Characteristic clinical features of adipsic hypernatremia patients with subfornical organ-targeting antibody

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Author(s)

    • Hiyama Takeshi Y.
    • Division of Molecular Neurobiology, National Institute for Basic Biology (NIBB), Aichi, Japan|School of Life Science, SOKENDAI (The Graduate University for Advanced Studies), Aichi, Japan
    • Okada Satoshi
    • Department of Pediatrics, Hiroshima University Hospital, Hiroshima. Japan
    • Noda Masaharu
    • Division of Molecular Neurobiology, National Institute for Basic Biology (NIBB), Aichi, Japan|School of Life Science, SOKENDAI (The Graduate University for Advanced Studies), Aichi, Japan
    • Kobayashi Masao
    • Department of Pediatrics, Hiroshima University Hospital, Hiroshima. Japan

Abstract

<p>Adipsic hypernatremia is a rare disease presenting as persistent hypernatremia with disturbance of thirst regulation and hypothalamic dysfunction. As a result of congenital disease, tumors, or inflammation, most cases are accompanied by structural abnormalities in the hypothalamic-pituitary area. While cases with no hypothalamic-pituitary structural lesion have been reported, their etiology has not been elucidated. Recently, we reported three patients with adipsic hypernatremia whose serum-derived immunoglobulin (Ig) specifically reacted with mouse subfornical organ (SFO) tissue. As one of the circumventricular organs (CVOs) that form a sensory interface between the blood and brain, the SFO is a critical site for generating physiological responses to dehydration and hypernatremia. Intravenous injection of the patient's Ig fraction induced hypernatremia in mice, along with inflammation and apoptosis in the SFO. These results support a new autoimmunity-related mechanism for inducing adipsic hypernatremia without demonstrable hypothalamic-pituitary structural lesions. In this review, we aim to highlight the characteristic clinical features of these patients, in addition to etiological mechanisms related to SFO function. These findings may be useful for diagnosing adipsic hypernatremia caused by an autoimmune response to the SFO, and support development of new strategies for prevention and treatment.</p>

Journal

  • Clinical Pediatric Endocrinology

    Clinical Pediatric Endocrinology 26(4), 197-205, 2017

    The Japanese Society for Pediatric Endocrinology

Codes

  • NII Article ID (NAID)
    130006105714
  • Text Lang
    ENG
  • ISSN
    0918-5739
  • Data Source
    J-STAGE 
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