What is oxidative disorder of fat?

The fat oxidative disorder is a hereditary problem that affects chemical reactions associated with the use and storage of fat. This disorder produces accumulation of fatty acids and this causes a decrease in energy production in the cell. Each metabolic disorder is associated with a specific enzyme in the cascade of chemical reactions associated with fat.

This disorder is associated with mitochondrial disorders. This reaction affects energy production within the mitochondria (is inside the cell) and is responsible for producing energy through electron transfer.

Children with this disorder may have hypoglycemia, hypotonia, low weight, persistent vomiting, hepatomegaly among others. Children with autism can have this metabolic disorder.

It is extremely important to make the diagnosis as early as possible to improve the prognosis, especially during childhood. If symptoms occur in childhood and is not diagnosed the prognosis is very poor.

A Geneticist usually does this diagnosis. The treatment is to limit the intake of fats and proteins, and in many cases is associated with a deficiency of carnitine which is supplemented with carnitine.

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