Odièvre M, Gentil C, Gautier M, Alagille D
Am J Dis Child. 1978 Jun;132(6):605-8. doi: 10.1001/archpedi.1978.02120310069014.
The early manifestations of hereditary fructose intolerance are described in a series of 55 patients. Management of this metabolic disorder depends on the severity of liver impairment. When the patients are given a fructose-free diet, the improvement is a dramatic but liver enlargement and fatty vacuolization of liver cells often persist. These hepatic findings were also observed in the five homozygous infants who were given a fructose-free diet from birth; this outcome may support the hypothesis that minimal amounts of fructose are esential for human beings.
对55例患者的系列研究描述了遗传性果糖不耐受的早期表现。这种代谢紊乱的治疗取决于肝损伤的严重程度。当患者采用无果糖饮食时，病情改善显著，但肝脏肿大和肝细胞脂肪空泡化往往持续存在。在5名从出生就采用无果糖饮食的纯合子婴儿中也观察到了这些肝脏表现；这一结果可能支持以下假说，即极少量的果糖对人类至关重要。
