%0 Journal Article
%A Cano, Ainara
%A Alcalde, Carlos
%A Belanger-Quintana, Amaya
%A Cañedo-Villarroya, Elvira
%A Ceberio, Leticia
%A Chumillas-Calzada, Silvia
%A Correcher, Patricia
%A Couce, María Luz
%A García-Arenas, Dolores
%A Gómez, Igor
%A Hernández, Tomás
%A Izquierdo-García, Elsa
%A Chicano, Dámaris Martínez
%A Morales, Montserrat
%A Pedrón-Giner, Consuelo
%A Jáuregui, Estrella Petrina
%A Peña-Quintana, Luis
%A Sánchez-Pintos, Paula
%A Serrano-Nieto, Juliana
%A Suarez, María Unceta
%A Miñana, Isidro Vitoria
%A de Las Heras, Javier
%T Vitamin C and folate status in hereditary fructose intolerance.
%D 2022
%U http://hdl.handle.net/10668/19533
%X Hereditary fructose intolerance (HFI) is a rare inborn error of fructose metabolism caused by the deficiency of aldolase B. Since treatment consists of a fructose-, sucrose- and sorbitol-restrictive diet for life, patients are at risk of presenting vitamin deficiencies. Although there is no published data on the status of these vitamins in HFI patients, supplementation with vitamin C and folic acid is common. Therefore, the aim of this study was to assess vitamin C and folate status and supplementation practices in a nationwide cohort of HFI patients. Vitamin C and folic acid dietary intake, supplementation and circulating levels were assessed in 32 HFI patients and 32 age- and sex-matched healthy controls. Most of the HFI participants presented vitamin C (96.7%) and folate (90%) dietary intake below the recommended population reference intake. Up to 69% received vitamin C and 50% folic acid supplementation. Among HFI patients, 15.6% presented vitamin C and 3.1% folate deficiency. The amount of vitamin C supplementation and plasma levels correlated positively (R = 0.443; p = 0.011). Interestingly, a higher percentage of non-supplemented HFI patients were vitamin C deficient when compared to supplemented HFI patients (30% vs. 9.1%; p = 0.01) and to healthy controls (30% vs. 3.1%; p  Our results provide evidence for the first time supporting vitamin C supplementation in HFI. There is great heterogeneity in vitamin supplementation practices and, despite follow-up at specialised centres, vitamin C deficiency is common. Further research is warranted to establish optimal doses of vitamin C and the need for folic acid supplementation in HFI.
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