Lucy Morgan | The University of Sydney (original) (raw)

Papers by Lucy Morgan

Journal of Clinical Medicine

Neonatal respiratory distress (NRD) is common among infants with primary ciliary dyskinesia (PCD)... more Neonatal respiratory distress (NRD) is common among infants with primary ciliary dyskinesia (PCD), but we do not know whether affected neonates receive a timely diagnosis. We used data from the international PCD cohort and assessed the proportion of patients with PCD who had a history of NRD and their age at diagnosis, stratifying by presence of laterality defects. First we analyzed data from all participants diagnosed after 2000, followed by individuals from a subgroup diagnosed using stricter criteria. Among the 1375 patients in the study, 45% had a history of NRD and 42% had laterality defects. Out of the 476 children with definite PCD diagnosis, 55% had a history of NRD and 50% had laterality defects. Overall, 30% of children with PCD were diagnosed during the first 12 months of life. This varied from 13% in those with situs solitus and no NRD, to 21% in those with situs solitus and NRD, 33% in those with situs anomalies but no NRD, and 52% in those with both situs anomalies and...

American Journal of Respiratory and Critical Care Medicine

The Journal of heart and lung transplantation : the official publication of the International Society for Heart Transplantation, 2018

Lung transplantation provides a unique opportunity to investigate the dynamics of the human pulmo... more Lung transplantation provides a unique opportunity to investigate the dynamics of the human pulmonary virome that is transplanted within the donor lungs. The pulmonary virome comprises both "resident" and "transient" viruses. In this study we aimed to analyze the dynamics of the "transient" members. We conducted a single-center, prospective, longitudinal investigation of community-acquired respiratory viruses detected in nasopharyngeal swabs, swabs of explanted and donor lungs, and serial bronchoalveolar lavages post-transplant. Fifty-two consecutive lung transplant recipients were recruited (bilateral:heart‒lung:bilateral lung-liver = 48:2:2) (age [mean ± SD] 48 ± 15 years, range 20 to 63 years; 27 males and 25 females). Follow-up was 344 ± 120 (range 186 to 534) days. Seventeen of 45 explanted lungs were positive for influenza A and/or B (A = 14, B = 2, A+B = 1), despite recipient vaccination and negative nasal swabs, and 4 of 45 had human rhinovirus ...

The European respiratory journal, 2018

Primary ciliary dyskinesia (PCD) has been considered a relatively mild disease, especially compar... more Primary ciliary dyskinesia (PCD) has been considered a relatively mild disease, especially compared to cystic fibrosis (CF), but studies on lung function in PCD patients have been few and small.This study compared lung function from spirometry of PCD patients to normal reference values and to published data from CF patients. We calculated z-scores and % predicted values for forced expiratory volume in 1 s (FEV) and forced vital capacity (FVC) using the Global Lung Function Initiative 2012 values for 991 patients from the international PCD Cohort. We then assessed associations with age, sex, country, diagnostic certainty, organ laterality, body mass index and age at diagnosis in linear regression models. Lung function in PCD patients was reduced compared to reference values in both sexes and all age groups. Children aged 6-9 years had the smallest impairment (FEV z-score -0.84 (-1.03 to -0.65), FVC z-score -0.31 (-0.51 to -0.11)). Compared to CF patients, FEV was similarly reduced in...

Multidisciplinary respiratory medicine, 2018

The contemporary diagnosis of bronchiectasis requires CT scanning to describe specific structural... more The contemporary diagnosis of bronchiectasis requires CT scanning to describe specific structural lung changes. Scoring systems have been designed and validated in some specific causes of bronchiectasis to allow investigators to correlate CT changes with other indices of disease severity, to describe changes over time, with exacerbation and in response to treatment. Several scoring systems have been developed for CF including the Bhalla Score, Brody score, and the Helbich score. These scoring systems have also been applied to other causes of bronchiectasis including Primary Ciliary Dyskinesia (PCD). This assumes that the nature of structural lung disease in these conditions, as well as the rate and nature of longitudinal changes are identical to, or at least very similar to, those found in CF. This assumption has not been tested. The underlying pathophysiology of PCD is not the same as CF and may cause bronchiectasis that is radiologically similar but not necessarily the same as CF ...

