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无渴感高钠血症在儿童是一种罕见疾病,由渴感的渗透调节失常引起,并最终将打破水的动态平衡,导致体液慢性高渗透压状态。无渴感常与调节血浆渗透压的加压素分泌异常相关联。这是因为下丘脑内控制渴感的渗透压感受器与调节加压素分泌的中枢位置紧密相连。多种导致下丘脑损伤的病变(血管异常、肿瘤、肉芽肿性疾病及创伤等)均可引起该综合征。此次报道1例因高钠血症脱水导致体重明显减轻的12岁男性患儿, 其渴感的渗透调节存在选择性异常而加压素分泌正常。同时没发现存在明显的结构异常。至今为止,仅有6 例类似的患儿见诸报道。结论:当脱水患儿不觉得口渴时,应怀疑其是否存在下丘脑性无渴感高钠血症。同时要检查其抗利尿功能,因为这种情况下其加压素的渗透压调节可能已发生了改变。
Thirst-free hypernatremia is a rare condition in children and is caused by a thirsty dysregulation of osmotic regulation that eventually will break the dynamic equilibrium of water and lead to chronic hyperosmolarity of fluid. Thirst-free often associated with the regulation of plasma osmolality and abnormal secretion of vasopressin. This is because the hypothalamus that controls thirsty osmoreceptors is tightly linked to the central site that regulates the secretion of vasopressin. A variety of pathologies that cause damage to the hypothalamus (vascular abnormalities, tumors, granulomatous diseases, trauma, etc.) can all cause the syndrome. This report of a case of 12-year-old male patients with significant weight loss due to dehydration of hypernatremia had selective abnormality of osmoregulation and normal secretion of vasopressin. At the same time, no obvious structural abnormalities were found. So far, only 6 cases of similar children have been reported. Conclusion: When dehydrated children do not feel thirsty, they should be suspected whether there is hypothalamic, thirst-free hypernatremia. At the same time to check its antidiuretic function, because in this case its vasopressin regulation of osmotic pressure may have changed.