What is the role of medium-chain triglycerides in the management of long-chain 3-hydroxyacyl-CoA dehydrogenase deficiency?
Cardiomyopathy is common in infants with long-chain 3-hydroxyacyl-CoA dehydrogenase deficiency. Resolution of the cardiomyopathy can often be achieved by avoidance of fasting and changing from a conventional infant formula to one in which most long-chain fat is replaced by medium-chain triglycerides (MCT). It is uncertain whether the clinical improvement is due to the restriction of long-chain fat or whether the MCT have specific beneficial effects. To clarify this, the metabolic effects of MCT were examined in 5 patients. When given at around the level found in MCT-based infant formula, MCT had no effect on blood concentrations of ketone bodies, specific fatty acids or acylcarnitines. The present study cannot, however, exclude the possibility that MCT per se may have beneficial effects.
Lund
A M
AM
Metabolic Department, Great Ormond Street Hospital for Children, London, UK.
Dixon
M A
MA
Vreken
P
P
Leonard
J V
JV
Morris
A A M
AA
D015094:3-Hydroxyacyl CoA Dehydrogenases / Q000172:deficiency*; D002331:Carnitine / Q000031:analogs & derivatives* / Q000097:blood; D002648:Child; D002675:Child, Preschool; D005230:Fatty Acids, Nonesterified / Q000097:blood; D005526:Food, Formulated; D006801:Humans; D008661:Metabolism, Inborn Errors / Q000097:blood / Q000178:diet therapy / Q000503:physiopathology; D015656:Respiratory Mechanics; D014280:Triglycerides / Q000008:administration & dosage* / Q000737:chemistry*
D005230:Fatty Acids, Nonesterified; D014280:Triglycerides; C116917:acylcarnitine; D015094:3-Hydroxyacyl CoA Dehydrogenases; D002331:Carnitine