LCHAD Deficiency

Definition

LCHAD deficiency is a metabolic disease characterized by long-chain 3-hydroxyacyl-CoA dehydrogenase enzyme deficiency, accompanied by hypoketotic hypoglycemia and liver failure.

Symptoms

  • Hypoketotic hypoglycemia
  • Liver failure
  • Rhabdomyolysis
  • Retinopathy

Reasons

HADHA gene mutation.

Diagnosis

Newborn screening, genetic testing.

Treatment

Dietary modification, MCT oil.

Which Polyclinic Should You Apply To?

Metabolism Polyclinic

What to Do in an Emergency?

In case of metabolic crisis, emergency services should be consulted.

This page is for general information purposes only.