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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
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.
ISSN: 1476-5640
DOI: 10.1038/s41430-022-01178-3
Source: European Journal of Clinical Nutrition [1476-5640], (Julio 2022)
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