title: Epithelial basement membrane thickening is related to TGF-Beta 1 expression in children with chronic respiratory diseases.
abstract: BACKGROUND
  The complex structural changes of bronchial mucosa, known as remodelling, have been considered unique and typical for asthma. However, similar changes were recently found in other chronic respiratory diseases. The aim of this study was to compare basement membrane (BM) thickness and the number of transforming growth factor beta 1 (TGF-β1) positive epithelial cells in children with asthma, cystic fibrosis (CF), primary ciliary dyskinesia (PCD) and healthy controls.
  
  
  METHODS
  A total of 58 children (11.1 ± 3.9 yr, 55% males) were enrolled in this cross-sectional study. Endobronchial biopsy was performed in 27 children with asthma, 12 with CF, 12 with PCD and in 7 control patients. We studied the samples using light microscopy to assess BM width, the number of TGF-β1 positive epithelial cells and their correlation.
  
  
  RESULTS
  We found increased BM thickness (6.65 ± 1.22 μm vs. 2.93 ± 0.75 μm, p < 0.01) and a higher number of TGF-β1 positive epithelial cells (61.39 ± 19.03 vs. 21.57 ± 12.58, p < 0.01) in children with chronic respiratory diseases compared to controls. There was no difference in these parameters between asthma, CF and PCD. A positive correlation between BM thickness and the number of TGF-β1 positive cells was observed in all groups including controls (r = 0.84, p < 0.01).
  
  
  CONCLUSIONS
  Increased BM thickness and number of TGF-β1 positive epithelial cells were found in children with asthma, CF and PCD. The number of TGF-β1 positive cells correlated positively with the BM thickness in all groups. We suggest that this might be a common generic feature of bronchial remodelling in chronic respiratory diseases.
authors.affiliation: Department of Paediatrics, 2nd Faculty of Medicine, Charles University in Prague and Motol University Hospital, Prague, Czech Republic.
authors.forename: Lenka
authors.initials: L
authors.lastname: Hoňková
authors.forename: Jiří
authors.initials: J
authors.lastname: Uhlík
authors.forename: Katarína
authors.initials: K
authors.lastname: Beránková
authors.forename: Tamara
authors.initials: T
authors.lastname: Svobodová
authors.forename: Petr
authors.initials: P
authors.lastname: Pohunek
mesh_terms: D000293:Adolescent; D056151:Airway Remodeling; D001249:Asthma / Q000276:immunology*; D001485:Basement Membrane / Q000473:pathology; D002648:Child; D002908:Chronic Disease; D002925:Ciliary Motility Disorders / Q000276:immunology*; D003430:Cross-Sectional Studies; D003550:Cystic Fibrosis / Q000276:immunology*; D004847:Epithelial Cells / Q000378:metabolism* / Q000473:pathology; D005260:Female; D006801:Humans; D008297:Male; D020545:Respiratory Mucosa / Q000276:immunology; D053773:Transforming Growth Factor beta1 / Q000378:metabolism*
chemical_list: D053773:Transforming Growth Factor beta1
keywords: TGF-β1; asthma; cystic fibrosis; primary ciliary dyskinesia; remodelling