Clinical Nutrition
Hereditary Fructose Intolerance: a hands-on approach
Fabiola Di Dato, Simona Spadarella, Fabrizia Chiatto, Maria Immacolata
Dipartimento di Scienze Mediche Traslazionali, Sezione di Pediatria, Università degli studi di Napoli Federico II
Hereditary fructose intolerance (HFI) is a genetic disease that requires a lifelong fructose-sorbitol-sucrose-free diet (FSSfd). A high index of suspicion and implementation of this diet are essential to avoid fatal complications. For HFI patients, the maximum tolerated daily fructose intake, the need for vitamins supplementation and the safety of drugs and vaccines containing fructose traces are still debated. For obesity prevention, a FSSfd could be beneficial for all infants before weaning.