IV glucose should be given as soon as the fall in blood glucose has been documented. Diagnosis and identification of heterozygous carriers of the mutated gene can be made by direct DNA analysis. Avoidance of sugar-containing sweets and fruit results in teeth that are usually free of caries. Fructose-induced hypoglycemia is treated with glucose. A genetic defect of fructokinase resulting in benign and asymptomatic excretion of fructose in the urine.
Inheritance is autosomal recessive.
No treatment is required. The incidence is unknown. The disorder results in fasting hypoglycemia, ketosis, and acidosis and can be fatal in newborns. Febrile illness can trigger later episodes. Symptoms can be relieved by oral glucose, or IV glucose if hypoglycemia is severe.