Document Detail


Skeletal muscle metabolism in cystic fibrosis and primary ciliary dyskinesia.
MedLine Citation:
PMID:  20938370     Owner:  NLM     Status:  In-Process    
Abstract/OtherAbstract:
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.
Authors:
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
Publication Detail:
Type:  Journal Article; Research Support, Non-U.S. Gov't    
Journal Detail:
Title:  Pediatric research     Volume:  69     ISSN:  1530-0447     ISO Abbreviation:  Pediatr. Res.     Publication Date:  2011 Jan 
Date Detail:
Created Date:  2010-12-14     Completed Date:  -     Revised Date:  -    
Medline Journal Info:
Nlm Unique ID:  0100714     Medline TA:  Pediatr Res     Country:  United States    
Other Details:
Languages:  eng     Pagination:  40-5     Citation Subset:  IM    
Affiliation:
Department of Physiology and Experimental Medicine, The Hospital for Sick Children, Toronto, Ontario, Canada. greg.wells@utoronto.ca
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