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

Akari Nakamura-Utsunomiya, Takeshi Y. Hiyama, Satoshi Okada, Masaharu Noda, Masao Kobayashi

Research output: Contribution to journalReview article

1 Citation (Scopus)

Abstract

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 hypothalamicpituitary 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 autoimmunityrelated 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.

Original languageEnglish
Pages (from-to)197-205
Number of pages9
Journalclinical pediatric endocrinology
Volume26
Issue number4
DOIs
Publication statusPublished - Jan 1 2017
Externally publishedYes

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Keywords

  • Adipsic hypernatremia
  • Hypothalamus dysfunction
  • Sensory circumventricular organs
  • Subfornical organ

ASJC Scopus subject areas

  • Pediatrics, Perinatology, and Child Health
  • Endocrinology, Diabetes and Metabolism
  • Endocrinology

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