Document Detail


Impaired oxygen extraction in metabolic myopathies: detection and quantification by near-infrared spectroscopy.
MedLine Citation:
PMID:  17143893     Owner:  NLM     Status:  MEDLINE    
Abstract/OtherAbstract:
Patients with mitochondrial myopathies (MM) or myophosphorylase deficiency (McArdle's disease, McA) show impaired capacity for O(2) extraction, low maximal aerobic power, and reduced exercise tolerance. Non-invasive tools are needed to quantify the metabolic impairment. Six patients with MM, 6 with McA, 25 with symptoms of metabolic myopathy but negative biopsy (patient-controls, P-CTRL) and 20 controls (CTRL) underwent an incremental cycloergometric test. Pulmonary O(2) uptake (VO(2)) and vastus lateralis oxygenation indices (by near-infrared spectroscopy, NIRS) were determined. Concentration changes of deoxygenated hemoglobin and myoglobin (Delta[deoxy(Hb + Mb)]) were considered an index of O(2) extraction. Delta[deoxy(Hb + Mb)] peak (percent limb ischemia) was lower in MM (25.3 +/- 12.0%) and McA (18.7 +/- 7.3) than in P-CTRL (62.4 +/- 3.9) and CTRL (71.3 +/- 3.9) subjects. VO(2) peak and Delta[deoxy(Hb + Mb)] peak were linearly related (r(2) = 0.83). In these patients, NIRS is a tool to detect and quantify non-invasively the metabolic impairment, which may be useful in the follow-up of patients and in the assessment of therapies and interventions.
Authors:
Bruno Grassi; Mauro Marzorati; Francesca Lanfranconi; Alessandra Ferri; Miriam Longaretti; Andrea Stucchi; Paola Vago; Claudio Marconi; Lucia Morandi
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Publication Detail:
Type:  Journal Article; Research Support, Non-U.S. Gov't    
Journal Detail:
Title:  Muscle & nerve     Volume:  35     ISSN:  0148-639X     ISO Abbreviation:  Muscle Nerve     Publication Date:  2007 Apr 
Date Detail:
Created Date:  2007-05-07     Completed Date:  2007-06-05     Revised Date:  2012-02-23    
Medline Journal Info:
Nlm Unique ID:  7803146     Medline TA:  Muscle Nerve     Country:  United States    
Other Details:
Languages:  eng     Pagination:  510-20     Citation Subset:  IM    
Affiliation:
Department of Science and Biomedical Technologies, University of Milan, LITA-Via Fratelli Cervi 93, I-20090 Segrate, MI, Italy. bruno.grassi@unimi.it
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MeSH Terms
Descriptor/Qualifier:
Adult
Aged
Energy Metabolism / physiology
Exercise / physiology
Exercise Test
Exercise Tolerance / physiology
Female
Glycogen Storage Disease Type V / diagnosis*,  metabolism*,  physiopathology
Heart Rate / physiology
Hemoglobins / analysis,  metabolism
Humans
Male
Mitochondrial Myopathies / diagnosis*,  metabolism*,  physiopathology
Muscle, Skeletal / metabolism,  physiopathology
Myoglobin / metabolism
Oxidative Phosphorylation
Oxygen Consumption* / physiology
Predictive Value of Tests
Reference Values
Spectroscopy, Near-Infrared / methods*,  trends
Grant Support
ID/Acronym/Agency:
GUP030534//Telethon
Chemical
Reg. No./Substance:
0/Hemoglobins; 0/Myoglobin

From MEDLINE®/PubMed®, a database of the U.S. National Library of Medicine


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