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果糖不耐受症又名先天性果糖血症,是一种遗传性疾病,由于先天缺乏或部分缺乏果糖代谢中的1—磷酸果糖醛缩酶或1.6—二磷酸果糖醛缩酶,而导致果糖代谢阻滞。可使肝细胞内,1—磷酸果糖含量升高,造成肝内磷酸化酶、果糖激酶受抑制,而干扰了肝细胞内糖原分解及粮原异生作用,使肝脏释放葡萄糖的功能受阻,故病人进食果糖后,发生低血糖,有些病例早期低血糖发作可造成永久性脑损伤。该病1975年统计,在瑞士发病率为1/2000,在英国为1/4000,国内未见报道。
Fructose intolerance, also known as congenital fructosemia, is a hereditary disease that causes fructose due to an inborn deficiency or partial deficiency of 1-phosphoglucosidase or 1.6-diphosphate fructose aldolase in fructose metabolism Metabolic block. Can make liver cells, 1-phosphate fructose content increased, resulting in intrahepatic phosphorylase, fructokinase inhibited, and interfere with the liver cells within the decomposition of glycogen and grain allergens, the function of the liver to block the release of glucose, Therefore, patients eating fructose, hypoglycemia, and some cases of early hypoglycemia can cause permanent brain damage. Statistics of the disease in 1975, the incidence in Switzerland was 1/2000, 1/4000 in the United Kingdom, no domestic reports.