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Exercise intolerance in Kearns-Sayre syndrome.
MedLine Citation:
PMID:  18638908     Owner:  NLM     Status:  In-Data-Review    
Abstract/OtherAbstract:
Exercise intolerance is a common finding in mitochondrial diseases, including Kearns-Sayre syndrome (KSS), characterised by progressive external ophthalmoplegia, cardiac conduction defects and atypical pigmentary degeneration of the retina. Exercise studies were performed in a 32 year old woman with KSS who had received an atrio-ventricular sequential pacemaker because of continuing breathlessness, having presented with complete heart block requiring a fixed rate demand pacemaker 6 years earlier. Minute ventilation, oxygen consumption, and carbon dioxide production were measured at different workloads on 3 consecutive days by collecting expired air. Compared to controls, after exercise at a subanaerobic workload, heart rate and ventilation were exaggerated relative to both oxygen consumption and carbon dioxide production. The findings are consistent with the hypothesis that, in mitochondrial disorders, impaired oxidative phosphorylation leads to uncoupling of cardiac ouput and ventilation relative to muscle metabolic rate.
Authors:
S Bower; S Nicole; G Kirkland; E Byrne
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Publication Detail:
Type:  Journal Article    
Journal Detail:
Title:  Journal of clinical neuroscience : official journal of the Neurosurgical Society of Australasia     Volume:  3     ISSN:  0967-5868     ISO Abbreviation:  J Clin Neurosci     Publication Date:  1996 Oct 
Date Detail:
Created Date:  2008-07-21     Completed Date:  -     Revised Date:  -    
Medline Journal Info:
Nlm Unique ID:  9433352     Medline TA:  J Clin Neurosci     Country:  Scotland    
Other Details:
Languages:  eng     Pagination:  386-9     Citation Subset:  -    
Affiliation:
Department of Clinical Neurosciences, St Vincent's Hospital, Fitzroy, Victoria, Australia.
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