Fructose-1,6-bisphosphatase Deficiency

Definition

Fructose-1,6-bisphosphatase deficiency is a metabolic disease characterized by impaired gluconeogenesis and accompanied by hypoglycemia and metabolic acidosis.

Symptoms

  • Hypoglycemia
  • Lactic acidosis
  • Hyperventilation
  • Hepatomegaly

Reasons

FBP1 gene mutation.

Diagnosis

Genetic testing, metabolic testing.

Treatment

Frequent feeding, fructose restriction.

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.