[Repeated perioperative administration of fructose and sorbitol in a female 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 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.
Sachs
M
M
Klinik für Allgemein- und Abdominalchirurgie, Klinikum der Johann Wolfgang Goethe-Universität, Frankfurt, Main.
Asskali
F
F
Förster
H
H
Encke
A
A
D058186:Acute Kidney Injury / Q000139:chemically induced*; D056486:Chemical and Drug Induced Liver Injury; D004573:Electrolytes / Q000008:administration & dosage; D005260:Female; D005632:Fructose / Q000008:administration & dosage* / Q000009:adverse effects; D005633:Fructose Intolerance / Q000209:etiology / Q000235:genetics*; D006801:Humans; D007262:Infusions, Intravenous; D007430:Intraoperative Care; D008875:Middle Aged; D013012:Sorbitol / Q000008:administration & dosage* / Q000009:adverse effects
D004573:Electrolytes; D005632:Fructose; D013012:Sorbitol
Wiederholte perioperative Applikation von Fructose und Sorbit bei einer Patientin mit hereditärer Fructoseintoleranz (HFI).