ARIEL, FERNANDEZ, CARLOS, FAN, CHUNPENG, ZHANG, XIANGMIN, and CHALMERS, JAMES D.

Published
2024
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30. Response

Author

Loebinger, Michael R., primary, Quint, Jennifer K., additional, van der Laan, Roald, additional, Obradovic, Marko, additional, Chawla, Rajinder, additional, Kishore, Amit, additional, and van Ingen, Jakko, additional

Published
2023
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31. A BEAT-PCD consensus statement: a core outcome set for pulmonary disease interventions in primary ciliary dyskinesia

Author

Kos, Renate, primary, Goutaki, Myrofora, additional, Kobbernagel, Helene E., additional, Rubbo, Bruna, additional, Shoemark, Amelia, additional, Aliberti, Stefano, additional, Altenburg, Josje, additional, Anagnostopoulou, Pinelopi, additional, Athanazio, Rodrigo A., additional, Beydon, Nicole, additional, Dell, Sharon D., additional, Emiralioglu, Nagehan, additional, Ferkol, Thomas W., additional, Loebinger, Michael R., additional, Lorent, Natalie, additional, Maître, Bernard, additional, Marthin, June, additional, Morgan, Lucy C., additional, Nielsen, Kim G., additional, Ringshausen, Felix C., additional, Shteinberg, Michal, additional, Tiddens, Harm A.W.M., additional, Maitland-Van der Zee, Anke H., additional, Chalmers, James D., additional, Lucas, Jane S.A., additional, and Haarman, Eric G., additional

Published
2023
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32. RCT Abstract - Safety and efficacy of idrevloride in people with primary ciliary dyskinesia: a double-blind, randomized, placebo-controlled crossover trial (CLEAN-PCD)

Author

Ringshausen, Felix C, primary, Shapiro, Adam J, additional, Nielsen, Kim G, additional, Mazurek, Henryk, additional, Pifferi, Massimo, additional, Donn, Karl H, additional, Van Der Eerden, Menno M, additional, Loebinger, Michael R, additional, Zariwala, Maimoona A, additional, Leigh, Margaret W, additional, Knowles, Michael R, additional, and Ferkol, Thomas W, additional

Published
2023
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33. Preliminary results from the “Genes encoding complex ciliopathies of bronchiectasis” GECCO study: An EMBARC project

Author

Shoemark, Amelia, primary, Fassad, Mahmoud, additional, Perea, Lidia, additional, Cant, Erin, additional, Bottier, Mathieu, additional, Altenburg, Josje, additional, De-Soyza, Anthony, additional, Lorent, Natalie, additional, Loebinger, Michael R, additional, Ringshausen, Felix C, additional, Mitchison, Hannah M, additional, and Chalmers, James D, additional

Published
2023
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34. Effect of treatment with tobramycin on the airway microbiota and resistome in bronchiectasis patients chronically colonised with Pseudomonas aeruginosa: iBEST study

Author

Tunney, Michael M, primary, Einarsson, Gisli G, additional, Lee, Andrew J, additional, Alkhatib, Aya, additional, Carson, Jack, additional, Marshall, Andrew, additional, Carson, Stephen, additional, Gilpin, Deirdre F, additional, Angyalosi, Gerhild, additional, Malaterre, Vincent, additional, Chalmers, James D, additional, Loebinger, Michael R, additional, Blasi, Francesco, additional, Haworth, Charles S, additional, and Elborn, J Stuart, additional

Published
2023
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36. Pseudomonas aeruginosa eradication in patients with bronchiectasis: data from the EMBARC registry

Author

Nigro, Mattia, primary, Haworth, Charles, additional, Polverino, Eva, additional, Crichton, Megan L., additional, Ringsahusen, Felix C., additional, De Soyza, Anthony, additional, Vendrell, Montserrat, additional, Regis-Burgel, Pierre, additional, Loebinger, Michael R., additional, Dimakou, Katerina, additional, Menendez, Rosario, additional, Torres, Antoni, additional, Welte, Tobias, additional, Blasi, Francesco, additional, Shteinberg, Michal, additional, Boersma, Wim, additional, Elborn, J. Stuart, additional, Goeminne, Pieter C., additional, Chalmers, James D., additional, and Aliberti, Stefano, additional

