Comparison of the Lung Clearance Index in Preschool Children With Primary Ciliary Dyskinesia and Cystic Fibrosis

Jobst F Roehmel; Friederike J Doerfler; Cordula Koerner-Rettberg; Folke Brinkmann; Anne Schlegtendal; Martin Wetzke; Isa Rudolf; Simone Helms; Joerg Große-Onnebrink; Yin Yu; Thomas Nuesslein; Irena Wojsyk-Banaszak; Sebastian Becker; Olaf Eickmeier; Olaf Sommerburg; Heymut Omran; Mirjam Stahl; Marcus A Mall

doi:10.1016/j.chest.2022.02.052

Comparison of the Lung Clearance Index in Preschool Children With Primary Ciliary Dyskinesia and Cystic Fibrosis

Chest. 2022 Sep;162(3):534-542. doi: 10.1016/j.chest.2022.02.052. Epub 2022 Mar 7.

Authors

Jobst F Roehmel¹, Friederike J Doerfler¹, Cordula Koerner-Rettberg², Folke Brinkmann³, Anne Schlegtendal³, Martin Wetzke⁴, Isa Rudolf⁴, Simone Helms⁵, Joerg Große-Onnebrink⁵, Yin Yu⁶, Thomas Nuesslein⁷, Irena Wojsyk-Banaszak⁸, Sebastian Becker⁹, Olaf Eickmeier¹⁰, Olaf Sommerburg⁶, Heymut Omran⁵, Mirjam Stahl¹¹, Marcus A Mall¹²

Affiliations

¹ Department of Pediatric Respiratory Medicine, Immunology and Critical Care Medicine, Berlin, Germany.
² Department of Pediatric Pulmonology, University Children's Hospital of Ruhr University Bochum, Bochum, Germany; Department of General Pediatrics, Marienhospital Wesel, Wesel, Germany.
³ Department of Pediatric Pulmonology, University Children's Hospital of Ruhr University Bochum, Bochum, Germany.
⁴ Department of Pediatric Pulmonology, Allergology and Neonatology, Hannover Medical School, Hannover, Germany; Biomedical Research in End-Stage and Obstructive Lung Disease Hannover, German Center for Lung Research, Hannover Medical University, Hannover, Germany.
⁵ Department of General Pediatrics, University Children's Hospital Muenster, Muenster, Germany.
⁶ Division of Pediatric Pulmonology and Allergy and Cystic Fibrosis Center, Department of Pediatrics, University of Heidelberg, Heidelberg, Germany; Translational Lung Research Center Heidelberg, German Center for Lung Research, University of Heidelberg, Heidelberg, Germany.
⁷ Department of General Pediatrics, Gemeinschaftsklinikum Mittelrhein, Koblenz, Germany.
⁸ Department of Pulmonology, Pediatric Allergy and Clinical Immunology, Poznań University of Medical Sciences, Poznań, Poland.
⁹ Darmstädter Kinderkliniken Prinzessin Margaret, Darmstadt, Germany.
¹⁰ Department of Pediatrics, Division of Allergology, Pulmonology, and Cystic Fibrosis, Goethe University, Frankfurt, Germany.
¹¹ Department of Pediatric Respiratory Medicine, Immunology and Critical Care Medicine, Berlin, Germany; Division of Pediatric Pulmonology and Allergy and Cystic Fibrosis Center, Department of Pediatrics, University of Heidelberg, Heidelberg, Germany; Translational Lung Research Center Heidelberg, German Center for Lung Research, University of Heidelberg, Heidelberg, Germany; Berlin Institute of Health, Charité-Universitätsmedizin Berlin, Freie Universität Berlin and Humboldt-Universität zu Berlin, Berlin, Germany; German Center for Lung Research, associated partner site, Berlin, Germany.
¹² Department of Pediatric Respiratory Medicine, Immunology and Critical Care Medicine, Berlin, Germany; Berlin Institute of Health, Charité-Universitätsmedizin Berlin, Freie Universität Berlin and Humboldt-Universität zu Berlin, Berlin, Germany; German Center for Lung Research, associated partner site, Berlin, Germany. Electronic address: marcus.mall@charite.de.

PMID: 35271842
DOI: 10.1016/j.chest.2022.02.052

Abstract

Background: Previous studies showed that the lung clearance index (LCI) determined by multiple-breath washout (MBW) is sensitive to detecting early lung disease in preschool children with cystic fibrosis (CF). In preschool children with primary ciliary dyskinesia (PCD), data on the onset and severity of lung disease and on the sensitivity of the LCI as a noninvasive quantitative outcome measure remain limited.

Research and study question: Is MBW feasible and sensitive to detect ventilation inhomogeneity in preschool children with PCD?

Study design and methods: This was a prospective, cross-sectional, multicenter study and included preschoolers with PCD, preschoolers with CF, and healthy control (HC) participants. LCI was determined using nitrogen MBW and was compared among the three groups.

Results: LCI was determined in 27 children with PCD, 34 children with CF, and 30 HC participants (mean age, 4.8 years; range, 2.2-6.9 years). The LCI in preschool children with PCD was increased (median, 9.1; 95% CI, 8.6-10.3) compared with HC participants (median, 7.0; 95% CI, 6.7-7.1; P < .0001), but did not differ from preschool children with CF (median, 8.6; 95% CI, 8.4-9.7; P = .71). The feasibility in the PCD group was 93.1% and was similar to that in the CF group (91.9%) and in HC participants (85.7%; P = .55).

Interpretation: This study demonstrated early onset of lung disease in preschool children with PCD and indicated that lung disease severity in PCD may be similar to that in CF during preschool years. These data support a need for early diagnostic monitoring and therapy and suggest the LCI as a noninvasive diagnostic tool and as a potential end point in clinical trials testing early interventions in children with PCD.

Keywords: cystic fibrosis; lung clearance index; lung function test; multiple-breath washout; preschool children; primary ciliary dyskinesia.

Publication types

Multicenter Study
Research Support, Non-U.S. Gov't

MeSH terms

Breath Tests
Child, Preschool
Ciliary Motility Disorders* / diagnosis
Cross-Sectional Studies
Cystic Fibrosis* / diagnosis
Humans
Lung
Prospective Studies