Analysis of water and electrolyte imbalance in a patient with adipsic hypernatremia associated with subfornical organ-targeting antibody
Analysis of water and electrolyte imbalance in a patient with adipsic hypernatremia associated with subfornical organ-targeting antibody
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穹窿下器官靶向抗体相关脂肪性高钠血症患者水电解质失衡分析
DOI：
10.1007/s13730-021-00638-2
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发表时间：
2021
期刊：
影响因子：
1
通讯作者：
Hattori Motoshi
中科院分区：
文献类型：
--
作者：
Shirai Yoko;Miura Kenichiro;Nakamura-Utsunomiya Akari;Ishizuka Kiyonobu;Hattori Miku;Hattori Motoshi
肥胖性高钠血症患者表现为慢性高钠血症，因为口渴感和盐食欲失调，没有明显的下丘脑结构病变。参与的自身抗体针对钠通道，纳欣穹窿下器官（SFO）最近已被报道。然而，该疾病背后的水和电解质失衡的病理生理学尚未阐明。我们描述的情况下，一个5岁的男孩谁抱怨头痛和呕吐，逐渐恶化。脑磁共振成像未发现异常病变。血液实验室检查显示血清钠（Na）浓度为152 mmol/L，血清渗透压为312 mOsm/L。他的体重略微下降，口渴的感觉消失了。他的血浆血管加压素浓度为0.9 pg/mL，尽管血清渗透压较高。鼓励患者饮水，并给予口服1-脱氨基-8-D-精氨酸-加压素。当血清钠浓度正常化时，血浆加压素浓度明显正常，范围为0.8 - 2.0 pg/mL。他在任何时候都没有出现多尿。使用小鼠脑切片和患者血清的免疫组织化学研究揭示了人免疫球蛋白G（IgG）抗体在小鼠SFO中的沉积。总之，我们的观察结果表明，水和电解质的不平衡，在肥胖性高钠血症的特点是一定量的加压素释放，无论血清钠浓度与无反应的高渗。
Patients with adipsic hypernatremia present with chronic hypernatremia because of defects in thirst sensation and dysregulated salt appetite, without demonstrable hypothalamic structural lesions. The involvement of autoantibodies directed against the sodium channel, Naxin the subfornical organ (SFO) has recently been reported. However, the pathophysiology of water and electrolyte imbalance underlying the disease has yet to be elucidated. We describe the case of a 5-year-old boy who complained of headaches and vomiting that gradually worsened. Brain magnetic resonance imaging detected no abnormal lesions. Blood laboratory testing revealed a serum sodium (Na) concentration of 152 mmol/L and a serum osmolarity of 312 mOsm/L. His body weight had slightly decreased, and his thirst sensation was absent. His plasma vasopressin concentration was 0.9 pg/mL, despite the high serum osmolarity. He was encouraged to drink water, and oral 1-deamino-8-D-arginine-vasopressin was administered. When serum sodium concentrations were normalized, plasma vasopressin concentrations were apparently normal and ranged from 0.8 to 2.0 pg/mL. He did not present with polyuria at any time. Immunohistochemical study using mouse brain sections and the patient’s serum revealed the deposition of human immunoglobulin G (IgG) antibody in the mouse SFO. In conclusion, our observations suggested that water and electrolyte imbalance in adipsic hypernatremia is characterized by a certain amount of vasopressin release regardless of serum sodium concentrations with no response to hyperosmolarity.
影响因子：
8
作者：
A. Hayek;G. Peake
通讯作者：
G. Peake
大脑的钠水平传感器
DOI：
--
发表时间：
2007
期刊：
影响因子：
--
作者：
Watanabe;E.
通讯作者：
E.
DOI：
10.1152/ajpregu.00432.2014
发表时间：
2015-11-01
影响因子：
2.8
作者：
Freiria-Oliveira, Andre H.;Blanch, Graziela T.;Colombari, Debora S. A.
通讯作者：
Colombari, Debora S. A.