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dc.contributor.authorCano, Ainara-
dc.contributor.authorAlcalde, Carlos-
dc.contributor.authorBelanger-Quintana, Amaya; Canedo-Villarroya, Elvira-
dc.contributor.authorCeberio, Leticia-
dc.contributor.authorChumillas-Calzada, Silvia-
dc.contributor.authorCorrecher, Patricia-
dc.contributor.authorCouce, Maria Luz-
dc.contributor.authorGarcia-Arenas, Dolores-
dc.contributor.authorGomez, Igor-
dc.contributor.authorHernandez, Tomas-
dc.contributor.authorIzquierdo-Garcia, Elsa; Chicano, Damaris Martinez-
dc.contributor.authorMorales, Montserrat-
dc.contributor.authorPedron-Giner, Consuelo-
dc.contributor.authorJauregui, Estrella Petrina-
dc.contributor.authorPena-Quintana, Luis; Sanchez-Pintos, Paula-
dc.contributor.authorSerrano-Nieto, Juliana-
dc.contributor.authorSuarez, Maria Unceta-
dc.contributor.authorMinana, Isidro Vitoria-
dc.contributor.authorde las Heras, Javier-
dc.date.accessioned2023-10-04T10:45:11Z-
dc.date.available2023-10-04T10:45:11Z-
dc.date.issued2022-
dc.identifierWOS:000827360200001-
dc.identifier.citationEUROPEAN JOURNAL OF CLINICAL NUTRITION, 2022, 76, 1733-1739-
dc.identifier.issn0954-3007-
dc.identifier.urihttp://dspace.azti.es/handle/24689/1649-
dc.description.abstractBackground 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.-
dc.language.isoEnglish-
dc.publisherSPRINGERNATURE-
dc.subjectDIETARY-MANAGEMENT-
dc.subjectDEFICIENCY-
dc.subjectCHILDHOOD-
dc.subjectDIAGNOSIS-
dc.titleVitamin C and folate status in hereditary fructose intolerance-
dc.typeArticle-
dc.identifier.journalEUROPEAN JOURNAL OF CLINICAL NUTRITION-
dc.format.page1733-1739-
dc.format.volume76-
dc.contributor.funderBasque Government, Health Department [2018111095]-
dc.contributor.funderFEDER, the Spanish Federation for Rare Diseases [FI18053]-
dc.identifier.e-issn1476-5640-
dc.identifier.doi10.1038/s41430-022-01178-3-
Appears in Publication types:Artículos científicos



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