Document Detail

Long-chain polyunsaturated fatty acids in inborn errors of metabolism.
MedLine Citation:
PMID:  22254065     Owner:  NLM     Status:  MEDLINE    
The treatment of children with inborn errors of metabolism (IEM) is mainly based on restricted dietary intake of protein-containing foods. However, dietary protein restriction may not only reduce amino acid intake, but may be associated with low intake of polyunsaturated fatty acids as well. This review focuses on the consequences of dietary restriction in IEM on the bioavailability of long-chain polyunsaturated fatty acids (LCPUFAs) and on the attempts to ameliorate these consequences. We were able to identify during a literature search 10 observational studies investigating LCPUFA status in patients with IEM and six randomized controlled trials (RCTs) reporting effect of LCPUFA supplementation to the diet of children with IEM. Decreased LCPUFA status, in particular decreased docosahexaenoic acid (DHA) status, has been found in patients suffering from IEM based on the evidence of observational studies. LCPUFA supplementation effectively improves DHA status without detectable adverse reactions. Further research should focus on functional outcomes of LCPUFA supplementation in children with IEM.
Katalin Fekete; Tamás Decsi
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Publication Detail:
Type:  Journal Article; Review     Date:  2010-09-15
Journal Detail:
Title:  Nutrients     Volume:  2     ISSN:  2072-6643     ISO Abbreviation:  Nutrients     Publication Date:  2010 Sep 
Date Detail:
Created Date:  2012-02-08     Completed Date:  2012-03-29     Revised Date:  2013-06-26    
Medline Journal Info:
Nlm Unique ID:  101521595     Medline TA:  Nutrients     Country:  Switzerland    
Other Details:
Languages:  eng     Pagination:  965-74     Citation Subset:  IM    
Department of Pediatrics, University of Pécs, József A. u. 7., H-7623 Pécs, Hungary.
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MeSH Terms
Amino Acids / administration & dosage
Arachidonic Acid / administration & dosage
Biological Availability
Child, Preschool
Diet, Protein-Restricted / adverse effects
Docosahexaenoic Acids / administration & dosage
Fatty Acids, Unsaturated* / administration & dosage,  pharmacokinetics
Linoleic Acid / administration & dosage
Metabolism, Inborn Errors / diet therapy*,  metabolism
Randomized Controlled Trials as Topic
alpha-Linolenic Acid / administration & dosage
Reg. No./Substance:
0/Amino Acids; 0/Fatty Acids, Unsaturated; 0/Placebos; 2197-37-7/Linoleic Acid; 25167-62-8/Docosahexaenoic Acids; 463-40-1/alpha-Linolenic Acid; 506-32-1/Arachidonic Acid

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