Published
2023
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37. Blood eosinophils, inhaled corticosteroids and exacerbations in bronchiectasis: Data from the EMBARC registry

Author

Pollock, Jennifer, primary, Polverino, Eva, additional, Crichton, Megan L., additional, Regis-Burgel, Pierre, additional, Haworth, Charles, additional, Dimakou, Katerina, additional, Loebinger, Michael R., additional, Menendez, Rosario, additional, Torres, Antoni, additional, Welte, Tobias, additional, Blasi, Francesco, additional, Ringshausen, Felix, additional, De Soyza, Anthony, additional, Vendrell, Montserrat, additional, Elborn, Stuart J., additional, Shoemark, Amelia, additional, Aliberti, Stefano, additional, Goeminne, Pieter, additional, and Chalmers, James D., additional

Published
2023
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39. Safety and efficacy of the epithelial sodium channel blocker idrevloride in people with primary ciliary dyskinesia (CLEAN-PCD): a multinational, phase 2, randomised, double-blind, placebo-controlled crossover trial

Author

Ringshausen, Felix C, primary, Shapiro, Adam J, additional, Nielsen, Kim G, additional, Mazurek, Henryk, additional, Pifferi, Massimo, additional, Donn, Karl H, additional, van der Eerden, Menno M, additional, Loebinger, Michael R, additional, Zariwala, Maimoona A, additional, Leigh, Margaret W, additional, Knowles, Michael R, additional, Ferkol, Thomas W, additional, Ringshausen, Felix C, additional, Ferkol, Jr, Thomas W, additional, Brown, Trey, additional, Carroll, Mary, additional, Church, Nina, additional, Couluris, Marisa, additional, Davis, Stephanie D, additional, Dell, Sharon D, additional, Di Cicco, Maria E, additional, Di Mango, Angela, additional, Escobar, Hugo, additional, Griffiths, Anne, additional, Haver, Kenan, additional, Hornick, Douglas, additional, Johnson, Christopher, additional, Milla, Carlos E, additional, O'Donnell, Anne, additional, Pink, Isabell, additional, Pogorzelski, Andrzej, additional, Prickett, Michelle, additional, Raby, Benjamin A, additional, Rosenfeld, Margaret, additional, Saba, Thomas G, additional, Sandvik, Rikke Mulvad, additional, Sagel, Scott D, additional, Salathe, Matthias, additional, Simmons, Ashley E, additional, Solomon, George M, additional, Sommerburg, Olaf, additional, Soussi, Najwa, additional, Strausbaugh, Steven D, additional, Sullivan, Kelli M, additional, and Werner, Claudius, additional

Published
2023
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41. A BEAT-PCD consensus statement: a core outcome set for pulmonary disease interventions in primary ciliary dyskinesia.

Author

Kos, Renate, Goutaki, Myrofora, Kobbernagel, Helene E., Rubbo, Bruna, Shoemark, Amelia, Aliberti, Stefano, Altenburg, Josje, Anagnostopoulou, Pinelopi, Athanazio, Rodrigo A., Beydon, Nicole, Dell, Sharon D., Emiralioglu, Nagehan, Ferkol, Thomas W., Loebinger, Michael R., Lorent, Natalie, Maître, Bernard, Marthin, June, Morgan, Lucy C., Nielsen, Kim G., and Ringshausen, Felix C.

