Glycogen Storage Disease Type I (Von Gierke)

Definition

Glycogen storage disease type I is a metabolic disease characterized by glucose-6-phosphatase enzyme deficiency and characterized by hypoglycemia, hepatomegaly and lactic acidosis.

Symptoms

  • Hypoglycemia
  • Hepatomegaly
  • Lactic acidosis
  • Growth retardation

Reasons

G6PC gene mutation.

Diagnosis

Liver biopsy, genetic testing.

Treatment

Frequent feeding, corn starch.

Which Polyclinic Should You Apply To?

Metabolism Polyclinic

What to Do in an Emergency?

In case of hypoglycemia, emergency services should be consulted.

This page is for general information purposes only.