Intolerance,Fructose

《英文msh詞典》Intolerance,Fructose
[入口詞] Intolerance,Fructose
[主題詞] Fructose Intolerance
[英文釋義] An autosomal recessive fructose metabolism disorder due to deficient fructose-1-phosphate aldolase (EC 2.1.2.13) activity,resulting in accumulation of fructose-1-phosphate. The accumulated fructose-1-phosphate inhibits glycogenolysis and gluconeogenesis,causing severe hypoglycemia following ingestion of fructose. Prolonged fructose ingestion in infants leads ultimately to hepatic failure and death. Patients develop a strong distaste for sweet food,and avoid a chronic course of the disease by remaining on a fructose- and sucrose-free diet.