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 he normalized by the oral administration of microencapsulated DHA and AA.
| Original language | English (US) |
|---|---|
| Pages (from-to) | 187-197 |
| Number of pages | 11 |
| Journal | Neurochemical Research |
| Volume | 24 |
| Issue number | 2 |
| DOIs | |
| State | Published - 1999 |
| Externally published | Yes |
Bibliographical note
Funding Information:Supported in part by grants FDR 001289 and 000685 from the Food and Drug Administration, and RR 00052 General Clinical Research Center from the National Institutes of Health. We thank the Martek Biosciences Corporation for the supply of the DHA and AA preparations (DHASCO and ARASCO).
Keywords
- Fatty acids
- Infantile Refsum disease
- Neonatal adrenoleukodystrophy
- Plasmalogens
- X-linked adrenoleukodystrophy
- Zellweger syndrome