RT Journal Article
T1 Vitamin C and folate status in hereditary fructose intolerance.
A1 Cano, Ainara
A1 Alcalde, Carlos
A1 Belanger-Quintana, Amaya
A1 Cañedo-Villarroya, Elvira
A1 Ceberio, Leticia
A1 Chumillas-Calzada, Silvia
A1 Correcher, Patricia
A1 Couce, María Luz
A1 García-Arenas, Dolores
A1 Gómez, Igor
A1 Hernández, Tomás
A1 Izquierdo-García, Elsa
A1 Chicano, Dámaris Martínez
A1 Morales, Montserrat
A1 Pedrón-Giner, Consuelo
A1 Jáuregui, Estrella Petrina
A1 Peña-Quintana, Luis
A1 Sánchez-Pintos, Paula
A1 Serrano-Nieto, Juliana
A1 Suarez, María Unceta
A1 Miñana, Isidro Vitoria
A1 de Las Heras, Javier
AB 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. 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. 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  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.
YR 2022
FD 2022-07-19
LK http://hdl.handle.net/10668/19533
UL http://hdl.handle.net/10668/19533
LA en
DS RISalud
RD Apr 6, 2025