excipients in patients with hereditary fructose intolerance
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EXCIPIENTS IN PATIENTS WITH HEREDITARY FRUCTOSE INTOLERANCE E. Izquierdo 1 , I. Cañamares 1 , A. Such 1 , J. Saez 1 , N. Barrueco 1 , C. Esteban 1 , I. Escobar 1 . 1 Infanta Leonor Hospital, Pharmacy Department, Madrid, Spain. BACKGROUND: Hereditary Fructose Intolerance (HFI) is an autosomal recessive disorder caused by aldolase B deficiency. Treatment consists of eliminaMon of fructose, sucrose and sorbitol from the diet. There are a lot of medicines with sweetener but there is disagreement with their tolerance. ALLOWED : there is not alert on the label for HIF paMents: Dextrinomaltose and glucose syrup , syntheMc sweeteners (acesulfame, aspartame or saccharin), sucralose, erythritol and xylitol. CAUTION : Evaluate benefit and risk according paMent and excipient characterisMcs (purity, metabolism and quanMty) 1.LegislaMon does not recommend: MALTITOL , LACTITOL , ISOMALTITOL (polyols: sorbitol disaccharides) but the dietary recommendaMon is not unanimous. Because of low affinity of the disaccharidases the sorbitol releases in the intesMne is low and variable. 2.LegislaMon does not alert about MANNITOL (unknown hepaMc metabolism), INULIN (fructose polysaccharide), POLYDEXTROSE (10% of sorbitol) and POLYSORBATES. Also, they could release a few fructose or sorbitol. CONTRAINDICATED : High fructose corn syrup, sucromalt or tagatose (metabolized by aldolase B) are not used in the pharmaceuMcal industry but they should avoid. Fructose, sucrose, invert sugar and sorbitol are a significant source of fructose and in label must appear an alert: “Pa%ents with rare hereditary problems of fructose intolerance should not take this medicine” References and/or Acknowledgements: Errores congénitos del metabolismo de la fructosa. DiagnósMco y tratamiento de enfermedades metabólicas hereditarias. Ergon. 2010 Excipients in the label and package leaflet of medicinal products. EMA. 2003. Información sobre excipientes: eMquetado, prospecto, ficha técnica. Circular 2/2008. Edulcorantes en pacientes con IHF. Acta Pediatr Esp. 2014 CONCLUSION: Excipients and sweeteners recommendaMons (especially polyols) do not match between legislaMon (contraindicated) and references. Furthermore, excipients legislaMon does not warn about mannitol, inulin, polydextrose and polysorbates. Because of there are not unanimous recommendaMons we develop informaMon material for health professionals in collaboraMon with HIF Spanish associaMon. PURPOSE: Our purpose is to provide informaMon to health professional (pharmacist and doctors) and paMents about excipients for HIF paMents. The work was conducted in close collaboraMon with the Spanish associaMon of HFI paMents . MATERIAL AND METHODS : We reviewed Spanish and European legislaMon about excipients and dietary recommendaMons for HIF paMents . RESULTS: We reviewed the European Guidelines (Excipients in the label and package leaflet of medicinal products. EMA. 2003) and Spanish legislaMon (Circular 2/2008) and classify the excipients listed in allowed, contraindicated or in which special cauMon is advised: [email protected] hcps://asociacionihf.wordpress.com/ DI073
Transcript of excipients in patients with hereditary fructose intolerance
EXCIPIENTS IN PATIENTS WITH HEREDITARY FRUCTOSE INTOLERANCE
E. Izquierdo1, I. Cañamares1, A. Such1, J. Saez1, N. Barrueco1, C. Esteban1, I. Escobar1. 1Infanta Leonor Hospital, Pharmacy Department, Madrid, Spain.
BACKGROUND: Hereditary Fructose Intolerance (HFI) is an autosomal recessive disorder caused by aldolase B deficiency. Treatment consists of eliminaMon of fructose, sucrose and sorbitol from the diet. There are a lot of medicines with sweetener but there is disagreement with their tolerance.
ALLOWED: there is not alert on the label for HIF paMents:
Dextrinomaltose and glucose syrup, syntheMc sweeteners (acesulfame, aspartame or saccharin), sucralose, erythritol and xylitol.
CAUTION: Evaluate benefit and risk according paMent and excipient
characterisMcs (purity, metabolism and quanMty)
1. LegislaMon does not recommend: MALTITOL, LACTITOL, ISOMALTITOL (polyols: sorbitol disaccharides) but the dietary recommendaMon is not unanimous. Because of low affinity of the disaccharidases the sorbitol releases in the intesMne is low and variable. 2. LegislaMon does not alert about MANNITOL (unknown hepaMc metabolism), INULIN (fructose polysaccharide), POLYDEXTROSE (10% of sorbitol) and POLYSORBATES. Also, they could release a few fructose or sorbitol.
CONTRAINDICATED:
High fructose corn syrup, sucromalt or tagatose (metabolized by aldolase B) are not used in the pharmaceuMcal industry but they should avoid.
Fructose, sucrose, invert sugar and sorbitol are a significant source of fructose and in label must appear an alert: “Pa%ents with rare hereditary problems of fructose intolerance should not take this medicine”
References and/or Acknowledgements: Errores congénitos del metabolismo de la fructosa. DiagnósMco y tratamiento de enfermedades metabólicas hereditarias. Ergon. 2010 Excipients in the label and package leaflet of medicinal products. EMA. 2003. Información sobre excipientes: eMquetado, prospecto, ficha técnica. Circular 2/2008. Edulcorantes en pacientes con IHF. Acta Pediatr Esp. 2014
CONCLUSION: Excipients and sweeteners recommendaMons (especially polyols) do not match between legislaMon (contraindicated) and references. Furthermore, excipients legislaMon does not warn about mannitol, inulin, polydextrose and polysorbates. Because of there are not unanimous recommendaMons we develop informaMon material for health professionals in collaboraMon with HIF Spanish associaMon.
PURPOSE: Our purpose is to provide informaMon to health professional (pharmacist and doctors) and paMents about excipients for HIF paMents. The work was conducted in close collaboraMon with the Spanish associaMon of HFI paMents. MATERIAL AND METHODS: We reviewed Spanish and European legislaMon about excipients and dietary recommendaMons for HIF paMents. RESULTS: We reviewed the European Guidelines (Excipients in the label and package leaflet of medicinal products. EMA. 2003) and Spanish legislaMon (Circular 2/2008) and classify the excipients listed in allowed, contraindicated or in which special cauMon is advised:
[email protected] hcps://asociacionihf.wordpress.com/
DI-‐073
