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单词 Fructose Intolerances
释义

Fructose Intolerances


《英文msh词典》Fructose Intolerances
[入口词] Fructose Intolerances
[主题词] Fructose Intolerance
[英文释义] 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.
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