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Home-Based Prescribed Pulmonary Exercise in Patients with Stable Chronic Obstructive Pulmonary Disease
Published on: August 24, 2019
Exercise intolerance in Kearns-Sayre syndrome
S Bower1, S Nicole, G Kirkland
1Department of Clinical Neurosciences, St Vincent's Hospital, Fitzroy, Victoria, Australia.
Summary
Patients with Kearns-Sayre syndrome (KSS) experience exercise intolerance due to impaired mitochondrial function. This study found exaggerated heart rate and ventilation relative to oxygen consumption in a KSS patient during exercise.
Area of Science:
- Cardiology
- Neurology
- Mitochondrial Medicine
Background:
- Kearns-Sayre syndrome (KSS) is a mitochondrial disease.
- KSS presents with progressive external ophthalmoplegia, cardiac defects, and retinal degeneration.
- Exercise intolerance is a common symptom in KSS.
Purpose of the Study:
- To investigate exercise physiology in a patient with KSS.
- To explore the relationship between cardiac output, ventilation, and metabolic rate in KSS.
Main Methods:
- Exercise testing was conducted on a 32-year-old female KSS patient with a pacemaker.
- Minute ventilation, oxygen consumption, and carbon dioxide production were measured.
- Expired air was collected to analyze respiratory gases during varying workloads.
Main Results:
- The KSS patient exhibited exaggerated heart rate and ventilation relative to oxygen consumption and carbon dioxide production.
- These responses occurred even at subanaerobic workloads.
- Findings suggest a dissociation between cardiac output, ventilation, and muscle metabolic rate.
Conclusions:
- Impaired oxidative phosphorylation in mitochondrial disorders may lead to uncoupling of physiological responses.
- This uncoupling contributes to exercise intolerance observed in KSS.
- The study supports the hypothesis of altered metabolic regulation in mitochondrial diseases.
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