3-hydroxy-3-methylglutaric aciduria (HMG)

Treatment

Is there a treatment for 3-Hydroxy-3-Methylglutaric Aciduria (HMG)?

The current treatment for 3-Hydroxy-3-methylglutaric aciduria is a strict diet. There are specific formulas for infants that are made specifically for this condition. These formulas may also be utilized through an individual's lifetime to ensure adequate and appropriate nutrition. The specialized formulas and medical foods are low in leucine, the protein building block that individuals with HMG CoA lyase deficiency are unable to break down appropriately. Furthermore, dietary modifications may include avoiding foods with high leucine levels. A dietitian will work with you closely to ensure that your child's diet is optimized for adequate nutrition and providing enough calories to promote growth and development.

Because of the risk of low blood sugar, parents are advised to avoid prolonged fasting. The number of hours or duration of fasting will be determined by your metabolic specialist based on your child's medical concerns, growth and development, and age. A doctor may also advise that a patient take L-carnitine. This is a substance that the body naturally makes. It is an over-the-counter supplement that can assist the body in utilizing alternative energy pathways.

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