Safety of vaccines administration in hereditary fructose intolerance
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LETTER TO THE EDITOR
Safety of vaccines administration in hereditary fructose intolerance Arianna Maiorana1* , Antonella Sabia2, Tiziana Corsetti2 and Carlo Dionisi‑Vici1
Abstract Patients with hereditary fructose intolerance need to follow a life-long fructose dietary and drug restriction to prevent symptoms of intoxication. Concerns about vaccines administration have been manifested overtime, for the risk of a life-threatening acute intoxication. For this reason, at Ospedale Pediatrico Bambino Gesù we performed a deepen research from open sources, datasheets and Pharmaceutical Companies informations from the most common Italian and European vaccines, which are carried out in infancy and childhood. As a safe threshold of 2.4 mg/kg/dose was recently established for oral and parenteral (other than i.v.) route, the manuscript clarifies the safe administration of majority of vaccines in patients with hereditary fructose intolerance. Keywords: Hereditary fructose intolerance, Fructose, Sorbitol, Sucrose, Vaccines Hereditary fructose intolerance (HFI, OMIM 229,600) is an autosomal-recessive disorder with a prevalence of 1:23,000, caused by deficiency of aldolase B, the main enzyme responsible for hepatic metabolism of fructose. The signs and symptoms upon introduction of fructose-containing foods include abdominal pain, nausea, recurrent vomiting, hypoglycemia and failure to thrive. Besides hypoglycemia, metabolic disturbances such as lactic acidemia, hypophosphatemia, hyperuricemia and hypermagnesemia are observed in case of acute fructose intoxication. Parenteral intravenously administration of fructose, sorbitol, or sucrose may cause death for severe hypoglycemia, acute hepato-renal failure associated with bleeding and jaundice and must be rigorously avoided [1]. For this reason, the diagnostic challenge with i.v. fructose 0.25 g/kg was abandoned and substituted by the molecular analysis. Also the diagnostic oral load of fructose 1 g/ kg is no longer recommended as deemed life threatening as well.
*Correspondence: [email protected] 1 Division of Metabolism, Ospedale Pediatrico Bambino Gesù, IRCSS, piazza S. Onofrio 4, 00165 Rome, Italy Full list of author information is available at the end of the article
Many individuals with HFI exhibit a self-imposed aversion to sweet foods, sufficiently to prevent an acute intoxication. However, prolonged fructose intake leads to poor feeding, vomiting, failure to thrive, hepatomegaly, liver and renal tubular dysfunction that might lead to irreversible liver and kidney damage [1, 2]. Upon dietary restriction of fructose, symptoms resolve and normal growth and development are achieved. Therefore, individuals with HFI need to be treated life-long with a fructose-restricted diet. They should be aware of the presence of fructose in certain medicinal formulations and such medications should be avoided. Particularly, concerns about oral or parenteral vaccines administration have been manifested overtime, because they contain amounts of sorbitol or sucrose or o
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