Fructose-1,6-bisphosphatase deficiency
Fructose-1,6-bisphosphatase deficiency
Definition
Fructose-1,6-biphosphatase (FBP) deficiency is a disorder of fructose metabolism characterized by recurrent episodes of fasting hypoglycemia with lactic acidosis, that may be life-threatening in neonates and infants.
Also known as fructose-1,6-bisphosphatase deficiency, fructose-1,6-diphosphatase deficiency — per MONDO