Mendoza MA

Language: Spanish
References: 21
Page: 111-119
PDF size: 336.56 Kb.

ABSTRACT

Both starvation and metabolic stress activate fatty acid oxidation in order to support energetically the organism, yielding important amount of ketone bodies in normal subjects. In patients with medium-chain acyl-CoA dehydrogenase deficiency, the fatty acid oxidation can not be completed and both acetyl CoA and ketone bodies are not produced. In addition, metabolites which can not suffer further oxidation are accumulated and are responsible for hepatic damage associated with no transformation of ammonia into urea. Patients on starvation or metabolic stress develop hypoketogenic hypoglycemia with severe neurological compromise. Neurological alterations are due to both hypoglycemia itself, which occurs as a consequence of excessive utilization of glucose because of the unavailability of fat-derived energy, and hyperammonemia due to hepatic damage.

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