Heart, lung & circulation, Jan 11, 2018

Increased access to reperfusion for ST elevation myocardial infarction (STEMI) has contributed to... more Increased access to reperfusion for ST elevation myocardial infarction (STEMI) has contributed to reduced mortality internationally. We describe temporal trends in pre-hospital care, in-hospital management and outcomes of the STEMI population in Australia. Temporal trends with multiple regression analysis on the management and outcomes of STEMI patients enrolled across 46 Australian hospitals in the Australian cohort of the Global Registry of Acute Coronary Events (GRACE) and the Cooperative National Registry of Acute Coronary Care Guideline Adherence and Clinical Events (CONCORDANCE) between February 1999 and August 2016. 4110 patients were treated for STEMI, mean age 62.5±13.7years (SD). The median door-to-balloon time of primary percutaneous coronary intervention (PPCI) decreased by 11minutes (p<0.01) although there was no increase in rates of PPCI (p=0.35). Access to non-primary PCI increased by 39% (p<0.01), provisioning of fibrinolysis decreased by 13% (p<0.01) and th...

BMC pulmonary medicine, Jan 22, 2018

Bronchiectasis is a chronic respiratory condition. Persistent bacterial colonisation in the stabl... more Bronchiectasis is a chronic respiratory condition. Persistent bacterial colonisation in the stable state with increased and sometimes altered bacterial burden during exacerbations are accepted as key features in the pathophysiology. The extent to which respiratory viruses are present during stable periods and in exacerbations is less well understood. This study aimed to determine the incidence of respiratory viruses within a cohort of bronchiectasis patients with acute exacerbations at a teaching hospital and, separately, in a group of patients with stable bronchiectasis. In the group of stable patients, a panel of respiratory viruses were assayed for using real time quantitative PCR in respiratory secretions and exhaled breath. The Impact of virus detection on exacerbation rates and development of symptomatic infection was evaluated. Routine hospital-based viral PCR testing was only requested in 28% of admissions for an exacerbation. In our cohort of stable bronchiectasis patients,...

American journal of respiratory and critical care medicine, Jan 15, 2018

This document presents the American Thoracic Society clinical practice guidelines for the diagnos... more This document presents the American Thoracic Society clinical practice guidelines for the diagnosis of primary ciliary dyskinesia (PCD). Clinicians investigating adult and pediatric patients for possible PCD. Systematic reviews and, when appropriate, meta-analyses were conducted to summarize all available evidence pertinent to our clinical questions. Evidence was assessed using the GRADE (Grading of Recommendations, Assessment, Development and Evaluation) approach for diagnosis and discussed by a multidisciplinary panel with expertise in PCD. Predetermined conflict-of-interest management strategies were applied, and recommendations were formulated, written, and graded exclusively by the nonconflicted panelists. Three conflicted individuals were also prohibited from writing, editing, or providing feedback on the relevant sections of the manuscript. After considering diagnostic test accuracy, confidence in the estimates for each diagnostic test, relative importance of test results stu...

mSphere, Apr 25, 2018

Long-term macrolide therapy reduces rates of pulmonary exacerbation in bronchiectasis. However, l... more Long-term macrolide therapy reduces rates of pulmonary exacerbation in bronchiectasis. However, little is known about the potential for macrolide therapy to alter the composition and function of the oropharyngeal commensal microbiota or to increase the carriage of transmissible antimicrobial resistance. We assessed the effect of long-term erythromycin on oropharyngeal microbiota composition and the carriage of transmissible macrolide resistance genes in 84 adults with bronchiectasis, enrolled in the Bronchiectasis and Low-dose Erythromycin Study (BLESS) 48-week placebo-controlled trial of twice-daily erythromycin ethylsuccinate (400 mg). Oropharyngeal microbiota composition and macrolide resistance gene carriage were determined by 16S rRNA gene amplicon sequencing and quantitative PCR, respectively. Long-term erythromycin treatment was associated with a significant increase in the relative abundance of oropharyngeal ( = 0.041) and with significant decreases in the relative abundance...

