Edulcorantes en pacientes con intolerancia hereditaria a la fructosa

  1. E. Izquierdo García
  2. José Manuel Moreno Villares
  3. Miguel León Sanz
Revue:
Acta pediátrica española

ISSN: 0001-6640

Année de publication: 2014

Volumen: 72

Número: 1

Pages: 15-23

Type: Article

D'autres publications dans: Acta pediátrica española

Résumé

Hereditary fructose intolerance (HFI) is an autosomal recessive disorder caused by aldolase B (fructose 1,6-bisphosphate aldolase) deficiency, this enzyme is involved in the metabolism of fructose. Currently, treatment consists of elimination of all fructose sources from the diet. Generally, glucose can be used as a sweetener, but alternatives are sought. The aim is to review the sweeteners characteristics and establish recommendations for consumption in these patients. Due to the structure of some sweeteners, they do not constitute a source of fructose and they are tolerated by patients with IHF such as glucose, dextrinomaltose and glucose polymers, artificial sweeteners (acesulfame, alitame, aspartame, cyclamate, neotame and saccharin), peptides as thaumatin, glucoflavonoids as nehosperidin-dihydrochalcone or diterpene glycosides as steviol glycosides. Others they are not metabolized in the human body, such as erythritol or they are metabolized by different routes, such as xylitol. Inulin, fructanes or sucralose are not metabolized by the human body but they have small amounts of fructose or sucrose, so it should be taken into account, as well as those who are absorbed in small proportion (depending on purity, patient tolerance and amount). The remainder should be avoided because they can provide a considerably amount of fructose due to their structure, absorption and metabolism.