Document Detail


Effect of dietary Lorenzo's oil and docosahexaenoic acid treatment for Zellweger syndrome.
MedLine Citation:
PMID:  18983586     Owner:  NLM     Status:  MEDLINE    
Abstract/OtherAbstract:
We investigated the possible therapeutic effect of decreasing plasma levels of very-long-chain fatty acids (C26:0) with a synthetic oil containing trioleate and trielucate (Lorenzo's oil) as well as increasing docosahexaenoic acid (DHA) in red blood cells (RBC) with DHA ethyl ester in four patients with Zellweger syndrome. We investigated serial changes of plasma C26:0 levels and DHA levels in RBC membranes by gas-liquid chromatography/mass spectrometry (GC/MS). After death, the fatty acid composition of each patient's cerebrum and liver was studied. Dietary administration of Lorenzo's oil diminished plasma C26:0 levels. Earlier administration of Lorenzo's oil was more effective and the response did not depend on the duration of administration. DHA was incorporated into RBC membrane lipids when administrated orally, and its level increased for several months. The final DHA level was correlated with the duration of administration and was not related to the timing of initiation of treatment. DHA levels in the brains and livers of treated patients were higher than in untreated patients. Early initiation of Lorenzo's oil and the long-term administration of DHA may be useful for patients with Zellweger syndrome.
Authors:
Yasuhiro Arai; Yohei Kitamura; Masaharu Hayashi; Kyoichi Oshida; Toshiaki Shimizu; Yuichiro Yamashiro
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Publication Detail:
Type:  Journal Article    
Journal Detail:
Title:  Congenital anomalies     Volume:  48     ISSN:  0914-3505     ISO Abbreviation:  Congenit Anom (Kyoto)     Publication Date:  2008 Dec 
Date Detail:
Created Date:  2008-11-05     Completed Date:  2009-01-21     Revised Date:  -    
Medline Journal Info:
Nlm Unique ID:  9306292     Medline TA:  Congenit Anom (Kyoto)     Country:  Japan    
Other Details:
Languages:  eng     Pagination:  180-2     Citation Subset:  IM    
Affiliation:
Department of Pediatrics, Juntendo University, School of Medicine, Tokyo, Japan. aria_trc@mtrc.jp
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MeSH Terms
Descriptor/Qualifier:
Brain Chemistry
Docosahexaenoic Acids / blood,  therapeutic use*
Drug Combinations
Erucic Acids / therapeutic use*
Fatty Acids / analysis
Female
Humans
Infant
Liver / chemistry
Male
Triolein / therapeutic use*
Zellweger Syndrome / diet therapy*
Chemical
Reg. No./Substance:
0/Drug Combinations; 0/Erucic Acids; 0/Fatty Acids; 0/Lorenzo's oil; 122-32-7/Triolein; 25167-62-8/Docosahexaenoic Acids

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


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