Froesch E R
Clin Endocrinol Metab. 1976 Nov;5(3):599-611. doi: 10.1016/s0300-595x(76)80042-4.
There are fundamental differences between the metabolic fate of fructose and of glucose. Whereas the metabolism of glucose is controlled by hormones such as insulin, fructose uptake and phosphorylation in the liver occurs independently of hormones and its ultimate metabolic fate is unpredictable. Essential fructosuria, a harmless inherited anomaly of fructose metabolism, is the least harmful of the disorders of fructose metabolism. Hereditary fructose intolerance and fructose-1,6-diphosphatase deficiency are discussed in greater detail with regard to biochemical abnormalities and clinical aspects. HFI is most serious in bottle-fed infants who cannot reject their sucrose-containing diet. Patients with HFI will have no clinical symptoms if kept on a fructose-free diet. In contrast, patients with fructose-1,6-diphosphatase deficiency can tolerate frucose. However, severe infections precipitate attacks of hypoglycaemia and lactic acidosis.
果糖和葡萄糖的代谢命运存在根本差异。葡萄糖的代谢受胰岛素等激素控制，而肝脏中果糖的摄取和磷酸化独立于激素发生，其最终代谢命运不可预测。原发性果糖尿症是一种无害的果糖代谢遗传性异常，是果糖代谢紊乱中危害最小的。遗传性果糖不耐受症和果糖-1,6-二磷酸酶缺乏症将在生化异常和临床方面进行更详细的讨论。遗传性果糖不耐受症在无法拒绝含蔗糖饮食的人工喂养婴儿中最为严重。患有遗传性果糖不耐受症的患者如果保持无果糖饮食将没有临床症状。相比之下，果糖-1,6-二磷酸酶缺乏症患者可以耐受果糖。然而，严重感染会引发低血糖和乳酸性酸中毒发作。
