Polyunsaturated fatty acid deficiency during dietary treatment of very long-chain acyl-CoA dehydrogenase deficiency. Rescue with soybean oil.
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.
Ruiz-Sanz
J I
JI
Paediatrics Department, University Cruces Hospital, Cruces-Baracaldo, Spain.
Aldamiz-Echevarria
L
L
Arrizabalaga
J
J
Aquino
L
L
Jimeno
P
P
Pérez-Nanclares
G
G
Sanjurjo
P
P
D044942:Acyl-CoA Dehydrogenase, Long-Chain / Q000172:deficiency*; D016718:Arachidonic Acid / Q000097:blood; D003241:Consanguinity; D004032:Diet; D004281:Docosahexaenoic Acids / Q000097:blood; D002149:Energy Intake; D004912:Erythrocytes / Q000737:chemistry; D015525:Fatty Acids, Omega-3 / Q000097:blood; D043371:Fatty Acids, Omega-6; D005231:Fatty Acids, Unsaturated / Q000097:blood / Q000172:deficiency*; D005260:Female; D006801:Humans; D007223:Infant; D019787:Linoleic Acid / Q000008:administration & dosage; D008055:Lipids / Q000097:blood; D010713:Phosphatidylcholines / Q000097:blood; D010714:Phosphatidylethanolamines / Q000097:blood; D013024:Soybean Oil / Q000627:therapeutic use*
D015525:Fatty Acids, Omega-3; D043371:Fatty Acids, Omega-6; D005231:Fatty Acids, Unsaturated; D008055:Lipids; D010713:Phosphatidylcholines; D010714:Phosphatidylethanolamines; D004281:Docosahexaenoic Acids; D016718:Arachidonic Acid; D013024:Soybean Oil; D019787:Linoleic Acid; D044942:Acyl-CoA Dehydrogenase, Long-Chain