Sachs M, Asskali F, Förster H, Encke A
Klinik für Allgemein- und Abdominalchirurgie, Klinikum der Johann Wolfgang Goethe-Universität, Frankfurt, Main.
Z Ernahrungswiss. 1993 Mar;32(1):56-66. doi: 10.1007/BF01610085.
The present paper reports on an adult female patient whose hereditary fructose intolerance (HFI) was at first not diagnosed and who, within the space of 2 years after repeated elective surgery and the perioperative administration of fructose and sorbitol, developed "hepatic and renal failure of unclear origin." At a later stage we were able to establish the diagnosis of HFI by means of a fructose tolerance test in both she and her brother, for whom intolerance to fruit and desserts had been known since early childhood. In addition, literature references to fatalities following the parenteral application of fructose and sorbitol were analyzed. During the course of fructose infusion in both the patient and her brother with HFI, the following metabolic changes were noted: hypoglycemia, elevated rise in the blood fructose concentration, hyperlactacidemia, elevated rise in the blood fructose concentration, hyperlactacidemia, and hyperammonemia. These metabolic changes proved to be reversible after discontinuing the fructose infusion. Analysis of the literature on the fatalities following parenteral fructose administration established that fruit and dessert intolerance was known for all collated patients with HFI, and that, clearly, no regular metabolic tests had been conducted.
本文报道了一名成年女性患者,其遗传性果糖不耐受(HFI)最初未被诊断出来。在反复进行择期手术后的2年时间里,以及围手术期给予果糖和山梨醇后,她出现了“不明原因的肝肾功能衰竭”。后来,我们通过对她和她哥哥进行果糖耐量试验确诊了HFI,她哥哥自幼就对水果和甜点不耐受。此外,还分析了关于静脉注射果糖和山梨醇后死亡的文献参考资料。在对患有HFI的患者及其哥哥进行果糖输注过程中,观察到以下代谢变化:低血糖、血果糖浓度升高、高乳酸血症、血果糖浓度升高、高乳酸血症和高氨血症。停止果糖输注后,这些代谢变化被证明是可逆的。对静脉注射果糖后死亡的文献分析表明,所有整理的患有HFI的患者都有水果和甜点不耐受的情况,而且显然没有进行常规的代谢测试。