Vitamin C and folate status in hereditary fructose intolerance

• DOI: 10.1038/s41430-022-01178-3
• Publication Date: 2022-07-19T13:02:58Z
• Authors: Ainara Cano; Carlos Alcalde; Amaya Belanger-Qu...; Elvira Cañedo-Vil...; Leticia Ceberio; Silvia Chumillas-...; Patricia Correcher; María Luz Couce; Dolores García-Ar...; Igor Gómez; Tomás Hernández; Elsa Izquierdo-Ga...; Dámaris Martínez ...; Montserrat Morales; Consuelo Pedrón-G...; Estrella Petrina ...; Luis Peña-Quintana; Paula Sánchez-Pintos; Juliana Serrano-N...; María Unceta Suarez; Isidro Vitoria Mi...; Javier de las Heras
• Keywords: Folate; Metabolic disorders; CHILDHOOD; Ascorbic Acid; Vitamins; Fructose; DIAGNOSIS; Fructose Intolerance; Article; 3206 Ciencias de la nutrición; 3. Good health; DEFICIENCY; Vitamin B 12; 03 medical and health sciences; Folic Acid; 0302 clinical medicine; Humans; Vitamin C; DIETARY-MANAGEMENT; 32 Ciencias médicas

AbstractBackgroundHereditary 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.MethodsVitamin C and folic acid dietary intake, supplementation and circulating levels were assessed in 32 HFI patients and 32 age- and sex-matched healthy controls.ResultsMost 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 healthy controls (30% vs. 3.1%;p = 0.036).ConclusionsOur 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.