Show simple item record

dc.contributor.authorSvedberg, Marcus
dc.date.accessioned2022-05-23T15:15:03Z
dc.date.available2022-05-23T15:15:03Z
dc.date.issued2022-05-23
dc.identifier.isbn978-91-8009-834-2 (PDF)
dc.identifier.isbn978-91-8009-833-5 (PRINT)
dc.identifier.urihttps://hdl.handle.net/2077/71506
dc.description.abstractBackground: Cystic Fibrosis (CF) is an inherited progressive disease that causes severe damage to the airways and other organs of the body. Many methods are available to track CF lung disease but longitudinal data are needed to better understand the clinical utility and associations between different methods. The main aim of this doctoral thesis was to analyse and compare longitudinal data from lung function tests and image tests in children with CF. Methods: In study I children aged 6–17 years attending Gothenburg’s CF clinic underwent multiple breath washout examinations, spirometry tests and a clinical stability assessment every 3rd month over a period of 1 year. Variability of the outcome parameters were analysed. In study II–IV 75 children aged 0–17 years underwent multiple breath washout examinations, spirometry tests, chest computer tomography (CT) and chest x-ray examinations between 1996– 2016 at Gothenburg CF centre. Longitudinal trends and associations between outcome measures were analysed together with the effect of respiratory infections and other confounding factors. Results: A total of 25 children completed a total of 107 visits of which 104 visits had complete data available in study I. The relative change in lung clearance index and FEV1% was +-17% (95th percentile) at clinical stable visits. In study II–IV a total of 75 participants with CF were included together with a healthy cohort of 140 children aged 0–17 years. Children with CF underwent lung functions tests and image tests and the healthy cohort only underwent multiple breath washout examinations. Study II demonstrated that intermittent and chronic infections were associated with an increased progression rate of structural lung disease measured with chest CT. Study III demonstrated associations between longitudinal LCI and the extent and progression rate of structural lung damage assessed with chest CT. The Lung clearance index was more sensitive than chest x-rays to detect early CF lung disease in study IV. The combined results of a normal chest x-ray and a normal lung clearance index were associated with a low extent of lung damage assessed with chest CT. Conclusions: Multiple breath washout is a sensitive method to detect early CF lung disease. Lung function and imagine tests captured different dimensions of CF lung disease. The use of multiple methods in clinical practice provides a more robust assessment of CF lung disease than using either measure alone.en
dc.language.isoengen
dc.relation.haspartSvedberg M, Gustafsson PM, Robinson PD, Rosberg M, Lindblad A. Variability of lung clearance index in clinically stable cystic fibrosis lung disease in school age children. Journal of cystic fibrosis. 2017:17:236-241. https://doi.org/10.1016/j.jcf.2017.08.004en
dc.relation.haspartSvedberg M, Gustafsson PM, Tiddens H, Imberg H, Piovic A, Lindblad A. Risk factors for progression of structural lung disease in school-age children with cystic fibrosis. Journal of cystic fibrosis. 2020;19:910-916. https://doi.org/10.1016/j.jcf.2019.10.014en
dc.relation.haspartSvedberg M, Imberg H, Gustafsson PM, Tiddens H, Davies G, Lindblad A. Longitudinal lung clearance index and association with structural lung damage in children with cystic fibrosis. Thorax. 2022 Mar 11;thoraxjnl-2021-218178. http://dx.doi.org/10.1136/thoraxjnl-2021-218178en
dc.relation.haspartSvedberg M, Imberg H, Gustafsson PM, Mela Brink, Håkan Caisander, Lindblad A. Chest x-rays are less sensitive than multiple breath washout examinations when it comes to detecting early cystic fibrosis lung disease. Acta Paediatr. 2022;00:1–8. https://doi.org/10.1111/apa.16302en
dc.subjectcystic fibrosisen
dc.subjectchildrenen
dc.subjectlung diseaseen
dc.titleMonitoring cystic fibrosis lung disease in children - Clinical utility and associations between functional and structural methodsen
dc.typetexteng
dc.type.svepDoctoral thesiseng
dc.gup.mailmarcus.svedberg@vgregion.seen
dc.type.degreeDoctor of Philosophy (Medicine)en
dc.gup.adminISBN nr var korrekt på den tryckta avhandlingen. Jag har täckt över ansiktet på personerna på bilderna.en
dc.gup.originUniversity of Gothenburg. Sahlgrenska Academyen
dc.gup.departmentInstitute of Clinical Sciences. Department of Pediatricsen
dc.gup.defenceplaceFredagen den 2 september 2022, kl. 9.00, Konferenssalen Tallen, Drottning Silvias barnsjukhus, Remissvägen 12, Göteborgen
dc.gup.defencedate2022-09-02
dc.gup.dissdb-fakultetSA


Files in this item

Thumbnail
Thumbnail
Thumbnail

This item appears in the following Collection(s)

Show simple item record