Abstract
Octanoic and other short and medium chain free fatty acid concentrations (FFA) have been noted to be elevated in Reye syndrome and infusion of these substances has produced a similar syndrome in animals. Thus, these substances have been implicated as causal factors for some of the symptoms in this problem. MCT's are used to treat a variety of children. They have been demonstrated to produce elevations of FFA in dogs. We measured medium chain FFA in patients receiving MCT's chronically. A patient with type IB glycogenosis had extreme elevations of C7-C10 FFA. She also had elevated butyrate and isobutyrate but not propionate or valerate levels. Patients receiving MCT's as part of a ketogenic diet had mildly elevated concentrations of C8 and C9 FFA and variable concentrations of C7 FFA. None of these patients had cerebral symptoms. Thus, elevations of medium chain FFA may be a secondary phenomenon in Reye syndrome.
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Hillman, R., Devivo, D., Keating, J. et al. 860 MARKED ELEVATIONS OF OCTANOIC ACID AND OTHER MEDIUM CHAIN FREE FATTY ACIDS UNASSOCIATED WITH CEREBRAL SYMPTOMS. Pediatr Res 12 (Suppl 4), 507 (1978). https://doi.org/10.1203/00006450-197804001-00865
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DOI: https://doi.org/10.1203/00006450-197804001-00865