The European respiratory journal, Dec 1, 2017

Chronic respiratory disease can affect growth and nutrition, which can influence lung function. W... more Chronic respiratory disease can affect growth and nutrition, which can influence lung function. We investigated height, body mass index (BMI), and lung function in patients with primary ciliary dyskinesia (PCD).In this study, based on the international PCD (iPCD) Cohort, we calculated z-scores for height and BMI using World Health Organization (WHO) and national growth references, and assessed associations with age, sex, country, diagnostic certainty, age at diagnosis, organ laterality and lung function in multilevel regression models that accounted for repeated measurements.We analysed 6402 measurements from 1609 iPCD Cohort patients. Height was reduced compared to WHO (z-score -0.12, 95% CI -0.17 to -0.06) and national references (z-score -0.27, 95% CI -0.33 to -0.21) in male and female patients in all age groups, with variation between countries. Height and BMI were higher in patients diagnosed earlier in life (p=0.026 and p<0.001, respectively) and closely associated with for...

Breathe

In contrast to airway diseases like chronic obstructive pulmonary disease or asthma, and rare dis... more In contrast to airway diseases like chronic obstructive pulmonary disease or asthma, and rare diseases such as cystic fibrosis, there has been little research and few clinical trials in bronchiectasis. Guidelines are primarily based on expert opinion and treatment is challenging because of the heterogeneous nature of the disease.In an effort to address decades of underinvestment in bronchiectasis research, education and clinical care, the European Multicentre Bronchiectasis Audit and Research Collaboration (EMBARC) was established in 2012 as a collaborative pan-European network to bring together bronchiectasis researchers. The European Respiratory Society officially funded EMBARC in 2013 as a Clinical Research Collaboration, providing support and infrastructure to allow the project to grow.EMBARC has now established an international bronchiectasis registry that is active in more than 30 countries both within and outside Europe. Beyond the registry, the network participates in design...

Respiratory care, Jan 20, 2017

Airway clearance techniques are a vital part of routine care for patients with bronchiectasis. Th... more Airway clearance techniques are a vital part of routine care for patients with bronchiectasis. There is no clear superior modality. The Flutter combines oscillations (6-20Hz) and positive expiratory pressure; the Lung Flute combines positive expiratory pressure and low frequency acoustic waves (18-22Hz), to augment clearance. This project aimed to compare these devices. This was a randomized crossover study of adult subjects with stable non-cystic fibrosis bronchiectasis (expectorating >25 mL/d). Subjects attended 2 separate out-patient visits, 1 week apart, and completed a supervised sputum clearance regime and Lickert scale (8 questions regarding subjects' perception of the experience using each device). Total sputum expectorated during supervised intervention (T1) and after 30 min from the end of T1 (T2) was recorded as wet sputum weight. Total wet sputum weight desiccated in a microwave (10 min at 300 watts), allowed measurement of total dry sputum weight. Data were compa...

Respirology (Carlton, Vic.), Jan 30, 2017

This review article focuses on common lower respiratory infections (LRIs) in indigenous populatio... more This review article focuses on common lower respiratory infections (LRIs) in indigenous populations in both developed and developing countries, where data is available. Indigenous populations across the world share some commonalities including poorer health and socio-economic disadvantage compared with their non-indigenous counterparts. Generally, acute and chronic respiratory infections are more frequent and more severe in both indigenous children and adults, often resulting in substantial consequences including higher rates of bronchiectasis and poorer outcomes for patients with chronic obstructive pulmonary disease (COPD). Risk factors for the development of respiratory infections require recognition and action. These risk factors include but are not limited to socio-economic factors (e.g. education, household crowding and nutrition), environmental factors (e.g. smoke exposure and poor access to health care) and biological factors. Risk mitigation strategies should be delivered i...

The European respiratory journal, Jun 1, 2017

There is a need for a clear definition of exacerbations used in clinical trials in patients with ... more There is a need for a clear definition of exacerbations used in clinical trials in patients with bronchiectasis. An expert conference was convened to develop a consensus definition of an exacerbation for use in clinical research.A systematic review of exacerbation definitions used in clinical trials from January 2000 until December 2015 and involving adults with bronchiectasis was conducted. A Delphi process followed by a round-table meeting involving bronchiectasis experts was organised to reach a consensus definition. These experts came from Europe (representing the European Multicentre Bronchiectasis Research Collaboration), North America (representing the US Bronchiectasis Research Registry/COPD Foundation), Australasia and South Africa.The definition was unanimously approved by the working group as: a person with bronchiectasis with a deterioration in three or more of the following key symptoms for at least 48 h: cough; sputum volume and/or consistency; sputum purulence; breath...

The European respiratory journal, 2017

European Respiratory Journal, 2016

The European respiratory journal, 2017

Data on primary ciliary dyskinesia (PCD) epidemiology is scarce and published studies are charact... more 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. Childr...

