Pediatric hereditary fructose intolerance

Hereditary fructose intolerance is a congenital metabolic disorder caused by a deficiency of fruetaldolase (fructose-1, 6-diphosphate aldolase). There are three types of disorders of the fructose metabolism pathway caused by autosomal recessive inheritance: 1. fructokinase deficiency (or essential fructosuria), 2. hereditary fructose intolerance ), 3. fructose-1,6-diphosphatase deficiency. Mainly manifested in hypoglycemia and vomiting after eating fructose-containing food.