ArticleEuropean journal of clinical nutrition2022
Vitamin C and folate status in hereditary fructose intolerance.
Article in European journal of clinical nutrition, 2022. The graph could read no effect estimate from its abstract, so it casts no vote on the map. An erratum has been issued. Cited by 8 papers.
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Who cites it
8 citing papers in PubMed, 9 citations in OpenAlex.
- Current Dietary Practices in Hereditary Fructose Intolerance: Results from a National Survey Across 15 Italian Metabolic Centres.Nutrients · 2026Article
- Genotypic and Phenotypic Characteristics of Turkish Patients with Hereditary Fructose Intolerance.Molecular syndromology · 2026Article
- Dietary Patterns in a Nationwide Cohort of Patients with Hereditary Fructose Intolerance.Nutrients · 2026Article
- A Case Study of a Rare Disease (Fructosemia) Diagnosed in a Patient with Abdominal Pain.Journal of clinical medicine · 2024Article
- Descriptive Analysis of Carrier and Affected Hereditary Fructose Intolerance in Women during Pregnancy.Healthcare (Basel, Switzerland) · 2024Article
- Clinical Practice Guidelines for the Diagnosis and Management of Hereditary Fructose Intolerance.Diseases (Basel, Switzerland) · 2024Article
- Micronutrient Deficiency in Inherited Metabolic Disorders Requiring Diet Regimen: A Brief Critical Review.International journal of molecular sciences · 2023Review
- Correction: Vitamin C and folate status in hereditary fructose intolerance.European journal of clinical nutrition · 2023Article
Corrections and comments
- Erratum issued
Authors and funding
22 authors at 15 institutions in 1 country.
Funding
No grant is acknowledged in the PubMed record.
Abstract
backgroundHereditary 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 supplemented HFI patients (30% vs. 9.1%; p = 0.01) and to healthy controls (30% vs. 3.1%; p < 0.001).
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.
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