ABSTRACT
In view of repeated communications in recent years reporting on lethal infusions of fructose or sorbitol in adults with hereditary fructose intolerance, the known statements on the incidence of 1:20,000 are critically analysed. The validity is relativated. The special indication for sorbitol as an osmotherapeutic preparation for lowering intracranial pressure is pointed out. A modified intravenous fructose tolerance test is suggested.
Subject(s)
Brain Diseases/surgery , Fructose Intolerance/mortality , Fructose Metabolism, Inborn Errors/mortality , Fructose/adverse effects , Sorbitol/adverse effects , Adult , Fructose Intolerance/genetics , Humans , Infusions, Intravenous , Risk FactorsABSTRACT
A 13-year-old girl with previously undiagnosed fructose intolerance was operated on for acute appendicitis. Postoperatively she received several infusions containing fructose or sorbitol. Haematemesis occurred on the fourth postoperative day, as well as tarry stools and jaundice. Blood sugar was 2 mg/100 ml, Quick test 3%, liver enzymes were markedly elevated, serum bilirubin was over 9 mg/100 ml, and there was a metabolic acidosis. Despite intensive treatment, including haemodialysis and plasmapheresis, she died on the 11th postoperative day.