Variability of clinically measured lung clearance index in children with cystic fibrosis.

Frauchiger, Bettina S; Ramsey, Kathryn A; Usemann, Jakob; Kieninger, Elisabeth; Casaulta, Carmen; Sirtes, Daniel; Yammine, Sophie; Spycher, Ben; Moeller, Alexander; Latzin, Philipp (2023). Variability of clinically measured lung clearance index in children with cystic fibrosis. Pediatric pulmonology, 58(1), pp. 197-205. Wiley-Blackwell 10.1002/ppul.26180

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RATIONALE

The lung clearance index (LCI) is increasingly being used in the clinical surveillance of patients with cystic fibrosis (CF). However, there are limited data on long-term variability and clinically relevant changes in LCI during routine clinical surveillance.

OBJECTIVES

To evaluate long-term variability of LCI and propose a threshold for a physiologically relevant change.

METHODS

Children with CF aged 4-18 years performed LCI measurements every three months as part of routine clinical surveillance during 2011-2020 in two centers. The variability of LCI during periods of clinical stability was assessed using mixed-effects models and was used to identify thresholds for clinically relevant changes.

RESULTS

Repeated LCI measurements of acceptable quality (N= 858) were available in 100 patients with CF, for 74 patients 399 visits at clinical stability were available. Variability of repeated LCI measurements over time expressed as coefficient of variation (CV%) was 7.4%. The upper limit of normal (ULN) for relative changes in LCI between visits was 19%.

CONCLUSION

We report the variability of LCI in children and adolescents with CF during routine clinical surveillance. According to our data, a change in LCI beyond 19% may be considered physiologically relevant. These findings will help guide clinical decisions according to LCI changes. This article is protected by copyright. All rights reserved.

Item Type:

Journal Article (Original Article)

Division/Institute:

04 Faculty of Medicine > Department of Gynaecology, Paediatrics and Endocrinology (DFKE) > Clinic of Paediatric Medicine
04 Faculty of Medicine > Pre-clinic Human Medicine > Institute of Social and Preventive Medicine (ISPM)
04 Faculty of Medicine > Pre-clinic Human Medicine > BioMedical Research (DBMR) > Unit Childrens Hospital > Forschungsgruppe Pneumologie (Pädiatrie)
04 Faculty of Medicine > Department of Gynaecology, Paediatrics and Endocrinology (DFKE) > Clinic of Paediatric Medicine > Paediatric Pneumology

UniBE Contributor:

Frauchiger, Bettina Sarah, Ramsey, Kathryn Angela, Kieninger, Elisabeth, Casaulta, Carmen, Sirtes, Daniel, Yammine, Sophie, Spycher, Ben, Latzin, Philipp

Subjects:

600 Technology > 610 Medicine & health
300 Social sciences, sociology & anthropology > 360 Social problems & social services

ISSN:

8755-6863

Publisher:

Wiley-Blackwell

Funders:

[4] Swiss National Science Foundation

Language:

English

Submitter:

Anette van Dorland

Date Deposited:

18 Oct 2022 09:58

Last Modified:

06 Jan 2023 11:57

Publisher DOI:

10.1002/ppul.26180

PubMed ID:

36251441

Uncontrolled Keywords:

Lung children cystic fibrosis

BORIS DOI:

10.48350/173838

URI:

https://boris.unibe.ch/id/eprint/173838

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