title: Ventilatory pattern and energy expenditure are altered in cystic fibrosis mice.
abstract: BACKGROUND
  Altered ventilatory pattern and increased energy expenditure are facets of the complex cystic fibrosis (CF) phenotype. It is not known whether these are inherent attributes of CF, secondary consequences of lung infection or other disease complications.
  
  
  METHODS
  Studies were performed in congenic C57BL/6J, F508del (Cftr((tm1kth))) and CF gut-corrected (F508del) mice. Ventilatory patterns were measured using whole-body plethysmography. Indirect calorimetry was used to determine oxygen consumption, carbon dioxide production and resting energy expenditure.
  
  
  RESULTS
  CF mice (F508del and F508del gut-corrected) have a significantly faster respiratory rate and increased ventilatory pattern variability as compared to non-CF mice. F508del but not CF gut-corrected mice had significantly increased energy expenditure per gram body weight.
  
  
  CONCLUSIONS
  CF mice exhibit a faster, more variable ventilatory pattern. These changes were present in the absence of detectable infection or illness due to gastrointestinal obstruction. Increased resting energy expenditure does not completely account for these differences.
authors.affiliation: Frances Payne Bolton School of Nursing, Case Western Reserve University, USA. rjm11@case.edu
authors.forename: Rebecca J
authors.initials: RJ
authors.lastname: Darrah
authors.forename: Ilya R
authors.initials: IR
authors.lastname: Bederman
authors.forename: Anna L
authors.initials: AL
authors.lastname: Mitchell
authors.forename: Craig A
authors.initials: CA
authors.lastname: Hodges
authors.forename: Cara K
authors.initials: CK
authors.lastname: Campanaro
authors.forename: Mitchell L
authors.initials: ML
authors.lastname: Drumm
authors.forename: Frank J
authors.initials: FJ
authors.lastname: Jacono
mesh_terms: D000818:Animals; D003550:Cystic Fibrosis / Q000235:genetics / Q000378:metabolism* / Q000503:physiopathology*; D004734:Energy Metabolism; D005260:Female; D008297:Male; D051379:Mice; D008810:Mice, Inbred C57BL; D056152:Respiratory Rate