REPEATED PERIOPERATIVE ADMINISTRATION OF FRUCTOSE AND SORBITOL IN A PATIENT WITH HEREDITARY FRUCTOSE INTOLERANCE (HFI)

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 periopera tive administration of fructose and sorbitol, developed ''hepatic and renal failure of unclear origin.'' At a later stage we were able to es tablish the diagnosis of HFI by means of a fructose tolerance test in both she and her brother, for whom intolerance to fruit and desserts h ad been known since early childhood. In addition, literature reference s to fatalities following the parenteral application of fructose and s orbitol 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 concentr ation, hyperlactacidemia, elevated rise in the blood fructose concentr ation, hyperlactacidemia, and hyperammonemia. These metabolic changes proved to be reversible after discontinuing the fructose infusion. Ana lysis of the literature on the fatalities following parenteral fructos e administration established that fruit and dessert intolerance was kn own for all collated patients with HFI, and that, clearly, no regular metabolic tests had been conducted.