title: High prevalence of vitamin D deficiency in patients with xeroderma pigmetosum-A under strict sun protection.
abstract: OBJECTIVE
  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.
  
  
  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.
authors: Department of Health and Nutrition, Osaka Shoin Women's University, Higashiosaka-shi, Osaka, Japan.
authors: A
authors: A
authors: Kuwabara
authors: Department of Hygienic Sciences, Kobe Pharmaceutical University, Kobe, Japan.
authors: N
authors: N
authors: Tsugawa
authors: Department of Food and Nutrition, Kyoto Women's University, Kyoto, Japan.
authors: K
authors: K
authors: Tanaka
authors: Department of Health and Nutrition, Osaka Shoin Women's University, Higashiosaka-shi, Osaka, Japan.
authors: Y
authors: Y
authors: Uejima
authors: Department of Health and Nutrition, Osaka Shoin Women's University, Higashiosaka-shi, Osaka, Japan.
authors: J
authors: J
authors: Ogawa
authors: Department of Health and Nutrition, Osaka Shoin Women's University, Higashiosaka-shi, Osaka, Japan.
authors: N
authors: N
authors: Otao
authors: Department of Health and Nutrition, Osaka Shoin Women's University, Higashiosaka-shi, Osaka, Japan.
authors: N
authors: N
authors: Yamada
authors: Department of Dermatology, Clinical Molecular Medicine, Graduate School of Medicine, Kobe University, Kobe, Japan.
authors: T
authors: 0000000317666661
authors: T
authors: Masaki
authors: Department of Dermatology, Clinical Molecular Medicine, Graduate School of Medicine, Kobe University, Kobe, Japan.
authors: C
authors: C
authors: Nishigori
authors: Department of Dermatology, Osaka Medical College, Osaka, Japan.
authors: S
authors: S
authors: Moriwaki
authors: Department of Hygienic Sciences, Kobe Pharmaceutical University, Kobe, Japan.
authors: T
authors: T
authors: Okano
mesh_terms: D015652:25-Hydroxyvitamin D 2 / Q000097:blood; D000293:Adolescent; D000328:Adult; D002112:Calcifediol / Q000097:blood; D002648:Child; D003131:Combined Modality Therapy / Q000009:adverse effects; D003430:Cross-Sectional Studies; D005260:Female; D006785:Hospitals, University; D006801:Humans; D007564:Japan / Q000453:epidemiology; D008019:Life Style; D008297:Male; D009752:Nutritional Status; D010044:Outpatient Clinics, Hospital; D010281:Parathyroid Hormone / Q000097:blood; D010349:Patient Compliance; D015995:Prevalence; D012306:Risk; D012878:Skin Neoplasms / Q000209:etiology / Q000517:prevention & control*; D013473:Sunscreening Agents / Q000009:adverse effects / Q000627:therapeutic use*; D014808:Vitamin D Deficiency / Q000453:epidemiology / Q000209:etiology*; D014983:Xeroderma Pigmentosum / Q000097:blood / Q000503:physiopathology / Q000628:therapy*; D055815:Young Adult
chemical_list: C470125:PTH protein, human; D010281:Parathyroid Hormone; D013473:Sunscreening Agents; D015652:25-Hydroxyvitamin D 2; D002112:Calcifediol