[Fructose metabolism disorders and infusions]

Area of Science:

• Biochemistry
• Clinical Nutrition
• Metabolic Disorders

Context:

• Parenteral nutrition is crucial for patients unable to consume food orally, especially during critical illness or post-surgery.
• Non-glucose energy sources like fructose and D-glucitol (sorbitol) are sometimes considered for parenteral nutrition.
• Hereditary fructose intolerance is a genetic metabolic disorder affecting fructose metabolism.

Purpose:

• To review the metabolic pathways of fructose and its associated disorders.
• To evaluate the safety and efficacy of non-glucose energy sources in parenteral nutrition, particularly in the context of hereditary fructose intolerance.
• To discuss the risks of administering fructose and D-glucitol (sorbitol) to susceptible individuals.

Summary:

• Fructose and D-glucitol (sorbitol) metabolism can lead to life-threatening hypoglycemia in patients with hereditary fructose intolerance, even without prior diagnosis.
• Hepatic and renal failure are potential complications, possibly independent of hypoglycemia, in individuals with this intolerance.
• Current evidence suggests avoiding fructose and D-glucitol (sorbitol) in parenteral nutrition due to these risks, a view supported by their removal from some pharmacopoeias.

Impact:

• Highlights the critical need for careful consideration of non-glucose energy sources in parenteral nutrition, especially in vulnerable patient populations.
• Informs clinical practice regarding the contraindications of fructose and D-glucitol (sorbitol) in parenteral nutrition for individuals with undiagnosed hereditary fructose intolerance.
• Contributes to patient safety by advocating for the exclusion of potentially harmful substances from therapeutic regimens.