| Blocked muscle fat oxidation during exercise in neutral lipid storage disease. | |
MedLine Citation:
|
PMID: 22491199 Owner: NLM Status: In-Data-Review |
Abstract/OtherAbstract:
|
OBJECTIVE: To determine whether impaired exercise capacity in neutral lipid storage disease with myopathy is solely caused by muscle weakness or whether a defect in energy metabolism (blocked fat oxidation) may also play a role. DESIGN: We studied a 37-year-old woman with neutral lipid storage disease with myopathy, who cycled while lipid oxidation was assessed using U-(13)C palmitate tracer dilution technique. The effect of a glucose infusion during exercise was also studied. SETTING: Neuromuscular research unit. RESULTS: The exercise-induced increase in fat oxidation was virtually abolished in the patient. Treatment with intravenous glucose infusion improved maximal oxygen uptake from 23 to 27 mL × kg(-1) × min(-1), and maximal workload from 75 to 100 W. CONCLUSIONS: These results demonstrate that in addition to fixed weakness, neutral lipid storage disease with myopathy is also characterized by a profound block in fat oxidation, which limits exercise tolerance. |
Authors:
|
Pascal Laforêt; Mette Orngreen; Nicolai Preisler; Grete Andersen; John Vissing |
Related Documents
:
|
19508829 - Exercise therapy across the lung cancer continuum. 3107849 - 5-fluorouracil, adriamycin, and mitomycin-c (fam) chemotherapy in advanced adenocarcino... 3975499 - Lung volume changes during respiration in ducks. 3512509 - Estimation of volume of epithelial lining fluid recovered by lavage using urea as marke... 970749 - Transit time analysis of the forced expiratory vital capacity in cystic fibrosis. 11872119 - Bacteria in heart and lungs of young chicks. 12412819 - Effect of terbinafine on the pharmacokinetics and pharmacodynamics of desipramine in he... 6207209 - New approaches to drug selection and serial drug testing. 7363289 - Physical activity and coronary heart disease. |
Publication Detail:
|
Type: Journal Article |
Journal Detail:
|
Title: Archives of neurology Volume: 69 ISSN: 1538-3687 ISO Abbreviation: Arch. Neurol. Publication Date: 2012 Apr |
Date Detail:
|
Created Date: 2012-04-11 Completed Date: - Revised Date: - |
Medline Journal Info:
|
Nlm Unique ID: 0372436 Medline TA: Arch Neurol Country: United States |
Other Details:
|
Languages: eng Pagination: 530-3 Citation Subset: AIM; IM |
Affiliation:
|
Centre de Référence de Pathologie Neuromusculaire Paris-Est, Bâtiment Babinski, Groupe Hospitalier Pitié-Salpêtrière, 47-83 Boulevard de l'Hôpital, 75651 Paris CEDEX 13, France. pascal.laforet@psl.aphp.fr. |
Export Citation:
|
APA/MLA Format Download EndNote Download BibTex |
| MeSH Terms | |
Descriptor/Qualifier:
|
|
From MEDLINE®/PubMed®, a database of the U.S. National Library of Medicine
Previous Document: Saccade abnormalities in autopsy-confirmed frontotemporal lobar degeneration and Alzheimer disease.
Next Document: Progressive weakness with respiratory failure in a patient with sarcoidosis.