title: Inspiratory muscle strength relative to disease severity in adults with stable cystic fibrosis.
abstract: BACKGROUND
  Due to heterogeneity in pulmonary disease, current literature may misrepresent inspiratory muscle involvement in cystic fibrosis (CF). This study investigated inspiratory muscle strength (IMS) relative to disease severity in adults with CF.
  
  
  METHODS
  Maximal inspiratory pressure (MIP) was assessed in 58 adults with stable CF grouped by disease severity (20 mild, 20 moderate, 18 severe) and compared to 20 controls. Relationships between MIP, lung function, dyspnea and anthropometrics were evaluated using multivariable linear models.
  
  
  RESULTS
  MIP in cmH2O and %-predicted was decreased in advanced CF lung disease as compared to mild disease and healthy controls (p<0.05). Disease severity accounted for 24% of the variance in IMS after controlling for confounding variables (p<0.001).
  
  
  CONCLUSIONS
  IMS is decreased in some adults with stable CF with moderate and severe pulmonary disease, and is related to dyspnea. Future studies should determine if decreased IMS contributes inefficient breathing patterns, respiratory pump dysfunction, and/or exercise intolerance in advanced CF.
authors.affiliation: Rutgers, The State University of New Jersey, School of Health Related Professions, Doctor of Physical Therapy Program - South, 40 East Laurel Road, Stratford, NJ 08084, USA. Electronic address: dekerlro@shrp.rutgers.edu.
authors.forename: Robert L
authors.initials: RL
authors.lastname: Dekerlegand
authors.affiliation: University of Pennsylvania, Adult Cystic Fibrosis Program, Perelman Center for Advanced Medicine, 3400 Civic Center Blvd, 1st Floor West, Philadelphia, PA 19104, USA. Electronic address: denis.hadjiliadis@uphs.upenn.edu.
authors.forename: Denis
authors.initials: D
authors.lastname: Hadjiliadis
authors.affiliation: West Virginia University, Division of Physical Therapy, Room 8314, PO Box 9226, Morgantown, WV 26506, USA. Electronic address: aswisher@hsc.wvu.edu.
authors.forename: Anne K
authors.initials: AK
authors.lastname: Swisher
authors.affiliation: Rutgers, The State University of New Jersey, School of Health Related Professions, Interdisciplinary Studies, 65 Bergen Street, Rm. 353A, Newark, NJ 07101, USA; Rutgers, The State University of New Jersey, School of Public Health, Quantitative Methods, 65 Bergen Street, Rm. 353A, Newark, NJ 07101, USA. Electronic address: parrotja@shrp.rutgers.edu.
authors.forename: J Scott
authors.initials: JS
authors.lastname: Parrott
authors.affiliation: Rutgers, The State University of New Jersey, School of Health Related Professions, Respiratory Therapy, 65 Bergen Street, Rm. 358, Newark, NJ 07101, USA. Electronic address: heueraj@shrp.rutgers.edu.
authors.forename: Albert J
authors.initials: AJ
authors.lastname: Heuer
authors.affiliation: Rutgers, The State University of New Jersey, School of Health Related Professions, Doctor of Physical Therapy Program - North, 65 Bergen Street, Rm. 721, Newark, NJ 07101, USA. Electronic address: myslinsk@shrp.rutgers.edu.
authors.forename: Mary Jane
authors.initials: MJ
authors.lastname: Myslinski
mesh_terms: D000328:Adult; D003430:Cross-Sectional Studies; D003550:Cystic Fibrosis / Q000175:diagnosis / Q000503:physiopathology*; D005260:Female; D005540:Forced Expiratory Flow Rates; D006801:Humans; D001239:Inhalation / Q000502:physiology*; D008297:Male; D008875:Middle Aged; D053580:Muscle Strength / Q000502:physiology*; D011379:Prognosis; D012129:Respiratory Function Tests; D012132:Respiratory Muscles / Q000503:physiopathology*; D012720:Severity of Illness Index; D014797:Vital Capacity; D055815:Young Adult
keywords: Adult; Cystic fibrosis; Inspiratory muscle strength; Maximal inspiratory pressure; Respiratory muscle