Skeletal muscle metabolism in cystic fibrosis and primary ciliary dyskinesia.

Abstract:

: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.

journal_name

Pediatr Res

journal_title

Pediatric research

authors

Wells GD,Wilkes DL,Schneiderman JE,Rayner T,Elmi M,Selvadurai H,Dell SD,Noseworthy MD,Ratjen F,Tein I,Coates AL

doi

10.1203/PDR.0b013e3181fff35f

subject

Has Abstract

pub_date

2011-01-01 00:00:00

pages

40-5

issue

1

eissn

0031-3998

issn

1530-0447

journal_volume

69

pub_type

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