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http://hdl.handle.net/10553/117748
Title: | Vitamin C and folate status in hereditary fructose intolerance | Authors: | Cano, Ainara Alcalde, Carlos Belanger-Quintana, Amaya Cañedo-Villarroya, Elvira Ceberio, Leticia Chumillas-Calzada, Silvia Correcher, Patricia Couce, María Luz García-Arenas, Dolores Gómez, Igor Hernández, Tomás Izquierdo-García, Elsa Martínez Chicano, Dámaris Morales, Montserrat Pedrón-Giner, Consuelo Petrina Jáuregui, Estrella Peña Quintana, Luis Sánchez-Pintos, Paula Serrano-Nieto, Juliana Unceta Suarez, María Vitoria Miñana, Isidro de las Heras, Javier |
UNESCO Clasification: | 32 Ciencias médicas 3206 Ciencias de la nutrición |
Keywords: | Metabolic disorders Vitamin C Folate Fructose |
Issue Date: | 2022 | Journal: | European Journal of Clinical Nutrition | Abstract: | Background 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. Methods 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. Results 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 < 0.001). Conclusions 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. | URI: | http://hdl.handle.net/10553/117748 | ISSN: | 1476-5640 | DOI: | 10.1038/s41430-022-01178-3 | Source: | European Journal of Clinical Nutrition [1476-5640], (Julio 2022) |
Appears in Collections: | Artículos |
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