European Respiratory Journal, 2016

Journal of Clinical Medicine

Neonatal respiratory distress (NRD) is common among infants with primary ciliary dyskinesia (PCD)... more Neonatal respiratory distress (NRD) is common among infants with primary ciliary dyskinesia (PCD), but we do not know whether affected neonates receive a timely diagnosis. We used data from the international PCD cohort and assessed the proportion of patients with PCD who had a history of NRD and their age at diagnosis, stratifying by presence of laterality defects. First we analyzed data from all participants diagnosed after 2000, followed by individuals from a subgroup diagnosed using stricter criteria. Among the 1375 patients in the study, 45% had a history of NRD and 42% had laterality defects. Out of the 476 children with definite PCD diagnosis, 55% had a history of NRD and 50% had laterality defects. Overall, 30% of children with PCD were diagnosed during the first 12 months of life. This varied from 13% in those with situs solitus and no NRD, to 21% in those with situs solitus and NRD, 33% in those with situs anomalies but no NRD, and 52% in those with both situs anomalies and...

American Journal of Respiratory and Critical Care Medicine

The Journal of heart and lung transplantation : the official publication of the International Society for Heart Transplantation, 2018

Lung transplantation provides a unique opportunity to investigate the dynamics of the human pulmo... more Lung transplantation provides a unique opportunity to investigate the dynamics of the human pulmonary virome that is transplanted within the donor lungs. The pulmonary virome comprises both "resident" and "transient" viruses. In this study we aimed to analyze the dynamics of the "transient" members. We conducted a single-center, prospective, longitudinal investigation of community-acquired respiratory viruses detected in nasopharyngeal swabs, swabs of explanted and donor lungs, and serial bronchoalveolar lavages post-transplant. Fifty-two consecutive lung transplant recipients were recruited (bilateral:heart‒lung:bilateral lung-liver = 48:2:2) (age [mean ± SD] 48 ± 15 years, range 20 to 63 years; 27 males and 25 females). Follow-up was 344 ± 120 (range 186 to 534) days. Seventeen of 45 explanted lungs were positive for influenza A and/or B (A = 14, B = 2, A+B = 1), despite recipient vaccination and negative nasal swabs, and 4 of 45 had human rhinovirus ...

The European respiratory journal, 2018

Primary ciliary dyskinesia (PCD) has been considered a relatively mild disease, especially compar... more Primary ciliary dyskinesia (PCD) has been considered a relatively mild disease, especially compared to cystic fibrosis (CF), but studies on lung function in PCD patients have been few and small.This study compared lung function from spirometry of PCD patients to normal reference values and to published data from CF patients. We calculated z-scores and % predicted values for forced expiratory volume in 1 s (FEV) and forced vital capacity (FVC) using the Global Lung Function Initiative 2012 values for 991 patients from the international PCD Cohort. We then assessed associations with age, sex, country, diagnostic certainty, organ laterality, body mass index and age at diagnosis in linear regression models. Lung function in PCD patients was reduced compared to reference values in both sexes and all age groups. Children aged 6-9 years had the smallest impairment (FEV z-score -0.84 (-1.03 to -0.65), FVC z-score -0.31 (-0.51 to -0.11)). Compared to CF patients, FEV was similarly reduced in...

Multidisciplinary respiratory medicine, 2018

The contemporary diagnosis of bronchiectasis requires CT scanning to describe specific structural... more The contemporary diagnosis of bronchiectasis requires CT scanning to describe specific structural lung changes. Scoring systems have been designed and validated in some specific causes of bronchiectasis to allow investigators to correlate CT changes with other indices of disease severity, to describe changes over time, with exacerbation and in response to treatment. Several scoring systems have been developed for CF including the Bhalla Score, Brody score, and the Helbich score. These scoring systems have also been applied to other causes of bronchiectasis including Primary Ciliary Dyskinesia (PCD). This assumes that the nature of structural lung disease in these conditions, as well as the rate and nature of longitudinal changes are identical to, or at least very similar to, those found in CF. This assumption has not been tested. The underlying pathophysiology of PCD is not the same as CF and may cause bronchiectasis that is radiologically similar but not necessarily the same as CF ...

