OpenLB
Open Library of Bioscience
Advanced Search
Journal of inherited metabolic disease
Aug, 2001;24 (4): 493-503.

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

J I Ruiz-Sanz, L Aldamiz-Echevarria, J Arrizabalaga, L Aquino, P Jimeno, G Pérez-Nanclares, P Sanjurjo
Author Information
  1. J I Ruiz-Sanz: Paediatrics Department, University Cruces Hospital, Cruces-Baracaldo, Spain.
PMID: 11596652 DOI: 10.1023/a:1010533730659

Abstract

Nutritional management of very long-chain acyl-CoA dehydrogenase (VLCAD) deficiency is based on the avoidance of fasting and substitution of medium-chain triglycerides for long- and very long-chain triglycerides. We report two cases of this disease, which developed omega-6 essential fatty acid deficiency after three and five months from the beginning of nutritional therapy (SHS product: Monogen). This alteration could be especially dangerous in these patients owing to their possible susceptibility to the development of pigmentary retinopathy. The incorporation of linoleic acid as 3-4% of total caloric intake supported as soybean oil ameliorates this deficiency. We wish to remark on this early complication in the nutritional management of VLCAD deficiency and the possibility of rescue by the incorporation of soybean oil into the diet.

References

  1. Biochem Biophys Res Commun. 1993 Mar 31;191(3):1369-72 [PMID: 8466512]
  2. J Biol Chem. 1991 Oct 25;266(30):19995-20000 [PMID: 1834642]
  3. Clin Chim Acta. 1998 Jan 12;269(1):43-62 [PMID: 9498103]
  4. J Inherit Metab Dis. 1999 Apr;22(2):123-31 [PMID: 10234607]
  5. J Lipid Res. 1967 Nov;8(6):667-75 [PMID: 6057495]
  6. Neurology. 1994 Mar;44(3 Pt 1):467-73 [PMID: 8145917]
  7. J Inherit Metab Dis. 1999 May;22(3):276-80 [PMID: 10384386]
  8. Biochem Mol Med. 1996 Jun;58(1):59-65 [PMID: 8809347]
  9. J Lipid Res. 1997 Jul;38(7):1482-9 [PMID: 9254073]
  10. Prog Clin Biol Res. 1990;321:303-11 [PMID: 2326295]
  11. Pediatr Res. 1985 Jul;19(7):666-71 [PMID: 4022672]
  12. J Lipid Res. 1995 Dec;36(12):2471-7 [PMID: 8847474]
  13. J Inherit Metab Dis. 1999 Oct;22(7):802-10 [PMID: 10518280]
  14. FEBS Lett. 2000 Feb 18;468(1):1-5 [PMID: 10683429]
  15. FEBS Lett. 1999 Mar 5;446(1):1-5 [PMID: 10100602]
  16. Ann Neurol. 1998 Apr;43(4):540-4 [PMID: 9546340]
  17. J Lipid Res. 1986 Jan;27(1):114-20 [PMID: 3958609]
  18. J Lipid Res. 1984 Dec 1;25(12):1391-6 [PMID: 6530596]
  19. J Inherit Metab Dis. 1997 Nov;20(6):848-50 [PMID: 9427163]
  20. Biochim Biophys Acta. 1993 Jan 22;1180(3):327-9 [PMID: 8422439]
  21. Pediatr Res. 1993 Jul;34(1):111-3 [PMID: 8356011]
  22. Acta Paediatr Scand. 1991 Aug-Sep;80(8-9):887-96 [PMID: 1957613]
  23. Mol Cell Biochem. 1997 Mar;168(1-2):101-15 [PMID: 9062899]

MeSH Term

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

Chemicals

Fatty Acids, Omega-3
Fatty Acids, Omega-6
Fatty Acids, Unsaturated
Lipids
Phosphatidylcholines
Phosphatidylethanolamines
Docosahexaenoic Acids
Arachidonic Acid
Soybean Oil
Linoleic Acid
Acyl-CoA Dehydrogenase, Long-Chain
Case Reports Journal Article

Word Cloud

Created with Highcharts 10.0.0deficiencylong-chainacidsoybeanoilmanagementacyl-CoAdehydrogenaseVLCADtriglyceridesfattynutritionalincorporationNutritionalbasedavoidancefastingsubstitutionmedium-chainlong-reporttwocasesdiseasedevelopedomega-6essentialthreefivemonthsbeginningtherapySHSproduct:Monogenalterationespeciallydangerouspatientsowingpossiblesusceptibilitydevelopmentpigmentaryretinopathylinoleic3-4%totalcaloricintakesupportedameliorateswishremarkearlycomplicationpossibilityrescuedietPolyunsaturateddietarytreatmentRescue

Similar Articles

Cited By