Document Detail

Odd-numbered long-chain fatty acids in erythrocyte phospholipids as long-term follow-up parameter in propionic acidemia.
MedLine Citation:
PMID:  15497464     Owner:  NLM     Status:  MEDLINE    
Odd-numbered long-chain fatty acids (OLCFAs) increase in total erythrocyte lipids or plasma in patients with propionic acidemia and have been proposed as a useful parameter of metabolic control. However, up to now no parameter for long-term metabolic control has been available for this disorder. In contrast to previous investigations, we investigated OLCFAs with particular reference to differences for two phospholipid fractions of erythrocyte lipids. The phosphatidylethanolamine (PE) fraction of erythrocyte lipids has a slow turnover rate and might reflect the metabolic propionyl-CoA burden over a period of some weeks. Distinct increases in OLCFA of up to 200% in the phosphatidylcholine fraction were observed after episodes of metabolic decompensation and maximum OLCFA levels were found 3 weeks after the onset of a crisis. As a sign of poor metabolic control, OLCFA levels in PE steadily increased after recurrent episodes of decompensation, but not in clinically stable patients. However, in one patient with clinically good metabolic control, OLCFAs continually increased after the second year of life for an as yet unknown reason. In conclusion, data from this study indicate the usefulness of OLCFA analysis in the PE fraction of erythrocyte lipids, in particular with regard to the need for such a parameter for evidence-based improvement of management strategies in propionic acidemia.
Thomas Meissner; Michael Leichsenring; Ertan Mayatepek
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Publication Detail:
Type:  Journal Article    
Journal Detail:
Title:  Clinical chemistry and laboratory medicine : CCLM / FESCC     Volume:  42     ISSN:  1434-6621     ISO Abbreviation:  Clin. Chem. Lab. Med.     Publication Date:  2004  
Date Detail:
Created Date:  2004-10-22     Completed Date:  2005-08-04     Revised Date:  -    
Medline Journal Info:
Nlm Unique ID:  9806306     Medline TA:  Clin Chem Lab Med     Country:  Germany    
Other Details:
Languages:  eng     Pagination:  1005-8     Citation Subset:  IM    
Department of General Pediatrics, University Children's Hospital, Düsseldorf, Germany.
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MeSH Terms
Biological Markers / analysis*
Child, Preschool
Disease Progression
Erythrocytes / chemistry*
Fatty Acids / analysis*
Metabolism, Inborn Errors / pathology*,  therapy*
Mitochondria / chemistry
Monitoring, Physiologic
Phospholipids / chemistry
Propionic Acids / blood*
Sensitivity and Specificity
Reg. No./Substance:
0/Biological Markers; 0/Fatty Acids; 0/Phospholipids; 0/Propionic Acids

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