title: Comparison of Lung Clearance Index and Magnetic Resonance Imaging for Assessment of Lung Disease in Children with Cystic Fibrosis.
abstract: Early onset and progression of lung disease in children with cystic fibrosis (CF) indicates that sensitive noninvasive outcome measures are needed for diagnostic monitoring and early intervention clinical trials. The lung clearance index (LCI) and chest magnetic resonance imaging (MRI) were shown to detect early lung disease in CF; however, the relationship between the two measures remains unknown.
  
  
  
  To correlate the LCI with abnormalities detected by MRI and compare the sensitivity of the two techniques to detect responses to therapy for pulmonary exacerbations in children with CF.
  
  
  
  LCI determined by age-adapted multiple breath washout techniques and MRI studies were performed in 97 clinically stable children with CF across the pediatric age range (0.2-21.1 yr). Furthermore, LCI (n = 26) or MRI (n = 10) were performed at the time of pulmonary exacerbation and after antibiotic therapy. MRI was evaluated using a dedicated morphofunctional score.
  
  
  
  The LCI correlated with the global MRI score as well as MRI-defined airway wall abnormalities, mucus plugging, and abnormal lung perfusion in infants and toddlers (P < 0.05 to P < 0.001) and in older children (P < 0.001) with CF. LCI and MRI were sensitive to detect response to antibiotic therapy for pulmonary exacerbations.
  
  
  
  Our results indicate that LCI and MRI may be useful complementary tools for noninvasive monitoring and as quantitative endpoints in early intervention trials in children with CF. In this context, MRI enables detection of disease heterogeneity, including regional mucus plugging associated with abnormal lung perfusion in early CF lung disease. Clinical trial registered with www.clinicaltrials.gov (NCT 02270476).
authors: 1 Department of Translational Pulmonology.|2 Division of Pediatric Pulmonology and Allergy and Cystic Fibrosis Center, Department of Pediatrics, and.|3 Translational Lung Research Center Heidelberg, German Center for Lung Research, Heidelberg, Germany.
authors: Mirjam
authors: M
authors: Stahl
authors: 4 Department of Diagnostic and Interventional Radiology, University of Heidelberg, Heidelberg, Germany.|3 Translational Lung Research Center Heidelberg, German Center for Lung Research, Heidelberg, Germany.|5 Department of Radiology, German Cancer Research Center, Heidelberg, Germany; and.
authors: Mark O
authors: MO
authors: Wielpütz
authors: 1 Department of Translational Pulmonology.|2 Division of Pediatric Pulmonology and Allergy and Cystic Fibrosis Center, Department of Pediatrics, and.|3 Translational Lung Research Center Heidelberg, German Center for Lung Research, Heidelberg, Germany.
authors: Simon Y
authors: SY
authors: Graeber
authors: 1 Department of Translational Pulmonology.|2 Division of Pediatric Pulmonology and Allergy and Cystic Fibrosis Center, Department of Pediatrics, and.|3 Translational Lung Research Center Heidelberg, German Center for Lung Research, Heidelberg, Germany.
authors: Cornelia
authors: C
authors: Joachim
authors: 1 Department of Translational Pulmonology.|2 Division of Pediatric Pulmonology and Allergy and Cystic Fibrosis Center, Department of Pediatrics, and.|3 Translational Lung Research Center Heidelberg, German Center for Lung Research, Heidelberg, Germany.
authors: Olaf
authors: O
authors: Sommerburg
authors: 4 Department of Diagnostic and Interventional Radiology, University of Heidelberg, Heidelberg, Germany.|3 Translational Lung Research Center Heidelberg, German Center for Lung Research, Heidelberg, Germany.
authors: Hans-Ulrich
authors: HU
authors: Kauczor
authors: 5 Department of Radiology, German Cancer Research Center, Heidelberg, Germany; and.|6 Department of Diagnostic and Interventional Radiology with Nuclear Medicine, Thoraxklinik at University Hospital Heidelberg, Heidelberg, Germany.
authors: Michael
authors: M
authors: Puderbach
authors: 3 Translational Lung Research Center Heidelberg, German Center for Lung Research, Heidelberg, Germany.|5 Department of Radiology, German Cancer Research Center, Heidelberg, Germany; and.|6 Department of Diagnostic and Interventional Radiology with Nuclear Medicine, Thoraxklinik at University Hospital Heidelberg, Heidelberg, Germany.
authors: Monika
authors: M
authors: Eichinger
authors: 1 Department of Translational Pulmonology.|2 Division of Pediatric Pulmonology and Allergy and Cystic Fibrosis Center, Department of Pediatrics, and.|3 Translational Lung Research Center Heidelberg, German Center for Lung Research, Heidelberg, Germany.
authors: Marcus A
authors: 0000-0002-4057-2199
authors: MA
authors: Mall
mesh_terms: D000293:Adolescent; D001944:Breath Tests / Q000379:methods; D002648:Child; D002675:Child, Preschool; D003430:Cross-Sectional Studies; D003550:Cystic Fibrosis / Q000150:complications* / Q000000981:diagnostic imaging / Q000473:pathology / Q000503:physiopathology; D018450:Disease Progression; D005260:Female; D005541:Forced Expiratory Volume; D005858:Germany; D006801:Humans; D007223:Infant; D008168:Lung / Q000000981:diagnostic imaging / Q000473:pathology* / Q000503:physiopathology*; D008279:Magnetic Resonance Imaging; D008297:Male; D011446:Prospective Studies; D011659:Pulmonary Gas Exchange / Q000502:physiology*; D012129:Respiratory Function Tests / Q000379:methods; D013147:Spirometry; D055815:Young Adult
keywords: cystic fibrosis; lung clearance index; lung disease; magnetic resonance imaging; noninvasive monitoring