Hereditary fructose intolerance
Summary of Hereditary fructose intolerance
Hereditary fructose intolerance is a fructose metabolism disorder caused by a deficiency of the enzyme aldolase B. Affected individuals are asymptomatic until ingesting fructose, sucrose, or sorbitol, when the deficiency of aldolase B causes an accumulation of fructose-1-phosphate, which in turn affects glycogenolysis, gluconeogenesis, and regeneration of adenosine triphosphate. Clinical symptoms of fructose intolerance include vomiting, hypoglycemia, jaundice, hemorrhage, hepatomegaly, hyperuricemia, and potentially kidney failure.