Cano, AinaraAlcalde, CarlosBelanger-Quintana, AmayaCañedo-Villarroya, ElviraCeberio, LeticiaChumillas-Calzada, SilviaCorrecher, PatriciaCouce, María LuzGarcia-Arenas, DoloresGomez, IgorHernandez, TomasIzquierdo-Garcia, ElsaMartinez-Chicano, DamarisMorales, MontserratPedron-Giner, ConsueloJauregui, Estrella PetrinaPeña-Quintana, LuisSanchez-Pintos, PaulaSerrano-Nieto, JulianaUnceta-Suarez, MariaMiñana, Isidro Vitoriade-Las-Heras, Javier2023-05-032023-05-032022-07-19Cano A, Alcalde C, Belanger-Quintana A, Cañedo-Villarroya E, Ceberio L, Chumillas-Calzada S, et al. Vitamin C and folate status in hereditary fructose intolerance. Eur J Clin Nutr. 2022 Dec;76(12):1733-1739. doi: 10.1038/s41430-022-01178-3. Epub 2022 Jul 19. Erratum in: Eur J Clin Nutr. 2023 Nov;77(11):1102-1103http://hdl.handle.net/10668/19533Hereditary 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.enAttribution 4.0 Internationalhttp://creativecommons.org/licenses/by/4.0/Fructose IntoleranceFructoseDietary SupplementsVitamin AFolic Acid DeficiencyHumansFructose IntoleranceFolic AcidAscorbic AcidVitaminsFructoseVitamin B 12Vitamin C and folate status in hereditary fructose intolerance.research article35854131open accessÁcido ascórbicoÁcido fólicoIngestión de alimentosVitaminasDeficiencia de ácido ascórbicoMetabolismo10.1038/s41430-022-01178-31476-5640PMC9708598https://www.nature.com/articles/s41430-022-01178-3.pdfhttps://www.ncbi.nlm.nih.gov/pmc/articles/PMC9708598/pdf