Plasma and erythrocyte fatty acid concentrations in long-chain 3-hydroxyacyl-CoA dehydrogenase deficiency

Journal of Inherited Metabolic Disease

Volumn 26, Issue 4, 2003, Pages 410-412

  Lund, A M ^a   Dixon, M A ^a   Vreken, P ^b   Leonard, J V ^a   Morris, A A M ^a  

^a GREAT ORMOND STREET HOSPITAL   (United Kingdom)

^b UNIVERSITY OF AMSTERDAM   (Netherlands)

Author keywords

[No Author keywords available]

Indexed keywords

3 HYDROXYACYL COENZYME A DEHYDROGENASE; ARACHIDONIC ACID; CARBOHYDRATE; DOCOSAHEXAENOIC ACID; FATTY ACID; LINOLENIC ACID; MEDIUM CHAIN TRIACYLGLYCEROL;

ARTICLE; CARBOHYDRATE DIET; CHILD; CLINICAL ARTICLE; CONCENTRATION (PARAMETERS); CONTROLLED STUDY; DIET RESTRICTION; DIET THERAPY; ENZYME DEFICIENCY; ERYTHROCYTE LEVEL; FATTY ACID BLOOD LEVEL; HUMAN; LOW FAT DIET; MEDICAL ASSESSMENT; NUTRITIONAL STATUS; PATHOPHYSIOLOGY;

3-HYDROXYACYL COA DEHYDROGENASES; AMINO ACID METABOLISM, INBORN ERRORS; CHILD; CHILD, PRESCHOOL; CHROMATOGRAPHY, GAS; DIET; ERYTHROCYTES; FATTY ACIDS; HUMANS; OSMOLAR CONCENTRATION; PLASMA; POSTPRANDIAL PERIOD;

EID: 0041629654     PISSN: 01418955     EISSN: None     Source Type: Journal    
DOI: 10.1023/A:1025175606891     Document Type: Article

References (4)

1. Dacremont G, Vincent G (1995) Assay of plasmalogens and polyunsaturated fatty acids (PUFA) in erythrocytes and fibroblasts. J Inherit Metab Dis 18(Supplement 1): 84-89.
2. Harding CO, Gillingham MB, van Calcar SC, Wolff JA, Verhoeve JN, Mills MD (1999) Docosahexaenoic acid and retinal function in children with long-chain 3-hydroxyacyl-CoA dehydrogenase deficiency. J Inherit Metab Dis 22: 276-280.
3. Lund AM, Dixon MA, Vreken P, Leonard JV, Morris AAM (2003) What is the role of medium-chain triglycerides in the management of long-chain 3-hydroxyacyl-CoA dehydrogenase deficiency? J Inherit Metab Dis 26: 353-360.
4. Tyni T, Kivela T, Lappi M, Summanen P, Nikoskelainen E, Pihko H (1998) Ophthalmologic findings in long-chain 3-hydroxyacyl-CoA dehydrogenase deficiency caused by the G1528C mutation: a new type of hereditary metabolic chorioretinopathy. Ophthalmology 105: 810-824.