Adipsic hypernatremia without hypothalamic lesions accompanied by autoantibodies to subfornical organ
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- Takeshi Y. Hiyama
- Division of Molecular Neurobiology National Institute for Basic Biology (NIBB) Okazaki Aichi 444‐8787 Japan
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- Akari N. Utsunomiya
- Department of Pediatrics Hiroshima University Graduate School of Biomedical Sciences Hiroshima Hiroshima 734‐8551 Japan
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- Masahito Matsumoto
- Division of Molecular Neurobiology National Institute for Basic Biology (NIBB) Okazaki Aichi 444‐8787 Japan
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- Akihiro Fujikawa
- Division of Molecular Neurobiology National Institute for Basic Biology (NIBB) Okazaki Aichi 444‐8787 Japan
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- Chia‐Hao Lin
- Division of Molecular Neurobiology National Institute for Basic Biology (NIBB) Okazaki Aichi 444‐8787 Japan
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- Keiichi Hara
- Department of Pediatrics National Hospital Organization Kure Medical Center Kure Hiroshima 737‐0023 Japan
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- Reiko Kagawa
- Department of Pediatrics Hiroshima University Graduate School of Biomedical Sciences Hiroshima Hiroshima 734‐8551 Japan
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- Satoshi Okada
- Department of Pediatrics Hiroshima University Graduate School of Biomedical Sciences Hiroshima Hiroshima 734‐8551 Japan
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- Masao Kobayashi
- Department of Pediatrics Hiroshima University Graduate School of Biomedical Sciences Hiroshima Hiroshima 734‐8551 Japan
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- Mayumi Ishikawa
- Department of Pediatrics Kawasaki Municipal Hospital Kawasaki Kanagawa 210‐0013 Japan
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- Makoto Anzo
- Department of Pediatrics Kawasaki Municipal Hospital Kawasaki Kanagawa 210‐0013 Japan
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- Hideo Cho
- Department of Pediatrics Kawasaki Municipal Hospital Kawasaki Kanagawa 210‐0013 Japan
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- Shinobu Takayasu
- Department of Endocrinology and Metabolism Hirosaki University Graduate School of Medicine Hirosaki Aomori 036‐8562 Japan
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- Takeshi Nigawara
- Department of Endocrinology and Metabolism Hirosaki University Graduate School of Medicine Hirosaki Aomori 036‐8562 Japan
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- Makoto Daimon
- Department of Endocrinology and Metabolism Hirosaki University Graduate School of Medicine Hirosaki Aomori 036‐8562 Japan
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- Tomohiko Sato
- Department of Pediatrics Hirosaki University Graduate School of Medicine Hirosaki Aomori 036‐8562 Japan
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- Kiminori Terui
- Department of Pediatrics Hirosaki University Graduate School of Medicine Hirosaki Aomori 036‐8562 Japan
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- Etsuro Ito
- Department of Pediatrics Hirosaki University Graduate School of Medicine Hirosaki Aomori 036‐8562 Japan
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- Masaharu Noda
- Division of Molecular Neurobiology National Institute for Basic Biology (NIBB) Okazaki Aichi 444‐8787 Japan
説明
<jats:title>Abstract</jats:title><jats:p>Adipsic (or essential) hypernatremia is a rare hypernatremia caused by a deficiency in thirst regulation and vasopressin release. In 2010, we reported a case in which autoantibodies targeting the sensory circumventricular organs (sCVOs) caused adipsic hypernatremia without hypothalamic structural lesions demonstrable by magnetic resonance imaging (MRI); sCVOs include the subfornical organ (SFO) and organum vasculosum of the lamina terminalis (OVLT), which are centers for the monitoring of body‐fluid conditions and the control of water and salt intakes, and harbor neurons innervating hypothalamic nuclei for vasopressin release. We herein report three newly identified patients (3‐ to 8‐year‐old girls on the first visit) with similar symptoms. The common features of the patients were extensive hypernatremia without any sensation of thirst and defects in vasopressin response to serum hypertonicity. Despite these features, we could not detect any hypothalamic structural lesions by MRI. Immunohistochemical analyses using the sera of the three patients revealed that antibodies specifically reactive to the mouse SFO were present in the sera of all cases; in one case, the antibodies also reacted with the mouse OVLT. The immunoglobulin (Ig) fraction of serum obtained from one patient was intravenously injected into wild‐type mice to determine whether the mice developed similar symptoms. Mice injected with a patient's Ig showed abnormalities in water/salt intake, vasopressin release, and diuresis, which resultantly developed hypernatremia. Prominent cell death and infiltration of reactive microglia was observed in the SFO of these mice. Thus, autoimmune destruction of the SFO may be the cause of the adipsic hypernatremia. This study provides a possible explanation for the pathogenesis of adipsic hypernatremia without demonstrable hypothalamus‐pituitary lesions.</jats:p>
収録刊行物
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- Brain Pathology
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Brain Pathology 27 (3), 323-331, 2016-08-02
Wiley
