[Hypoglycemia and hyperlactacidemia in relation with a new case of fructose-1,6-diphosphatase deficiency. Determination of enzymatic activity in leukocytes].

A 11.5 month-old girl with recurrent episodes of hypoglycemia and lactic acidosis was identified as having fructose-1,6-diphosphatase deficiency. The diagnosis may be realised with a simple way by an oral fructose tolerance test and the activity dosage of this enzyme in white blood cells. The fructose and sucrose-free diet and avoidance of prolonged fasting resulted in a decrease of hepatomegaly and normal values of lactate between the episodes.