Pyruvate Dehydrogenase Deficiency

Definition

Pyruvate dehydrogenase deficiency is a metabolic disease characterized by pyruvate metabolism disorder, lactic acidosis, and neurological disorders.

Symptoms

  • Lactic acidosis
  • Neurological decline
  • Hypotonia
  • Convulsions

Reasons

PDHA1 and other gene mutations.

Diagnosis

Enzyme activity, genetic testing.

Treatment

Ketogenic diet, thiamine.

Which Polyclinic Should You Apply To?

Metabolism Polyclinic

What to Do in an Emergency?

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

This page is for general information purposes only.