Published
2024
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43. Clinimetric Properties of Outcome Measures in Bronchiectasis

Author

Bradley, Judy M., primary, Ferguson, Kathryn, additional, Bailey, Andrew, additional, O’Neill, Katherine, additional, McLeese, Rebecca H., additional, Hill, Adam T., additional, Loebinger, Michael R., additional, Carroll, Mary, additional, Chalmers, James D., additional, Gatheral, Timothy, additional, Johnson, Christopher, additional, De Soyza, Anthony, additional, Hurst, John R., additional, Downey, Damian G., additional, and Elborn, J. Stuart, additional

Published
2023
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49. Combined approaches, including long-read sequencing, address the diagnostic challenge of HYDINin primary ciliary dyskinesia

Author

Fleming, Andrew, Galey, Miranda, Briggs, Lizi, Edwards, Matthew, Hogg, Claire, John, Shibu, Wilkinson, Sam, Quinn, Ellie, Rai, Ranjit, Burgoyne, Tom, Rogers, Andy, Patel, Mitali P., Griffin, Paul, Muller, Steven, Carr, Siobhan B., Loebinger, Michael R., Lucas, Jane S., Shah, Anand, Jose, Ricardo, Mitchison, Hannah M., Shoemark, Amelia, Miller, Danny E., and Morris-Rosendahl, Deborah J.

Abstract

Primary ciliary dyskinesia (PCD), a disorder of the motile cilia, is now recognised as an underdiagnosed cause of bronchiectasis. Accurate PCD diagnosis comprises clinical assessment, analysis of cilia and the identification of biallelic variants in one of 50 known PCD-related genes, including HYDIN. HYDIN-related PCD is underdiagnosed due to the presence of a pseudogene, HYDIN2, with 98% sequence homology to HYDIN. This presents a significant challenge for Short-Read Next Generation Sequencing (SR-NGS) and analysis, and many diagnostic PCD gene panels do not include HYDIN. We have used a combined approach of SR-NGS with bioinformatic masking of HYDIN2, and state-of-the-art long-read Nanopore sequencing (LR_NGS), together with analysis of respiratory cilia including transmission electron microscopy and immunofluorescence to address the underdiagnosis of HYDINas a cause of PCD. Bioinformatic masking of HYDIN2after SR-NGS facilitated the detection of biallelic HYDINvariants in 15 of 437 families, but compromised the detection of copy number variants. Supplementing testing with LR-NGS detected HYDINdeletions in 2 families, where SR-NGS had detected a single heterozygous HYDINvariant. LR-NGS was also able to confirm true homozygosity in 2 families when parental testing was not possible. Utilising a combined genomic diagnostic approach, biallelic HYDINvariants were detected in 17 families from 242 genetically confirmed PCD cases, comprising 7% of our PCD cohort. This represents the largest reported HYDINcohort to date and highlights previous underdiagnosis of HYDIN-associated PCD. Moreover this provides further evidence for the utility of LR-NGS in diagnostic testing, particularly for regions of high genomic complexity.

Published
2024
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50. Prevalence and course of disease after lung resection in primary ciliary dyskinesia: a cohort & nested case-control study

Author

Kouis, Panayiotis, Goutaki, Myrofora, Halbeisen, Florian S., Gioti, Ifigeneia, Middleton, Nicos, Amirav, Israel, on behalf of the Israeli PCD Consortium, Barbato, Angelo, on behalf of the Italian PCD Consortium, Behan, Laura, Boon, Mieke, Emiralioglu, Nagehan, Haarman, Eric G., Karadag, Bulent, Koerner-Rettberg, Cordula, Lazor, Romain, on behalf of the Swiss PCD Group, Loebinger, Michael R., Maitre, Bernard, on behalf of the French Reference Centre for Rare Lung Diseases, Mazurek, Henryk, Morgan, Lucy, Nielsen, Kim Gjerum, Omran, Heymut, Özçelik, Ugur, Price, Mareike, Pogorzelski, Andrzej, Snijders, Deborah, on behalf of the PCD Italian Consortium, Thouvenin, Guillaume, on behalf of the French Reference Centre for Rare Lung Diseases, Werner, Claudius, Zivkovic, Zorica, Kuehni, Claudia E., and Yiallouros, Panayiotis K.

Published
2019
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