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Fructose-1,6-bisphosphatase deficiency

What is Fructose-1,6-bisphosphatase deficiency?

Fructose-1,6-biphosphatase (FBP) deficiency is a disorder of fructose metabolism (see this term) characterized by recurrent episodes of fasting hypoglycemia with lactic acidosis, that may be life-threatening in neonates and infants.

Other condition names

  • FBPase deficiency
  • Fructose-1,6-diphosphatase deficiency

Inheritance type

Autosomal recessive

Age of Onset

  • All ages
Orphadata: Free access data from Orphanet. © INSERM 1999. Available on "https://www.orphadata.com". Data version 1.3.16 / 4.1.7.