title: Childhood Activity on Progression in Limb Girdle Muscular Dystrophy 2I.
abstract: Limb girdle muscular dystrophy 2I is a slowly progressive muscular dystrophy due to mutations in the Fukutin-related protein ( FKRP) gene. Clinicians are frequently asked if physical activity is harmful for pediatric patients with limb girdle muscular dystrophy 2I. The primary objective of this study was to determine if there is a relationship between self-reported childhood activity level and motor function and respiratory function in older children and adults with limb girdle muscular dystrophy 2I. We compared retrospective self-reported middle school activity level and sport participation with age at onset of weakness, 10-meter walk test, and forced vital capacity later in life in 41 participants with FKRP mutations. We found no relationship between activity level in childhood and disease course later in life, suggesting that self-directed physical activity in children with limb girdle muscular dystrophy 2I does not negatively affect disease progression and outcome.
authors.affiliation: 1 Department of Pediatrics, University of Iowa Hospitals and Clinics, Iowa City, IA, USA.
authors.forename: Brianna N
authors.initials: BN
authors.lastname: Brun
authors.affiliation: 2 Center for Disabilities and Development, University of Iowa Children's Hospital, Iowa City, IA, USA.
authors.forename: Shelley R H
authors.initials: SR
authors.lastname: Mockler
authors.affiliation: 2 Center for Disabilities and Development, University of Iowa Children's Hospital, Iowa City, IA, USA.
authors.forename: Katie M
authors.initials: KM
authors.lastname: Laubscher
authors.affiliation: 1 Department of Pediatrics, University of Iowa Hospitals and Clinics, Iowa City, IA, USA.
authors.forename: Carrie M
authors.initials: CM
authors.lastname: Stephan
authors.affiliation: 1 Department of Pediatrics, University of Iowa Hospitals and Clinics, Iowa City, IA, USA.
authors.forename: Julia A
authors.initials: JA
authors.lastname: Collison
authors.affiliation: 3 Department of Biostatistics, College of Public Health, University of Iowa, Iowa City, IA, USA.
authors.forename: M Bridget
authors.initials: MB
authors.lastname: Zimmerman
authors.affiliation: 1 Department of Pediatrics, University of Iowa Hospitals and Clinics, Iowa City, IA, USA.|4 Department of Neurology, University of Iowa Hospitals and Clinics, Iowa City, IA, USA.
authors.forename: Katherine D
authors.initials: KD
authors.lastname: Mathews
mesh_terms: D000293:Adolescent; D000328:Adult; D017668:Age of Onset; D000368:Aged; D002648:Child; D018450:Disease Progression; D015444:Exercise; D006801:Humans; D008875:Middle Aged; D049288:Muscular Dystrophies, Limb-Girdle / Q000235:genetics / Q000503:physiopathology*; D010430:Pentosyltransferases; D011506:Proteins / Q000235:genetics; D012189:Retrospective Studies; D057566:Self Report; D013177:Sports; D014797:Vital Capacity; D000070857:Walk Test; D055815:Young Adult
chemical_list: D011506:Proteins; C443107:FKRP protein, human; D010430:Pentosyltransferases
keywords: FKRP; dystroglycanopathy; exercise; muscular dystrophies; α-dystroglycan