Comparative demographics of the European cystic fibrosis population: a cross-sectional database analysis

McCormick, Jonathan; Mehta, Gita; Olesen, Hanne Vebert; Viviani, Laura; Maçek, Milan; Mehta, Anil

doi:10.1016/s0140-6736(09)62161-9

Cited by 118 publications

(100 citation statements)

References 16 publications

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“…Cystic fibrosis research has benefitted greatly from the creation of national and international networks such as the European Cystic Fibrosis Society (ECFS), the ECFS clinical trials network and the ECFS patient registry [115].…”

Section: Figurementioning

confidence: 99%

Management of bronchiectasis in adults

2015

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Formerly regarded as a rare disease, bronchiectasis is now increasingly recognised and a renewed interest in the condition is stimulating drug development and clinical research. Bronchiectasis represents the final common pathway of a number of infectious, genetic, autoimmune, developmental and allergic disorders and is highly heterogeneous in its aetiology, impact and prognosis.The goals of therapy should be: to improve airway mucus clearance through physiotherapy with or without adjunctive therapies; to suppress, eradicate and prevent airway bacterial colonisation; to reduce airway inflammation; and to improve physical functioning and quality of life.Fortunately, an increasing body of evidence supports interventions in bronchiectasis. The field has benefited greatly from the introduction of evidence-based guidelines in some European countries and randomised controlled trials have now demonstrated the benefit of long-term macrolide therapy, with accumulating evidence for inhaled therapies, physiotherapy and pulmonary rehabilitation.This review provides a critical update on the management of bronchiectasis focussing on emerging evidence and recent randomised controlled trials. @ERSpublications Bronchiectasis is a rapidly developing field: review of recent RCTs and progress towards developing new therapies

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Section: Figurementioning

confidence: 99%

Management of bronchiectasis in adults

2015

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“…Work in this area builds on existing protocols and best-practice recommendations produced by the leading database and registry initiatives represented within RDConnect, including the disease-specific networks established for neuromuscular diseases, 32 cystic fibrosis, 33 and Huntington's disease, 34 the recommendations of the European Platform for Rare Disease Registries project, and the rare disease common data elements developed and published by the NIH's Office of Rare Diseases Research, 35 all of which will be leveraged to provide an initial framework for participating registries. Best practices for information collection by biomaterial collections have been developed by established biobanks such as EuroBioBank, 36 the Telethon Network of Genetic Biobanks, 37 and BBMRI, and their further development will also be promoted by connection with the BioMedBridges initiative, whose mission statement includes "Building data bridges and services between biological and medical infrastructures in Europe", and which comprises the ten biological and medical sciences research infrastructures selected by the European Strategic Forum for Research Infrastructures.…”

Section: Generation Of Common Data Elements and Standard Operating Prmentioning

confidence: 99%

RD-Connect: An Integrated Platform Connecting Databases, Registries, Biobanks and Clinical Bioinformatics for Rare Disease Research

et al. 2014

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“…FARRELL [3] estimated the prevalence of CF in European Union (EU) countries, while MCCORMICK et al [4] and MEHTA et al [5] provided cross-sectional data on demographics of CF in EU and non-EU countries. The increased survival in CF patients resulted in the number of adults (age ⩾18 years) with CF being larger than the number of children in several EU countries with well-established healthcare systems [4,6]. In many non-EU countries, where allocation of resource to healthcare is lower, survival has also improved but the numbers of CF adults are still lower than those of CF children [4].…”

Section: Introductionmentioning

confidence: 99%

“…The increased survival in CF patients resulted in the number of adults (age ⩾18 years) with CF being larger than the number of children in several EU countries with well-established healthcare systems [4,6]. In many non-EU countries, where allocation of resource to healthcare is lower, survival has also improved but the numbers of CF adults are still lower than those of CF children [4]. Although these data shed light on changing epidemiology of CF, the impact of prolonged survival on future numbers of CF adults has not been evaluated.…”

Section: Introductionmentioning

confidence: 99%

Future trends in cystic fibrosis demography in 34 European countries

et al. 2015

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Median survival has increased in people with cystic fibrosis (CF) during the past six decades, which has led to an increased number of adults with CF. The future impact of changes in CF demographics has not been evaluated. The aim of this study was to estimate the number of children and adults with CF in 34 European countries by 2025.Data were obtained from the European Cystic Fibrosis Society Patient Registry. Population forecasts were performed for countries that have extensive CF population coverage and at least 4 years of longitudinal data by modelling future entering and exiting flows in registry cohorts. For the other countries, population projections were performed based on assumptions from knowledge of current CF epidemiology.Western European countries' forecasts indicate that an increase in the overall number of CF patients by 2025, by approximately 50%, corresponds to an increase by 20% and by 75% in children and adults, respectively. In Eastern European countries the projections suggest a predominant increase in the CF child population, although the CF adult population would also increase.It was concluded that a large increase in the adult CF population is expected in the next decade. A significant increase in adult CF services throughout Europe is urgently required. @ERSpublications A large increase in the number of CF adults is expected by 2025 that will require increase in adult CF care services http://ow.ly/INkvb

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Comparative demographics of the European cystic fibrosis population: a cross-sectional database analysis

Cited by 118 publications

References 16 publications

Management of bronchiectasis in adults

Management of bronchiectasis in adults

RD-Connect: An Integrated Platform Connecting Databases, Registries, Biobanks and Clinical Bioinformatics for Rare Disease Research

Future trends in cystic fibrosis demography in 34 European countries

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