title: Immune dysfunction in hypophosphatemic vitamin D-resistant rickets: immunoregulatory reaction of 1 alpha(OH) vitamin D3.
abstract: We investigated immunologic function in six cases with hypophosphatemic vitamin D-resistant rickets (VDRR) before and after treatment with 1 alpha-hydroxycholecalciferol (1 alpha(OH) vitamin D3). All cases suffered frequent episodes of infection, which tended to be more severe in the older patients. OKT9-, OKT10-, and OKM1-positive cells and adenosine deaminase (ADA) were significantly increased, whereas numbers and activity of natural killer (NK) cells were lower than normal before treatment. After administration of 1 alpha(OH) vitamin D3, however, the susceptibility to infection apparently decreased, and NK cell number and activity increased in all patients. ADA was also significantly decreased and remained in the normal range after treatment. These results suggest that vitamin D plays a role in the impaired immunoregulatory functions of NK cells in VDRR. Furthermore, ADA may be one parameter reflecting this immunologic impairment.
authors.affiliation: Third Department of Internal Medicine, Faculty of Medicine, Kagoshima University, Japan.
authors.forename: I
authors.initials: I
authors.lastname: Kitajima
authors.forename: I
authors.initials: I
authors.lastname: Maruyama
authors.forename: H
authors.initials: H
authors.lastname: Matsubara
authors.forename: M
authors.initials: M
authors.lastname: Osame
authors.forename: A
authors.initials: A
authors.lastname: Igata
mesh_terms: D000243:Adenosine Deaminase / Q000032:analysis; D000293:Adolescent; D000328:Adult; D000368:Aged; D002648:Child; D004198:Disease Susceptibility; D005260:Female; D006801:Humans; D006887:Hydroxycholecalciferols / Q000494:pharmacology / Q000627:therapeutic use*; D007015:Hypophosphatemia, Familial / Q000150:complications / Q000188:drug therapy / Q000235:genetics / Q000276:immunology*; D007111:Immunity, Cellular / Q000187:drug effects; D007153:Immunologic Deficiency Syndromes / Q000188:drug therapy / Q000209:etiology*; D007694:Killer Cells, Natural / Q000187:drug effects / Q000276:immunology; D007970:Leukopenia / Q000188:drug therapy / Q000209:etiology; D008214:Lymphocytes / Q000145:classification; D008297:Male; D008875:Middle Aged
chemical_list: D006887:Hydroxycholecalciferols; D000243:Adenosine Deaminase; C008088:alfacalcidol