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Plasma and red blood cell fatty acids in peroxisomal disorders.

Authors
  • Moser, A B
  • Jones, D S
  • Raymond, G V
  • Moser, H W
Type
Published Article
Journal
Neurochemical research
Publication Date
Feb 01, 1999
Volume
24
Issue
2
Pages
187–197
Identifiers
PMID: 9972864
Source
Medline
License
Unknown

Abstract

The demonstration of abnormal levels of fatty acids or plasmalogens in plasma or red blood cells is key to the diagnosis of peroxisomal disorders. We report the levels of 62 fatty acids and plasmalogens in patients with X-linked adrenoleukodystrophy (X-ALD), Zellweger syndrome (ZS), neonatal adrenoleukodystrophy (NALD), and infantile Refsum disease (IRD), both at baseline and after dietary interventions. "Lorenzo's Oil" therapy in X-ALD normalizes the levels of saturated very long chain fatty acids in plasma, but leads to reduced levels of omega 6 and other omega 3 fatty acids, and requires monitoring and appropriate dietary supplements. Patients with ZS, NALD and IRD have reduced levels of docosahexaenoic acid (DHA) and arachidonic acid (AA) which can be normalized by the oral administration of microencapsulated DHA and AA.

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