Heart, lung & circulation, Jan 11, 2018

Increased access to reperfusion for ST elevation myocardial infarction (STEMI) has contributed to... more Increased access to reperfusion for ST elevation myocardial infarction (STEMI) has contributed to reduced mortality internationally. We describe temporal trends in pre-hospital care, in-hospital management and outcomes of the STEMI population in Australia. Temporal trends with multiple regression analysis on the management and outcomes of STEMI patients enrolled across 46 Australian hospitals in the Australian cohort of the Global Registry of Acute Coronary Events (GRACE) and the Cooperative National Registry of Acute Coronary Care Guideline Adherence and Clinical Events (CONCORDANCE) between February 1999 and August 2016. 4110 patients were treated for STEMI, mean age 62.5±13.7years (SD). The median door-to-balloon time of primary percutaneous coronary intervention (PPCI) decreased by 11minutes (p<0.01) although there was no increase in rates of PPCI (p=0.35). Access to non-primary PCI increased by 39% (p<0.01), provisioning of fibrinolysis decreased by 13% (p<0.01) and th...

BMC pulmonary medicine, Jan 22, 2018

Bronchiectasis is a chronic respiratory condition. Persistent bacterial colonisation in the stabl... more Bronchiectasis is a chronic respiratory condition. Persistent bacterial colonisation in the stable state with increased and sometimes altered bacterial burden during exacerbations are accepted as key features in the pathophysiology. The extent to which respiratory viruses are present during stable periods and in exacerbations is less well understood. This study aimed to determine the incidence of respiratory viruses within a cohort of bronchiectasis patients with acute exacerbations at a teaching hospital and, separately, in a group of patients with stable bronchiectasis. In the group of stable patients, a panel of respiratory viruses were assayed for using real time quantitative PCR in respiratory secretions and exhaled breath. The Impact of virus detection on exacerbation rates and development of symptomatic infection was evaluated. Routine hospital-based viral PCR testing was only requested in 28% of admissions for an exacerbation. In our cohort of stable bronchiectasis patients,...

American journal of respiratory and critical care medicine, Jan 15, 2018

This document presents the American Thoracic Society clinical practice guidelines for the diagnos... more This document presents the American Thoracic Society clinical practice guidelines for the diagnosis of primary ciliary dyskinesia (PCD). Clinicians investigating adult and pediatric patients for possible PCD. Systematic reviews and, when appropriate, meta-analyses were conducted to summarize all available evidence pertinent to our clinical questions. Evidence was assessed using the GRADE (Grading of Recommendations, Assessment, Development and Evaluation) approach for diagnosis and discussed by a multidisciplinary panel with expertise in PCD. Predetermined conflict-of-interest management strategies were applied, and recommendations were formulated, written, and graded exclusively by the nonconflicted panelists. Three conflicted individuals were also prohibited from writing, editing, or providing feedback on the relevant sections of the manuscript. After considering diagnostic test accuracy, confidence in the estimates for each diagnostic test, relative importance of test results stu...

mSphere, Apr 25, 2018

Long-term macrolide therapy reduces rates of pulmonary exacerbation in bronchiectasis. However, l... more Long-term macrolide therapy reduces rates of pulmonary exacerbation in bronchiectasis. However, little is known about the potential for macrolide therapy to alter the composition and function of the oropharyngeal commensal microbiota or to increase the carriage of transmissible antimicrobial resistance. We assessed the effect of long-term erythromycin on oropharyngeal microbiota composition and the carriage of transmissible macrolide resistance genes in 84 adults with bronchiectasis, enrolled in the Bronchiectasis and Low-dose Erythromycin Study (BLESS) 48-week placebo-controlled trial of twice-daily erythromycin ethylsuccinate (400 mg). Oropharyngeal microbiota composition and macrolide resistance gene carriage were determined by 16S rRNA gene amplicon sequencing and quantitative PCR, respectively. Long-term erythromycin treatment was associated with a significant increase in the relative abundance of oropharyngeal ( = 0.041) and with significant decreases in the relative abundance...

The European respiratory journal, Dec 1, 2017

Chronic respiratory disease can affect growth and nutrition, which can influence lung function. W... more Chronic respiratory disease can affect growth and nutrition, which can influence lung function. We investigated height, body mass index (BMI), and lung function in patients with primary ciliary dyskinesia (PCD).In this study, based on the international PCD (iPCD) Cohort, we calculated z-scores for height and BMI using World Health Organization (WHO) and national growth references, and assessed associations with age, sex, country, diagnostic certainty, age at diagnosis, organ laterality and lung function in multilevel regression models that accounted for repeated measurements.We analysed 6402 measurements from 1609 iPCD Cohort patients. Height was reduced compared to WHO (z-score -0.12, 95% CI -0.17 to -0.06) and national references (z-score -0.27, 95% CI -0.33 to -0.21) in male and female patients in all age groups, with variation between countries. Height and BMI were higher in patients diagnosed earlier in life (p=0.026 and p<0.001, respectively) and closely associated with for...

