Childhood Activity on Progression in Limb Girdle Muscular Dystrophy 2I
| Autor: | Carrie Stephan, Katie M. Laubscher, M. Bridget Zimmerman, Julia A. Collison, Brianna N. Brun, Shelley R. H. Mockler, Katherine D. Mathews |
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| Rok vydání: | 2016 |
| Předmět: |
Adult
Weakness medicine.medical_specialty Vital capacity Adolescent Vital Capacity Walk Test Article Disease course 03 medical and health sciences Young Adult 0302 clinical medicine Physical medicine and rehabilitation medicine Humans Respiratory function Pentosyltransferases Muscular dystrophy Age of Onset Child Exercise Aged Retrospective Studies business.industry Disease progression Proteins 030229 sport sciences Middle Aged medicine.disease Muscular Dystrophies Limb-Girdle Walk test Pediatrics Perinatology and Child Health Disease Progression Neurology (clinical) Self Report medicine.symptom business 030217 neurology & neurosurgery Limb-girdle muscular dystrophy Sports |
| Zdroj: | Journal of child neurology. 32(2) |
| ISSN: | 1708-8283 |
| Popis: | 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. |
| Databáze: | OpenAIRE |
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