caa a22 4500 530059908 20181208073322.0 cr unu---uuuuu 181025e201812 xx s 000 0 eng 2329-6933 10.1513/AnnalsATS.201712-967OC doi (BORIS)oai:boris.unibe.ch:120536 610 ddc 360 ddc 30290127 pmid Structural and Functional Lung Impairment in PCD: Assessment with MRI and Multiple Breath Washout in Comparison to Spirometry [Elektronische Daten] [Sylvia Nyilas, Grzegorz Bauman, Orso Pusterla, Gregor Sommer, Florian Singer, Enno Stranzinger, Christoph Heyer, Kathryn Angela Ramsey, Anne Schlegtendal, Stefanie Benzrath, Carmen Casaulta, Myrofora Goutaki, Claudia E Kuehni, Oliver Bieri, Cordula Koerner-Rettberg, Philipp Latzin] publishedVersion openAccess eu-repo RATIONALE Primary ciliary dyskinesia (PCD) is an inherited disorder characterized by heterogeneous airway disease. Traditional lung function techniques (e.g. spirometry) may underestimate severity and complexity of PCD. OBJECTIVES We assessed lung impairment in individuals with PCD using structural and functional magnetic resonance imaging (MRI) and different lung function techniques. METHODS Thirty study participants with PCD (median 13.4 years, range 5-28) underwent structural and functional MRI, spirometry, and multiple breath washout (MBW) on the same day. Primary endpoints included structural MRI morphology scores, relative ventilation and perfusion impairment from functional MRI, FEV1 from spirometry, and lung clearance index (LCI) from MBW. RESULTS Severity and complexity of PCD lung disease varied significantly between individuals. Structural lung disease was detected in all subjects with a median (IQR) extent score of 10.3 (7 to 19; maximum score = 60). Functional MRI ventilation impairment was present in 52% of subjects affecting 24.2% (21.1 to 25.2%) of the lung. Relative perfusion impairment was detected in 78% of individuals affecting 21.1% (19.4 to 25.9%) of the lung. LCI was abnormal in 83% (median 8.3 (2.6 to 13.2) z-scores) and FEV1 was abnormal in 27% (-0.5 (-1.6 to 0.3) z-scores) of individuals. Concordance between spirometry and imaging outcomes was poor, with 52% of patients showing both abnormal MRI and LCI values, but normal FEV1. CONCLUSIONS Discordance between lung function and imaging outcomes in patients with PCD supports the use of both imaging and lung function, such as MBW, for surveillance of this heterogeneous disease. Nyilas Sylvia joint author Bauman Grzegorz joint author Pusterla Orso joint author Sommer Gregor joint author Singer Florian joint author Stranzinger Enno joint author Heyer Christoph joint author Ramsey Kathryn Angela joint author Schlegtendal Anne joint author Benzrath Stefanie joint author Casaulta Carmen joint author Goutaki Myrofora joint author Kuehni Claudia E. joint author Bieri Oliver joint author Koerner-Rettberg Cordula joint author Latzin Philipp joint author Annals of the American Thoracic Society 15(12), pp. 1434-1442 2329-6933 https://boris.unibe.ch/120536/7/Nyilas%20AnnAmThoracSoc%202018_manuscript.pdf text/html WWW-Backlink auf das Repository 1 info:eu-repo/semantics/article boris PeerReviewed boris peerstatus BORIS 856 40 https://boris.unibe.ch/120536/7/Nyilas%20AnnAmThoracSoc%202018_manuscript.pdf text/html WWW-Backlink auf das Repository BORIS 700 1- Nyilas Sylvia joint author BORIS 700 1- Bauman Grzegorz joint author BORIS 700 1- Pusterla Orso joint author BORIS 700 1- Sommer Gregor joint author BORIS 700 1- Singer Florian joint author BORIS 700 1- Stranzinger Enno joint author BORIS 700 1- Heyer Christoph joint author BORIS 700 1- Ramsey Kathryn Angela joint author BORIS 700 1- Schlegtendal Anne joint author BORIS 700 1- Benzrath Stefanie joint author BORIS 700 1- Casaulta Carmen joint author BORIS 700 1- Goutaki Myrofora joint author BORIS 700 1- Kuehni Claudia E. joint author BORIS 700 1- Bieri Oliver joint author BORIS 700 1- Koerner-Rettberg Cordula joint author BORIS 700 1- Latzin Philipp joint author BORIS 773 0- Annals of the American Thoracic Society 15(12), pp. 1434-1442 2329-6933 SWISSBIB 530059908 BK010053 XK010053 XK010000 BORIS BORIS BORIS info:eu-repo/semantics/article openAccess