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Vitamins and plant ingredients

High prevalence of vitamin D deficiency in patients with xeroderma pigmetosum-A under strict sun protection

European Journal of Clinical Nutrition volume 69, pages 693–696 (2015)Cite this article

Subjects

Abstract

Background/objectives:

Xeroderma pigmentosum (XP) is a rare autosomal recessive disease characterized by defective repair of ultraviolet (UV) irradiation-induced DNA damage and high risk of skin cancer. Thus, these patients require strict photoprotection. Considering the importance of UV-mediated cutaneous vitamin D production, such rigorous photoprotection would cause vitamin D deficiency. Then, we have studied the vitamin D status in patients with XP-A, a group requiring the most strict photoprotection.

Subjects/methods:

Twenty-one patients with XP-A (aged 6–25) were evaluated for their vitamin D intake, serum levels of 25-hydroxy-vitamin D (25OHD) and parathyroid hormone (PTH). Vitamin D intake was assessed by a 2-day food weighing method.

Results:

Median dietary intake of vitamin D was 4.1 μg/day, and the median concentrations of serum 25OHD and PTH were 7.7 and 49.9 pg/ml, respectively. In 76% of the patients, serum 25OHD level was lower than 10 ng/ml, indicating vitamin D deficiency. Vitamin D intake and serum 25OHD level were significantly lower in patients under enteral nutrition (EN) than those with oral intake (OI). Multivariate analyses revealed that EN was a significant predictor of decreased serum 25OHD level (β coefficient=−0.59, P=0.03).

Conclusions:

Vitamin D deficiency is highly prevalent in XP-A patients, and supplementation should be considered to avoid unfavorable skeletal consequences in these patients. In addition, determination of dietary vitamin D requirement has been a difficult work issue in the decision of dietary reference intakes (DRIs) because of its cutaneous production. Data from XP patients would yield useful information for the determination of DRIs for vitamin D.

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Acknowledgements

This work was supported by JSPS KAKENHI Grant Number 25750061 and 25350157.

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Authors and Affiliations

  1. Department of Health and Nutrition, Osaka Shoin Women’s University, Higashiosaka-shi, Osaka, Japan

    A Kuwabara, Y Uejima, J Ogawa, N Otao & N Yamada

  2. Department of Hygienic Sciences, Kobe Pharmaceutical University, Kobe, Japan

    N Tsugawa & T Okano

  3. Department of Food and Nutrition, Kyoto Women’s University, Kyoto, Japan

    K Tanaka

  4. Department of Dermatology, Clinical Molecular Medicine, Graduate School of Medicine, Kobe University, Kobe, Japan

    T Masaki & C Nishigori

  5. Department of Dermatology, Osaka Medical College, Osaka, Japan

    S Moriwaki

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  1. A Kuwabara
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  2. N Tsugawa
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  3. K Tanaka
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  4. Y Uejima
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  10. S Moriwaki
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Correspondence to A Kuwabara.

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Kuwabara, A., Tsugawa, N., Tanaka, K. et al. High prevalence of vitamin D deficiency in patients with xeroderma pigmetosum-A under strict sun protection. Eur J Clin Nutr 69, 693–696 (2015). https://doi.org/10.1038/ejcn.2015.1

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