Itemaren erregistro erraza erakusten du
Vitamin C and folate status in hereditary fructose intolerance
dc.contributor.author | Cano San José, Ainara | |
dc.contributor.author | Alcalde, Carlos | |
dc.contributor.author | Belanger Quintana, Amaya | |
dc.contributor.author | Cañedo Villarroya, Elvira | |
dc.contributor.author | Ceberio, Leticia | |
dc.contributor.author | Chumillas Calzada, Silvia | |
dc.contributor.author | Correcher, Patricia | |
dc.contributor.author | Couce, María Luz | |
dc.contributor.author | García Arenas, Dolores | |
dc.contributor.author | Gómez, Igor | |
dc.contributor.author | Hernández, Tomás | |
dc.contributor.author | Izquierdo García, Elsa | |
dc.contributor.author | Martínez Chicano, Dámaris | |
dc.contributor.author | Morales, Montserrat | |
dc.contributor.author | Pedrón Giner, Consuelo | |
dc.contributor.author | Petrina Jáuregui, Estrella | |
dc.contributor.author | Peña Quintana, Luis | |
dc.contributor.author | Sánchez Pintos, Paula | |
dc.contributor.author | Serrano Nieto, Juliana | |
dc.contributor.author | Unceta Suárez, María | |
dc.contributor.author | Vitoria Miñana, Isidro | |
dc.contributor.author | De las Heras Montero, Javier Adolfo | |
dc.date.accessioned | 2023-02-14T16:40:47Z | |
dc.date.available | 2023-02-14T16:40:47Z | |
dc.date.issued | 2022-12 | |
dc.identifier.citation | European Journal of Clinical Nutrition 76(12) : 1733-1739 (2022) | es_ES |
dc.identifier.issn | 0954-3007 | |
dc.identifier.issn | 1476-5640 | |
dc.identifier.uri | http://hdl.handle.net/10810/59838 | |
dc.description.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. | es_ES |
dc.description.sponsorship | This work was supported by Exp. No. 2018111095, Basque Government, Health Department; FEDER, the Spanish Federation for Rare Diseases (FI18053); and Danone-Nutricia-Metabolics, which was not involved in the study hypothesis/design, execution, analysis, or interpretation. | es_ES |
dc.language.iso | eng | es_ES |
dc.publisher | SpringerNature | es_ES |
dc.rights | info:eu-repo/semantics/openAccess | es_ES |
dc.rights.uri | http://creativecommons.org/licenses/by/3.0/es/ | * |
dc.title | Vitamin C and folate status in hereditary fructose intolerance | es_ES |
dc.type | info:eu-repo/semantics/article | es_ES |
dc.rights.holder | © The Author(s) 2022. Open Access This article is licensed under a Creative Commons Attribution 4.0 International License, which permits use, sharing, adaptation, distribution and reproduction in any medium or format, as long as you give appropriate credit to the original author(s) and the source, provide a link to the Creative Commons license, and indicate if changes were made. The images or other third party material in this article are included in the article’s Creative Commons license, unless indicated otherwise in a credit line to the material. If material is not included in the article’s Creative Commons license and your intended use is not permitted by statutory regulation or exceeds the permitted use, you will need to obtain permission directly from the copyright holder. To view a copy of this license, visit http:// creativecommons.org/licenses/by/4.0/. | es_ES |
dc.rights.holder | Atribución 3.0 España | * |
dc.relation.publisherversion | https://www.nature.com/articles/s41430-022-01178-3 | es_ES |
dc.identifier.doi | 10.1038/s41430-022-01178-3 | |
dc.departamentoes | Pediatría | es_ES |
dc.departamentoeu | Pediatria | es_ES |
Item honetako fitxategiak
Item hau honako bilduma honetan/hauetan agertzen da
Bestelakorik adierazi ezean, itemaren baimena horrela deskribatzen da:© The Author(s) 2022. Open Access This article is licensed under a Creative Commons
Attribution 4.0 International License, which permits use, sharing,
adaptation, distribution and reproduction in any medium or format, as long as you give
appropriate credit to the original author(s) and the source, provide a link to the Creative
Commons license, and indicate if changes were made. The images or other third party
material in this article are included in the article’s Creative Commons license, unless
indicated otherwise in a credit line to the material. If material is not included in the
article’s Creative Commons license and your intended use is not permitted by statutory
regulation or exceeds the permitted use, you will need to obtain permission directly
from the copyright holder. To view a copy of this license, visit http://
creativecommons.org/licenses/by/4.0/.