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. 2022 Dec;76(12):1733-1739.
doi: 10.1038/s41430-022-01178-3. Epub 2022 Jul 19.

Vitamin C and folate status in hereditary fructose intolerance

Ainara Cano  1   2 , Carlos Alcalde  3 , Amaya Belanger-Quintana  4 , Elvira Cañedo-Villarroya  5 , Leticia Ceberio  1   6 , Silvia Chumillas-Calzada  7 , Patricia Correcher  8 , María Luz Couce  9 , Dolores García-Arenas  10 , Igor Gómez  11 , Tomás Hernández  12 , Elsa Izquierdo-García  13 , Dámaris Martínez Chicano  10 , Montserrat Morales  7 , Consuelo Pedrón-Giner  5 , Estrella Petrina Jáuregui  14 , Luis Peña-Quintana  15 , Paula Sánchez-Pintos  9 , Juliana Serrano-Nieto  16 , María Unceta Suarez  17 , Isidro Vitoria Miñana  8 , Javier de Las Heras  18   19   20
Affiliations

Vitamin C and folate status in hereditary fructose intolerance

Ainara Cano et al. Eur J Clin Nutr. 2022 Dec.

Erratum in

  • Correction: Vitamin C and folate status in hereditary fructose intolerance.
    Cano A, Alcalde C, Belanger-Quintana A, Cañedo-Villarroya E, Ceberio L, Chumillas-Calzada S, Correcher P, Couce ML, García-Arenas D, Gómez I, Hernández T, Izquierdo-García E, Chicano DM, Morales M, Pedrón-Giner C, Jáuregui EP, Peña-Quintana L, Sánchez-Pintos P, Serrano-Nieto J, Suarez MU, Miñana IV, de Las Heras J. Cano A, et al. Eur J Clin Nutr. 2023 Nov;77(11):1102-1103. doi: 10.1038/s41430-023-01334-3. Eur J Clin Nutr. 2023. PMID: 37673953 Free PMC article. No abstract available.

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.

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Conflict of interest statement

The authors declare no competing interests.

Figures

Fig. 1
Fig. 1. Vitamin C and folic acid supplementation in HFI patients (n = 32).
The percentage of subjects that received vitamin C and folic acid is represented in dark and light grey columns, respectively.
Fig. 2
Fig. 2. Vitamin C deficiency in HFI patients and healthy controls.
Columns represent the percentage of vitamin C deficiency in HFI patients altogether (n = 32), HFI patients without vitamin C supplementation (n = 10), and healthy controls (n = 32).
Fig. 3
Fig. 3. Correlation between the amount of vitamin C supplementation and plasma levels in HFI patients.
The horizontal dashed line indicates the lower limit of normal for vitamin C plasma concentrations (23 µmol/L).
Fig. 4
Fig. 4. Correlation between the amount of folic acid supplementation and serum folate levels in HFI patients.
The horizontal dashed line indicates the lower limit of normal for folate serum concentrations (6.8 nmol/L).
See this image and copyright information in PMC

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