Background Hereditary fructose intolerance (HFI) is an autosomal recessive disorder caused by aldolase B deficiency. Treatment consists of elimination of fructose, sucrose and sorbitol from the diet. There are a lot of medicines with sweeteners but there is disagreement about their tolerance.

Purpose Our purpose was to provide information to health professional (pharmacist and doctors) and patients about excipients for HIF patients. We collaborated with the Spanish HIF Association.

Material and methods We reviewed Spanish and European legislation about excipients and dietary recommendations for HIF patients.

Results We checked European Guidelines (2003) and Spanish legislation (2008).

Contraindicated: fructose, sucrose, invert sugar and sorbitol are a significant source of fructose and the label must contain an alert. Patients with rare hereditary problems of fructose intolerance should not take this medicine. High fructose corn syrup, sucromalt or tagatose (metabolised by aldolase B) are not used in the pharmaceutical industry but they should be avoided.

Allowed. There is agreement about glucose, dextrinomaltose and glucose syrup, synthetic sweeteners (acesulfame, aspartame or saccharin), sucralose, erythritol and xylitol. In these cases, there is no need for an alert on the label for HIF patients.

Caution. Legislation does not recommend maltitol, lactitol, isomaltitol (polyols: sorbitol disaccharides) but the dietary recommendation is not unanimous. Because of the low affinity of the disaccharidases, sorbitol release in the intestine is low and variable. Legislation does not have an alert about mannitol (unknown hepatic metabolism), inulin (fructose polysaccharide), polydextrose (10% of sorbitol) or polysorbates. Also, they could release some fructose or sorbitol. In this group it is necessary to evaluate benefit and risk according to the characteristics of the patient and excipient (purity, metabolism and quantity).

Conclusion Excipient and sweetener recommendations (especially polyols) do not match between legislations (contraindicated) and references. Furthermore, excipient legislation does not warn about mannitol, inulin, polydextrose or polysorbates.

Because there are no unanimous recommendations, we have developed materials for health professionals in collaboration with the HIF Spanish Association.

References and/or Acknowledgements

1. Excipients in the label and package leaflet of medicinal products. EMA. 2003

2. Información sobre excipientes: etiquetado, prospecto, ficha técnica. Circular 2/2008

3. Edulcorantes en pacientes con IHF. Acta Pediatr Esp. 2014

4. Errores congénitos del metabolismo de la fructosa. Diagnóstico y tratamiento de enfermedades metabólicas hereditarias. Ergon. 2010

References and/or AcknowledgementsNo conflict of interest.