dc.contributor.author | Svedberg, Marcus | |
dc.date.accessioned | 2022-05-23T15:15:03Z | |
dc.date.available | 2022-05-23T15:15:03Z | |
dc.date.issued | 2022-05-23 | |
dc.identifier.isbn | 978-91-8009-834-2 (PDF) | |
dc.identifier.isbn | 978-91-8009-833-5 (PRINT) | |
dc.identifier.uri | https://hdl.handle.net/2077/71506 | |
dc.description.abstract | Background: 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.iso | eng | en |
dc.relation.haspart | Svedberg 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.2017.08.004 | en |
dc.relation.haspart | Svedberg 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.2019.10.014 | en |
dc.relation.haspart | Svedberg 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-2021-218178 | en |
dc.relation.haspart | Svedberg 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.16302 | en |
dc.subject | cystic fibrosis | en |
dc.subject | children | en |
dc.subject | lung disease | en |
dc.title | Monitoring cystic fibrosis lung disease in children - Clinical utility and associations between functional and structural methods | en |
dc.type | text | eng |
dc.type.svep | Doctoral thesis | eng |
dc.gup.mail | marcus.svedberg@vgregion.se | en |
dc.type.degree | Doctor of Philosophy (Medicine) | en |
dc.gup.admin | ISBN nr var korrekt på den tryckta avhandlingen.
Jag har täckt över ansiktet på personerna på bilderna. | en |
dc.gup.origin | University of Gothenburg. Sahlgrenska Academy | en |
dc.gup.department | Institute of Clinical Sciences. Department of Pediatrics | en |
dc.gup.defenceplace | Fredagen den 2 september 2022, kl. 9.00, Konferenssalen Tallen, Drottning Silvias barnsjukhus, Remissvägen 12, Göteborg | en |
dc.gup.defencedate | 2022-09-02 | |
dc.gup.dissdb-fakultet | SA | |