Breathe

In contrast to airway diseases like chronic obstructive pulmonary disease or asthma, and rare dis... more In contrast to airway diseases like chronic obstructive pulmonary disease or asthma, and rare diseases such as cystic fibrosis, there has been little research and few clinical trials in bronchiectasis. Guidelines are primarily based on expert opinion and treatment is challenging because of the heterogeneous nature of the disease.In an effort to address decades of underinvestment in bronchiectasis research, education and clinical care, the European Multicentre Bronchiectasis Audit and Research Collaboration (EMBARC) was established in 2012 as a collaborative pan-European network to bring together bronchiectasis researchers. The European Respiratory Society officially funded EMBARC in 2013 as a Clinical Research Collaboration, providing support and infrastructure to allow the project to grow.EMBARC has now established an international bronchiectasis registry that is active in more than 30 countries both within and outside Europe. Beyond the registry, the network participates in design...

Respiratory care, Jan 20, 2017

Airway clearance techniques are a vital part of routine care for patients with bronchiectasis. Th... more Airway clearance techniques are a vital part of routine care for patients with bronchiectasis. There is no clear superior modality. The Flutter combines oscillations (6-20Hz) and positive expiratory pressure; the Lung Flute combines positive expiratory pressure and low frequency acoustic waves (18-22Hz), to augment clearance. This project aimed to compare these devices. This was a randomized crossover study of adult subjects with stable non-cystic fibrosis bronchiectasis (expectorating >25 mL/d). Subjects attended 2 separate out-patient visits, 1 week apart, and completed a supervised sputum clearance regime and Lickert scale (8 questions regarding subjects' perception of the experience using each device). Total sputum expectorated during supervised intervention (T1) and after 30 min from the end of T1 (T2) was recorded as wet sputum weight. Total wet sputum weight desiccated in a microwave (10 min at 300 watts), allowed measurement of total dry sputum weight. Data were compa...

Respirology (Carlton, Vic.), Jan 30, 2017

This review article focuses on common lower respiratory infections (LRIs) in indigenous populatio... more This review article focuses on common lower respiratory infections (LRIs) in indigenous populations in both developed and developing countries, where data is available. Indigenous populations across the world share some commonalities including poorer health and socio-economic disadvantage compared with their non-indigenous counterparts. Generally, acute and chronic respiratory infections are more frequent and more severe in both indigenous children and adults, often resulting in substantial consequences including higher rates of bronchiectasis and poorer outcomes for patients with chronic obstructive pulmonary disease (COPD). Risk factors for the development of respiratory infections require recognition and action. These risk factors include but are not limited to socio-economic factors (e.g. education, household crowding and nutrition), environmental factors (e.g. smoke exposure and poor access to health care) and biological factors. Risk mitigation strategies should be delivered i...

The European respiratory journal, Jun 1, 2017

There is a need for a clear definition of exacerbations used in clinical trials in patients with ... more There is a need for a clear definition of exacerbations used in clinical trials in patients with bronchiectasis. An expert conference was convened to develop a consensus definition of an exacerbation for use in clinical research.A systematic review of exacerbation definitions used in clinical trials from January 2000 until December 2015 and involving adults with bronchiectasis was conducted. A Delphi process followed by a round-table meeting involving bronchiectasis experts was organised to reach a consensus definition. These experts came from Europe (representing the European Multicentre Bronchiectasis Research Collaboration), North America (representing the US Bronchiectasis Research Registry/COPD Foundation), Australasia and South Africa.The definition was unanimously approved by the working group as: a person with bronchiectasis with a deterioration in three or more of the following key symptoms for at least 48 h: cough; sputum volume and/or consistency; sputum purulence; breath...

The European respiratory journal, 2017

European Respiratory Journal, 2016

The European respiratory journal, 2017

Data on primary ciliary dyskinesia (PCD) epidemiology is scarce and published studies are charact... more 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. Childr...

European Respiratory Journal, 2016