title: Respiratory dysfunction in unsedated dogs with golden retriever muscular dystrophy.
abstract: Golden retriever muscular dystrophy (GRMD) is a well-established model of Duchenne muscular dystrophy. The value of this model would be greatly enhanced with practical tools to monitor progression of respiratory dysfunction during treatment trials. Arterial blood gas analysis, tidal breathing spirometry, and respiratory inductance plethysmography (RIP) were performed to determine if quantifiable abnormalities could be identified in unsedated, untrained, GRMD dogs. Results from 11 dogs with a mild phenotype of GRMD and 11 age-matched carriers were compared. Arterial blood gas analysis was successfully performed in all dogs, spirometry in 21 of 22 (95%) dogs, and RIP in 18 of 20 (90%) dogs. Partial pressure of carbon dioxide and bicarbonate concentration were higher in GRMD dogs. Tidal breathing peak expiratory flows were markedly higher in GRMD dogs. Abnormal abdominal motion was present in 7 of 10 (70%) GRMD dogs. Each technique provided objective, quantifiable measures that will be useful for monitoring respiratory function in GRMD dogs during clinical trials while avoiding the influence of sedation on results. Increased expiratory flows and the pattern of abdominal breathing are novel findings, not reported in people with Duchenne muscular dystrophy, and might be a consequence of hyperinflation.
authors.affiliation: Department of Clinical Sciences, College of Veterinary Medicine, North Carolina State University, Raleigh, NC 27607, United States.
authors.forename: Justin C
authors.initials: JC
authors.lastname: DeVanna
authors.affiliation: Department of Pathology and Laboratory Medicine, School of Medicine, University of North Carolina, Chapel Hill, NC 27599, United States; Department of Neurology, School of Medicine, University of North Carolina, Chapel Hill, NC 27599, United States; The Gene Therapy Center, School of Medicine, University of North Carolina, Chapel Hill, NC 27599, United States.
authors.forename: Joe N
authors.initials: JN
authors.lastname: Kornegay
authors.affiliation: Department of Pathology and Laboratory Medicine, School of Medicine, University of North Carolina, Chapel Hill, NC 27599, United States; Department of Neurology, School of Medicine, University of North Carolina, Chapel Hill, NC 27599, United States; The Gene Therapy Center, School of Medicine, University of North Carolina, Chapel Hill, NC 27599, United States.
authors.forename: Daniel J
authors.initials: DJ
authors.lastname: Bogan
authors.affiliation: Department of Pathology and Laboratory Medicine, School of Medicine, University of North Carolina, Chapel Hill, NC 27599, United States; Department of Neurology, School of Medicine, University of North Carolina, Chapel Hill, NC 27599, United States; The Gene Therapy Center, School of Medicine, University of North Carolina, Chapel Hill, NC 27599, United States.
authors.forename: Janet R
authors.initials: JR
authors.lastname: Bogan
authors.affiliation: Department of Pathology and Laboratory Medicine, School of Medicine, University of North Carolina, Chapel Hill, NC 27599, United States; Department of Neurology, School of Medicine, University of North Carolina, Chapel Hill, NC 27599, United States; The Gene Therapy Center, School of Medicine, University of North Carolina, Chapel Hill, NC 27599, United States.
authors.forename: Jennifer L
authors.initials: JL
authors.lastname: Dow
authors.affiliation: Department of Clinical Sciences, College of Veterinary Medicine, North Carolina State University, Raleigh, NC 27607, United States. Electronic address: eleanor_hawkins@ncsu.edu.
authors.forename: Eleanor C
authors.initials: EC
authors.lastname: Hawkins
mesh_terms: D000818:Animals; D001784:Blood Gas Analysis; D018450:Disease Progression; D004285:Dogs; D005260:Female; D006339:Heart Rate; D008297:Male; D009137:Muscular Dystrophy, Animal / Q000097:blood / Q000503:physiopathology*; D012120:Respiration Disorders / Q000097:blood / Q000503:physiopathology*; D056152:Respiratory Rate
keywords: Blood gas analysis; Flow-volume loop; Golden retriever muscular dystrophy; Muscular dystrophy; Pulmonary function test; Respiratory inductance plethysmography; Spirometry