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Long-chain 3-hydroxyacyl-CoA dehydrogenase deficiency

(Long • chain • 3 • hie-DRAHK-see-ace-uhl • koh-AY • dee-hie-DRAH-juh-nace • di-FISH-uhn-see)

General Condition Information

Other Names

  • LCHAD deficiency
  • Long chain 3 hydroxyacyl-CoA dehydrogenase deficiency
  • Long chain 3 hydroxyacyl‑CoA dehydrogenase deficiency
  • Long chain 3 hydroxyl acyl-CoA dehydrogenase deficiency
  • Long chain 3-hydroxyacyl CoA dehydrogenase deficiency
  • Long chain 3-hydroxyacyl-CoA dehydrogenase deficiency
  • Long chain 3-OH coA dehydrogenase deficiency
  • Long chain hydroxy acyl-CoA dehydrogenase deficiency
  • Long chain hydroxyacyl CoA dehydrogenase deficiency
  • Long chain hydroxyacyl-CoA dehydrogenase deficiency
  • Long chain hydroxyl acylCoA dehydrogenase deficiency
  • Long chain L-3-hydroxyacyl-CoA dehydrogenase deficiency
  • Long-chain 3-OH acyl-CoA dehydrogenase deficiency
  • Long-chain hydroxyacyl-CoA dehydrogenase deficiency
  • Long-chain L-3 hydroxy acyl-CoA dehydrogenase deficiency
  • Long-chain L-3 hydroxyacyl-CoA dehydrogenase
  • Long-chain L-3 hydroxyacyl-CoA dehydrogenase deficiency
  • Long-chain L-3-hydroxy acyl-CoA dehydrogenase deficiency
  • Long-chain L-3-hydroxyacyl- CoA dehydrogenase deficiency
  • Long-chain L-3-hydroxyacyl-CoA dehydrogenase
  • Long-chain L-3-hydroxyacyl-CoA dehydrogenase deficiency
  • Long-chain L-3-OH acyl-CoA dehydrogenase deficiency

Condition Type

Birth Prevalence

It is unknown how many babies are born with this rare condition each year in the United States.

Screening Finding

Elevated C16-OH +/- C18 and other long chain acylcarnitines

What is Long-chain 3-hydroxyacyl-CoA dehydrogenase deficiency

Long-chain 3-hydroxyacyl-CoA dehydrogenase (LCHAD) deficiency is an inherited (genetic) condition that prevents the body from breaking down certain fats and turning them into energy.

Certain enzymes help process a type of fat called long-chain fatty acids. Without enough of these enzymes, your baby will have trouble using fats for energy. This condition can be more or less severe depending on how much of these enzymes your baby can make.

Breaking down fat for energy allows the body to work properly, and it is especially important after a long time without food (fasting) and during illness. If your baby does not make enough energy from fat, blood sugar levels can become dangerously low.

When fats are not processed, waste (including toxins) can build up and damage your heart, muscles, and eyes. If untreated, the lack of energy and increased toxins lead to the signs and symptoms of the condition.

Newborn Screening and Follow-Up

Condition Details

Treatment and Management

It is important to talk to your health care provider about which treatment(s) are best for your baby. The goal of treatment is to prevent the health problems caused by this condition. 

Treatments may include the following:

  • Regular and frequent meals and snacks
  • Diet high in carbohydrates and low in fat
  • Medium chain triglyceride (MCT) oil to help give the body fats it can break down
  • L-carnitine supplements to help the body break down fats
  • People with LCHAD deficiency must be very careful if they get sick and have vomiting or diarrhea, or do not want to eat. They may need emergency care to prevent low blood sugar levels.

Children who receive early and ongoing treatment for LCHAD deficiency can have healthy growth and development. Continuing care is needed for problems that occur later in affected people (muscle and vision problems).

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