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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
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. Data version 1.3.16 / 4.1.7.