Long-Chain L-3 Hydroxyacyl-CoA Dehydrogenase Deficiency (LCHAD)



Long-chain L-3 hydroxyacyl-CoA dehydrogenase deficiency (LCHAD) is a condition in which the body is unable to break down certain fats. Individuals affected by LCHAD are unable to convert some of the fats they eat into energy the body needs to function. Instead, too many unused fatty acids build up in the body. Detecting the condition early and beginning treatment can prevent many of the severe outcomes of LCHAD. Not all babies with LCHAD show signs of the condition. For babies who do, the first signs often appear between birth and 2 years of age. Signs of LCHAD include sleeping longer or more often, tiredness, behavior changes, irritability, restlessness, nausea and vomiting, and muscle weakness (hypotonia). Many of these signs may occur when a baby eats foods the body cannot break down. Symptoms can be triggered by long periods of time without eating, illnesses, and infections.

Condition Type:

Core Conditions


The incidence of long-chain L-3 hydroxyacyl-CoA dehydrogenase deficiency (LCHAD) deficiency is unknown.

More Information for Parents:
Also known as:
  • Long-chain L-3 hydroxyacyl-CoA dehydrogenase deficiency
  • Long-chain 3-hydroxyacyl-coenzyme a dehydrogenase deficiency
  • Long-chain 3-OH acyl-CoA dehydrogenase deficiency
  • Type 1 trifunctional protein deficiency

Core Conditions