期刊
EUROPEAN RESPIRATORY JOURNAL
卷 49, 期 1, 页码 -出版社
EUROPEAN RESPIRATORY SOC JOURNALS LTD
DOI: 10.1183/13993003.01090-2016
关键词
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资金
- European Union [8305404]
- NIHR Respiratory Biomedical Research Unit at the University Hospital Southampton NHS Foundation Trust (Southampton, UK)
- AAIR Charity
- NIHR Respiratory Disease Biomedical Research Unit at the Royal Brompton and Harefield NHS Foundation Trust (London, UK)
- Imperial College London (London, UK)
- Bernese Lung League
- Milena-Pro Kartagener foundation
- Swiss National Foundation [32003B_162820/1]
- NIH through the Genetic Disorders of Mucociliary Clearance Consortium [U54HL096458]
- NIH Office of Rare Diseases Research at the National Center for Advancing Translational Science
- National Heart, Lung and Blood Institute
- Swiss National Science Foundation (SNF) [32003B_162820] Funding Source: Swiss National Science Foundation (SNF)
- National Institute for Health Research [NF-SI-0514-10053, NIHR-HCS-P13-04-004] Funding Source: researchfish
- National Institutes of Health Research (NIHR) [NIHR-HCS-P13-04-004] Funding Source: National Institutes of Health Research (NIHR)
The diagnosis of primary ciliary dyskinesia is often confirmed with standard, albeit complex and expensive, tests. In many cases, however, the diagnosis remains difficult despite the array of sophisticated diagnostic tests. There is no gold standard reference test. Hence, a Task Force supported by the European Respiratory Society has developed this guideline to provide evidence-based recommendations on diagnostic testing, especially in light of new developments in such tests, and the need for robust diagnoses of patients who might enter randomised controlled trials of treatments. The guideline is based on pre-defined questions relevant for clinical care, a systematic review of the literature, and assessment of the evidence using the GRADE (Grading of Recommendations, Assessment, Development and Evaluation) approach. It focuses on clinical presentation, nasal nitric oxide, analysis of ciliary beat frequency and pattern by high-speed video-microscopy analysis, transmission electron microscopy, genotyping and immunofluorescence. It then used a modified Delphi survey to develop an algorithm for the use of diagnostic tests to definitively confirm and exclude the diagnosis of primary ciliary dyskinesia; and to provide advice when the diagnosis was not conclusive. Finally, this guideline proposes a set of quality criteria for future research on the validity of diagnostic methods for primary ciliary dyskinesia.
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