Polyunsaturated fatty acid deficiency during dietary treatment of very long-chain acyl-CoA dehydrogenase deficiency. Rescue with soybean oil

Journal of Inherited Metabolic Disease

Volumn 24, Issue 4, 2001, Pages 493-503

  Ruiz Sanz, J I ^a   Aldamiz Echevarria, L ^a   Arrizabalaga, J ^a   Aquino, L ^a   Jimeno, P ^a   Perez Nanclares G ^a   Sanjurjo, P ^a  

^a HOSPITAL DE CRUCES   (Spain)

Author keywords

[No Author keywords available]

Indexed keywords

5,8,11 ICOSATRIENOIC ACID; ACYL COENZYME A DEHYDROGENASE; ARACHIDONIC ACID; DOCOSAHEXAENOIC ACID; LINOLEIC ACID; LONG CHAIN FATTY ACID; LONG CHAIN TRIACYLGLYCEROL; MEDIUM CHAIN TRIACYLGLYCEROL; OMEGA 6 FATTY ACID; PHOSPHATIDYLCHOLINE; PHOSPHATIDYLETHANOLAMINE; POLYUNSATURATED FATTY ACID; SOYBEAN OIL;

ARTICLE; CALORIC INTAKE; CASE REPORT; DIET THERAPY; DISEASE COURSE; DISEASE PREDISPOSITION; ENZYME DEFICIENCY; ESSENTIAL FATTY ACID DEFICIENCY; FATTY ACID BLOOD LEVEL; FEMALE; HEART VENTRICLE HYPERTROPHY; HUMAN; HYPOGLYCEMIA; INFANT; LIPID DIET; NUTRITION; RETINA PIGMENT DEGENERATION; RETINOPATHY;

ACYL-COA DEHYDROGENASE, LONG-CHAIN; ARACHIDONIC ACID; CONSANGUINITY; DIET; DOCOSAHEXAENOIC ACIDS; ENERGY INTAKE; ERYTHROCYTES; FATTY ACIDS, OMEGA-3; FATTY ACIDS, OMEGA-6; FATTY ACIDS, UNSATURATED; FEMALE; HUMANS; INFANT; LINOLEIC ACID; LIPIDS; PHOSPHATIDYLCHOLINES; PHOSPHATIDYLETHANOLAMINES; SOYBEAN OIL;

EID: 0034827804     PISSN: 01418955     EISSN: None     Source Type: Journal    
DOI: 10.1023/A:1010533730659     Document Type: Article

References (25)

1. ESPGAN Committee on Nutrition
2. A novel disease with deficiency in mitochondrial very-long-chain acyl-CoA dehydrogenase
3. Very long chain acyl-CoA dehydrogenase deficiency: Identification of a new error of mitochondrial fatty acid oxidation by fibroblasts
4. Very long chain CoA dehydrogenase deficiency: Successful treatment of acute cardiomyopathy
5. Cornstarch therapy in type I glycogen storage disease
6. Composition of phospholipids and phospholipid fatty acids and aldehydes in human red cells
7. Dietary management of long-chain 3-hydroxyacyl-CoA dehydrogenase deficiency (LCHADD). A case report and survey
8. Long-chain acyl coenzyme A dehydrogenase deficiency: An inherited cause of nonketotic hypoglycemia
9. The long chain acyl-CoA dehydrogenase deficiency
10. Docosahexaenoic acid and retinal function in children with long chain 3-hydroxyacyl-CoA dehydrogenase deficiency
11. A function for the vitamin E metabolite α-tocopherol quinone as an essential enzyme cofactor for the mitochondrial fatty acid desaturases
12. On the molecular etiology of decreased arachidonic (20:4n-6), docosapentaenoic (22:5n-6) and docosahexaenoic (22:6n-3) acids in Zellweger syndrome and other peroxisomal disorders
13. Secondary carnitine deficiency and impaired docosahexaenoic (22:6n-3) acid synthesis: A common denominator in the pathophysiology of diseases of oxidative phosphorylation and β-oxidation
14. Improved recovery of fatty acids through direct transesterification without prior extraction or purification
15. Direct transesterification of all clases of lipids in one step reation
16. Adolescent myopathic presentation in two sisters with very long-chain acyl-CoA dehydrogenase deficiency
17. Very long-chain acyl coenzyme A dehydrogenase deficiency presenting with exercise-induced myoglobinuria
18. Mitochondrial fatty acid oxidation disorders
19. Quantification in the subnanomolar range of phospholipids and neutral lipids by monodimensional thin-layer chromatography and image analysis
20. Very long chain acyl-coenzyme A dehydrogenase deficiency with adult onset
21. Reevaluation of the pathways for the biosynthesis of polyunsaturated fatty acids
22. L-3-Hydroxyacyl-CoA dehydrogenase deficiency: Two cases with pigmentary retinopathy
23. Mitochondrial very-long-chain acyl-coenzyme A dehydrogenase deficiency: Clinical characteristics and diagnostic considerations in 30 patients
24. The metabolism of 7,10,13,16,19-docosapentaenoic acid is independent of a 4-desaturase
25. Identification of very-long-chain acyl-CoA dehydrogenase deficiency in three patients previously diagnosed with long-chain acyl-CoA dehydrogenase deficiency