Methylmalonic Acidemia

Definition

Methylmalonic acidemia is an organic acidemia characterized by methylmalonyl-CoA mutase enzyme deficiency, accompanied by metabolic acidosis and hyperammonemia.

Symptoms

  • Metabolic acidosis
  • Hyperammonemia
  • Vomiting
  • Lethargy

Reasons

MUT gene mutation.

Diagnosis

Urine organic acids, genetic testing.

Treatment

Dietary restriction, B12, L-carnitine.

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.