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Title: Vitamin C and folate status in hereditary fructose intolerance
Authors: Cano, Ainara; Alcalde, Carlos; Belanger-Quintana, Amaya; Canedo-Villarroya, Elvira; Ceberio, Leticia; Chumillas-Calzada, Silvia; Correcher, Patricia; Couce, Maria Luz; Garcia-Arenas, Dolores; Gomez, Igor; Hernandez, Tomas; Izquierdo-Garcia, Elsa; Chicano, Damaris Martinez; Morales, Montserrat; Pedron-Giner, Consuelo; Jauregui, Estrella Petrina; Pena-Quintana, Luis; Sanchez-Pintos, Paula; Serrano-Nieto, Juliana; Suarez, Maria Unceta; Minana, Isidro Vitoria; de las Heras, Javier
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.
Issue Date: 2022
Type: Article
DOI: 10.1038/s41430-022-01178-3
ISSN: 0954-3007
E-ISSN: 1476-5640
Funder: Basque Government, Health Department [2018111095]
FEDER, the Spanish Federation for Rare Diseases [FI18053]
Appears in Publication types:Artículos científicos

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