Hereditary fructose intolerance

There are three kinds of obstacles to the fructose metabolism pathway caused by autosomal recessive inheritance: 1. Fructose kinase deficiency (or idiopathic fructouria, essential fructosuria) is caused by the lack of fructokinase in the liver, making fructose unable to carry out phosphate It can not be further metabolized in the liver, so the fructose concentration in the blood of the patient is significantly increased after ingestion of fructose and excreted from the urine. The disease has no obvious clinical symptoms. The significance of correct diagnosis is to prevent misdiagnosis of diabetes and add treatment. 2. Hereditary fructose intolerance is caused by fruetaldolase (fructose-, 6-diphosphatealdolase) deficiency, which is the focus of this article. 3. Fructose-1,6-bisphosphatase deficiency, which is a catalytic enzyme in the glucose metabolism pathway, but it is customarily summarized in fructose metabolism defects.