[Characteristics of a case of hepatic fructose-1,6-diphosphatase deficiency]

The authors report a new case of hepatic fructose-1.6-diphosphatase deficiency, diagnosed in a 14 month-old girl on the occasion of an episode of prostration associated with acidosis, hyperlactacidemia and hypoglycemia without hepatomegaly. The unusual features of this case, especially the glucagon induced hyperglycemia after a 12 hour fast, emphasize the clinical and biological polymorphism of this enzymopathy.