title: Breathing pattern and chest wall volumes during exercise in patients with cystic fibrosis, pulmonary fibrosis and COPD before and after lung transplantation.
abstract: BACKGROUND
  Pulmonary fibrosis (PF), cystic fibrosis (CF) and chronic obstructive pulmonary disease (COPD) often cause chronic respiratory failure (CRF).
  
  
  METHODS
  In order to investigate if there are different patterns of adaptation of the ventilatory pump in CRF, in three groups of lung transplant candidates with PF (n=9, forced expiratory volume in 1 s (FEV(1))=37+/-3% predicted, forced vital capacity (FVC)=32+/-2% predicted), CF (n=9, FEV(1)=22+/-3% predicted, FVC=30+/-3% predicted) and COPD (n=21, FEV(1)=21+/-1% predicted, FVC=46+/-2% predicted), 10 healthy controls and 16 transplanted patients, total and compartmental chest wall volumes were measured by opto-electronic plethysmography during rest and exercise.
  
  
  RESULTS
  Three different breathing patterns were found during CRF in PF, CF and COPD. Patients with COPD were characterised by a reduced duty cycle at rest and maximal exercise (34+/-1%, p<0.001), while patients with PF and CF showed an increased breathing frequency (49+/-6 and 34+/-2/min, respectively) and decreased tidal volume (0.75+/-0.10 and 0.79+/-0.07 litres) (p<0.05). During exercise, end-expiratory chest wall and rib cage volumes increased significantly in patients with COPD and CF but not in those with PF. End-inspiratory volumes did not increase in CF and PF. The breathing pattern of transplanted patients was similar to that of healthy controls.
  
  
  CONCLUSIONS
  There are three distinct patterns of CRF in patients with PF, CF and COPD adopted by the ventilatory pump to cope with the underlying lung disease that may explain why patients with PF and CF are prone to respiratory failure earlier than patients with COPD. After lung transplantation the chronic adaptations of the ventilatory pattern to advanced lung diseases are reversible and indicate that the main contributing factor is the lung itself rather than systemic effects of the disease.
authors.affiliation: Innere Medizin V, Universitätsklinikum des Saarlandes, Homburg/Saar, Germany.
authors.forename: H
authors.initials: H
authors.lastname: Wilkens
authors.forename: B
authors.initials: B
authors.lastname: Weingard
authors.forename: A
authors.initials: A
authors.lastname: Lo Mauro
authors.forename: E
authors.initials: E
authors.lastname: Schena
authors.forename: A
authors.initials: A
authors.lastname: Pedotti
authors.forename: G W
authors.initials: GW
authors.lastname: Sybrecht
authors.forename: A
authors.initials: A
authors.lastname: Aliverti
mesh_terms: D000222:Adaptation, Physiological / Q000502:physiology; D000328:Adult; D003550:Cystic Fibrosis / Q000150:complications / Q000503:physiopathology / Q000601:surgery; D015444:Exercise / Q000502:physiology*; D005260:Female; D005541:Forced Expiratory Volume / Q000502:physiology; D006801:Humans; D008171:Lung Diseases / Q000503:physiopathology* / Q000601:surgery; D016040:Lung Transplantation / Q000502:physiology*; D008297:Male; D008875:Middle Aged; D010991:Plethysmography / Q000379:methods; D011184:Postoperative Period; D029424:Pulmonary Disease, Chronic Obstructive / Q000150:complications / Q000503:physiopathology / Q000601:surgery; D011658:Pulmonary Fibrosis / Q000150:complications / Q000503:physiopathology / Q000601:surgery; D012131:Respiratory Insufficiency / Q000209:etiology / Q000503:physiopathology / Q000601:surgery; D015656:Respiratory Mechanics / Q000502:physiology*; D035441:Thoracic Wall / Q000503:physiopathology*; D014797:Vital Capacity / Q000502:physiology