Klin Padiatr 2022; 234(05): 338-339
DOI: 10.1055/s-0042-1754498
Abstracts
Poster
Poster Walk 3: Seltene Erkrankungen (PCD, ChILD und andere)

Comparison of the Lung Clearance Index in preschool children with Primary Ciliary Dyskinesia and Cystic Fibrosis

J Röhmel
1   Charité- Universitätsmedizin Berlin, Klinik für Pädiatrie mit Schwerpunkt Pneumologie, Immunologie und Intensivmedizin, Berlin, Germany
,
F Dörfler
1   Charité- Universitätsmedizin Berlin, Klinik für Pädiatrie mit Schwerpunkt Pneumologie, Immunologie und Intensivmedizin, Berlin, Germany
,
C Koerner-Rettberg
2   University Children's Hospital of Ruhr University Bochum, Department of Pediatric Pulmonology, Bochum, Germany
3   Marienhospital Wesel, Department of General Pediatrics,, Wesel, Germany
,
F Brinkmann
2   University Children's Hospital of Ruhr University Bochum, Department of Pediatric Pulmonology, Bochum, Germany
,
A Schlegtendal
2   University Children's Hospital of Ruhr University Bochum, Department of Pediatric Pulmonology, Bochum, Germany
,
M Wetzke
4   Hannover Medical School, Hannover, Germany, Department of Pediatric Pulmonology, Allergology and Neonatology,, Hannover, Germany
5   Biomedical Research in End-Stage and Obstructive Lung Disease Hannover, German Center for Lung Research (DZL), Hannover Medical University Hannover, Hannover, Germany
,
I Rudolf
4   Hannover Medical School, Hannover, Germany, Department of Pediatric Pulmonology, Allergology and Neonatology,, Hannover, Germany
5   Biomedical Research in End-Stage and Obstructive Lung Disease Hannover, German Center for Lung Research (DZL), Hannover Medical University Hannover, Hannover, Germany
,
S Helms
6   University Children's Hospital Muenster, Department of General Pediatrics, Münster, Germany
,
J Große-Onnebrink
6   University Children's Hospital Muenster, Department of General Pediatrics, Münster, Germany
,
Y Yu
7   University of Heidelberg, Division of Pediatric Pulmonology and Allergy and Cystic Fibrosis Center, Department of Pediatrics, Heidelberg, Germany
8   University of Heidelberg, Translational Lung Research Center (TLRC) Heidelberg, German Center for Lung Research (DZL), Heidelberg, Germany
,
T Nuesslein
9   Gemeinschaftsklinikum Mittelrhein, Department of General Pediatrics, Koblenz, Germany
,
I Wojsyk-Banaszak
10   Poznań University of Medical Sciences, Poland, Department of Pulmonology, Pediatric Allergy and Clinical Immunology,, Poznań, Poland
,
S Becker
11   Darmstädter Kinderkliniken Prinzessin Margaret, Darmstadt, Germany
,
O Eickmeier
12   Goethe University, Division of Allergology, Department of Pediatrics, Pulmonology and Cystic Fibrosis Center, Frankfurt, Germany
,
O Sommerburg
7   University of Heidelberg, Division of Pediatric Pulmonology and Allergy and Cystic Fibrosis Center, Department of Pediatrics, Heidelberg, Germany
8   University of Heidelberg, Translational Lung Research Center (TLRC) Heidelberg, German Center for Lung Research (DZL), Heidelberg, Germany
,
H Omran
6   University Children's Hospital Muenster, Department of General Pediatrics, Münster, Germany
,
M Stahl
1   Charité- Universitätsmedizin Berlin, Klinik für Pädiatrie mit Schwerpunkt Pneumologie, Immunologie und Intensivmedizin, Berlin, Germany
13   Berlin Institute of Health (BIH) at Charité – Universitätsmedizin Berlin, Berlin, Germany
14   German Center for Lung Research (DZL), associated partner site, Berlin, Germany
8   University of Heidelberg, Translational Lung Research Center (TLRC) Heidelberg, German Center for Lung Research (DZL), Heidelberg, Germany
7   University of Heidelberg, Division of Pediatric Pulmonology and Allergy and Cystic Fibrosis Center, Department of Pediatrics, Heidelberg, Germany
,
M Mall
15   Charité – Universitätsmedizin Berlin, Klinik für Pädiatrie mit Schwerpunkt Pneumologie, Immunologie und Intensivmedizin, Berlin, Germany
13   Berlin Institute of Health (BIH) at Charité – Universitätsmedizin Berlin, Berlin, Germany
14   German Center for Lung Research (DZL), associated partner site, Berlin, Germany
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Introduction Previous studies have shown that the lung clearance index (LCI) determined by multiple-breath washout (MBW) is sensitive to detect 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. The study question was, if MBW is feasible and sensitive to detect ventilation inhomogeneity in preschool children with PCD?

Methods This was a prospective cross-sectional multicenter study and included preschoolers with PCD and preschoolers with CF and healthy controls. LCI was determined using nitrogen MBW and compared between the three groups.

Results LCI was determined in 27 children with PCD, 34 children with CF and 30 healthy controls (mean age, 4.8 years; range, 2.2 – 6.9 years). The LCI in preschool children with PCD was increased (median, 9.1; CI 95%, 8.6-10.3) compared to healthy controls (median, 7.0; CI 95%, 6.7-7.1), (P < 0.0001), but did not differ from preschool children with CF (median, 8.6; CI 95%, 8.4-9.7), (P = 0.71). The feasibility in the PCD group was 93.1% and was similar to that in the CF group (91.9%) and in healthy controls (85.7%), (P = 0.55).

Conclusion This study demonstrates early onset of lung disease in preschool children with PCD and indicates 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 endpoint in clinical trials testing early interventions in children with PCD.



Publication History

Article published online:
21 September 2022

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