title: Morphological and Functional Assessment of Oesophageal Mucosa Integrity in Children With Cystic Fibrosis.
abstract: The aim of the study was to investigate morphological and functional characteristics of oesophageal epithelial barrier in children with cystic fibrosis (CF) with or without gastro-oesophageal reflux disease (GORD) in comparison to healthy controls.
  
  
  
  Oesophagogastroduodenoscopy with oesophageal biopsies and combined oesophageal multichannel intraluminal impedance-pH monitoring was performed in 17 children with CF (CFtot) with (CFgord) or without GORD (CFnorm). Histological combined severity score was calculated and widths of spaces between epithelial cells were measured. Basal impedance value was used to assess functional integrity of epithelial barrier. Results of each investigation were compared with a group of children without oesophageal disease.
  
  
  
  CFtot, but also CFnorm, had more severe pathohistological changes included in the compound severity score than controls (0.75 ± 0.32 and 0.75 ± 0.20 vs 0.27 ± 0.25; P < 0.001 and P = 0.001, respectively). They also had more dilated intercellular spaces (2.6 μm ± 0.6 and 2.7 μm ± 0.5 vs 1.9 μm ± 0.2; P = 0.001 and P < 0.001, respectively). Baseline impedance values between proximal and distal pairs of electrodes were significantly lower in CFtot (2876 Ω ± 484, 2590 Ω ± 1013) and also in CFnorm (2922 Ω ± 363, 2844 Ω ± 457) than in controls (3703 Ω ± 859, 3753 Ω ± 1070) (P = 0.012 and P = 0.002; and P = 0.027 and P = 0.005, respectively). The treatment of CFgord with proton pump inhibitor increased, but did not normalise the baseline impedance values (2860 Ω ± 560 to 3355 Ω ± 750 and 2178 Ω ± 1564 to 3057 Ω ± 594).
  
  
  
  Children with CF had morphological and functional changes of oesophageal mucosal integrity even in the absence of GORD.
authors.affiliation: *Department of Gastroenterology, Hepatology and Nutrition, University Children's Hospital, University Medical Centre Ljubljana †Department of Pediatrics ‡Institute of Pathology, Faculty of Medicine, University of Ljubljana, Ljubljana, Slovenia.
authors.forename: Jernej
authors.initials: J
authors.lastname: Brecelj
authors.forename: Nina
authors.initials: N
authors.lastname: Zidar
authors.forename: Jera
authors.initials: J
authors.lastname: Jeruc
authors.forename: Rok
authors.initials: R
authors.lastname: Orel
mesh_terms: D000293:Adolescent; D016022:Case-Control Studies; D002648:Child; D002662:Child Health Services; D002675:Child, Preschool; D003550:Cystic Fibrosis / Q000150:complications / Q000473:pathology / Q000503:physiopathology*; D016145:Endoscopy, Digestive System; D050782:Esophageal pH Monitoring; D004947:Esophagus / Q000000981:diagnostic imaging / Q000473:pathology / Q000503:physiopathology*; D005260:Female; D005764:Gastroesophageal Reflux / Q000150:complications / Q000473:pathology / Q000503:physiopathology*; D006801:Humans; D007223:Infant; D007413:Intestinal Mucosa / Q000000981:diagnostic imaging / Q000473:pathology / Q000503:physiopathology; D008297:Male; D012720:Severity of Illness Index