Skeletal muscle metabolism in cystic fibrosis and primary ciliary dyskinesia

Greg D. Wells, Donna L. Wilkes, Jane E. Schneiderman, Tammy Rayner, Maryam Elmi, Hiran Selvadurai, Sharon D. Dell, Michael D. Noseworthy, Felix Ratjen, Ingrid Tein, Allan L. Coates

Research output: Contribution to journalArticlepeer-review

41 Scopus citations


Previous studies have reported differences in muscle function and metabolism between patients with cystic fibrosis (CF) and healthy controls (HC), but it is currently unknown whether these abnormalities are specific to CF or also seen in other airway diseases. In this study, we used magnetic resonance spectroscopy (MRS) during exercise to assess muscle metabolism in CF patients. Twenty patients with CF and 20 age, gender, and habitual activity-matched HCs and a respiratory disease comparison group with primary ciliary dyskinesia (PCD; n = 10) were studied. Phosphorus MRS (P-MRS) was used to characterize muscle bioenergetic metabolism at rest and after high-, moderate-, and low-intensity exercise. CF patients exhibited lower resting ATP/phosphocreatine (PCr) ratio and significantly higher end-exercise pH values compared with both HC and PCD patients. Both CF and PCD patients demonstrated significantly slower PCr recovery time constants after high-intensity exercise. Our results suggest that not only there are specific abnormalities of muscle metabolism in CF patients but also there is a nonspecific impact of respiratory disease on muscle function.

Original languageEnglish (US)
Pages (from-to)40-45
Number of pages6
JournalPediatric Research
Issue number1
StatePublished - Jan 2011
Externally publishedYes

ASJC Scopus subject areas

  • Pediatrics, Perinatology, and Child Health


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