Document Detail

Biochemical monitoring of the treatment in paediatric patients with mitochondrial disease.
MedLine Citation:
PMID:  9870209     Owner:  NLM     Status:  MEDLINE    
Treatment strategies in mitochondrial diseases consist of several drugs that diminish the deleterious effects of the abnormal respiratory chain function, reduce the presence of toxic agents or correct deficiencies in essential cofactors. In this study we evaluated the monitoring of tocopherol, carnitine and ubiquinone concentrations in a group of paediatric patients during a follow-up period of 18 months and the response to treatment of these patients by means of the determination of blood lactate, plasma alanine and oxygen consumption by lymphocytes in relation to the clinical status of the patients. Tocopherol, carnitine and ubiquinone concentrations were easily corrected with therapy. Blood lactate proved the best biochemical tool to assess the response to treatment in paediatric patients. According to our results, improvement or stabilization of the clinical course seems to be more related to the biochemical or molecular defect than to the effectiveness of the treatment.
R Artuch; M A Vilaseca; M Pineda
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Publication Detail:
Type:  Journal Article; Research Support, Non-U.S. Gov't    
Journal Detail:
Title:  Journal of inherited metabolic disease     Volume:  21     ISSN:  0141-8955     ISO Abbreviation:  J. Inherit. Metab. Dis.     Publication Date:  1998 Dec 
Date Detail:
Created Date:  1999-03-05     Completed Date:  1999-03-05     Revised Date:  2007-03-21    
Medline Journal Info:
Nlm Unique ID:  7910918     Medline TA:  J Inherit Metab Dis     Country:  NETHERLANDS    
Other Details:
Languages:  eng     Pagination:  837-45     Citation Subset:  IM    
Servei de Bioquímica, Hospital Universitari Sant Joan de Déu, Barcelona, Spain.
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MeSH Terms
Alanine / blood
Ascorbic Acid / therapeutic use
Carnitine / blood,  therapeutic use
Child, Preschool
Erythrocytes / metabolism
Lactic Acid / blood
Lymphocytes / metabolism
Mitochondrial Encephalomyopathies / blood*,  drug therapy*
Oxygen Consumption
Riboflavin / therapeutic use
Ubiquinone / blood,  therapeutic use
Vitamin E / blood,  therapeutic use
Vitamin K / therapeutic use
Reg. No./Substance:
12001-79-5/Vitamin K; 1339-63-5/Ubiquinone; 1406-18-4/Vitamin E; 50-21-5/Lactic Acid; 50-81-7/Ascorbic Acid; 541-15-1/Carnitine; 56-41-7/Alanine; 83-88-5/Riboflavin

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