Medizinische Universität Graz - Research portal
Navigation/Search
- Researchers
- Institutions
- Projects
- Publications
- Partners
- Sponsors
- Equipment
- Knowhow
- Fields of Research
"Faces" of the day:
Selected Publication:
SHR Neuro Cancer Cardio Lipid Metab Microb
Bernasconi, N; Kieninger, E; Shaw, M; Kurz, J; Moeller, A; Ratjen, F; Rochat, I; Stanojevic, S; Singer, F.
CFTR-function and ventilation inhomogeneity in individuals with cystic fibrosis.
J Cyst Fibros. 2021; 20(4):641-647
Doi: 10.1016/j.jcf.2020.12.009
Web of Science
PubMed
FullText
FullText_MUG
- Leading authors Med Uni Graz
- Singer Florian
- Altmetrics:
- Dimensions Citations:
- Plum Analytics:
- Scite (citation analytics):
- Abstract:
- BACKGROUND: Increased (abnormal) ventilation inhomogeneity in individuals with mild Cystic Fibrosis (CF) lung disease may become a treatable trait for small-molecule therapeutics improving Cystic Fibrosis Transmembrane Regulator (CFTR) function. The relationship between CFTR function and ventilation inhomogeneity is unknown. We aimed to identify and quantify increased ventilation inhomogeneity in relation to CFTR function. METHODS: This was an international, multi-center, cross-sectional study. We collated data from individuals aged 3-25 years with minimal (CFTR-MF) or residual (CFTR-RF) function of a variety of CFTR genotypes and FEV1 ≥ 70% predicted. We measured lung function using nitrogen multiple-breath washout and spirometry. We compared lung clearance index (LCI) and FEV1 between individuals with CFTR-MF vs CFTR-RF using a mixed effects multi-variable linear regression model to account for study differences and a logistic model based on propensity-score matching to adjust for possible confounding. RESULTS: We included 141 with CFTR-MF and 35 with CFTR-RF. LCI (> 1.96 z-score) was elevated in 71.6% individuals with CFTR-MF and in 40.0% with CFTR-RF. FEV1 (< -1.96 z-score) was reduced in 11.3% individuals with CFTR-MF and in 5.7% with CFTR-RF. The mean difference (95% CI) of LCI and FEV1 between CFTR-MF and CFTR-RF was 3.71 (1.63 to 5.79) and -0.40 (-0.83 to 0.02) z-score. The LCI differences were similar after adjustment for confounders and in individuals with normal FEV1. CONCLUSION: Increased ventilation inhomogeneity is associated with less CFTR function. In individuals with mild CF lung disease, LCI can identify and quantify increased ventilation inhomogeneity, a candidate treatable trait.
- Find related publications in this database (using NLM MeSH Indexing)
- Adolescent - administration & dosage
- Child - administration & dosage
- Child, Preschool - administration & dosage
- Cross-Sectional Studies - administration & dosage
- Cystic Fibrosis - physiopathology
- Cystic Fibrosis Transmembrane Conductance Regulator - physiology
- Female - administration & dosage
- Humans - administration & dosage
- Male - administration & dosage
- Pulmonary Ventilation - administration & dosage
- Respiratory Function Tests - administration & dosage
- Retrospective Studies - administration & dosage
- Find related publications in this database (Keywords)
- Cystic fibrosis
- Child
- Pulmonary function
- Treatment