渴感减退而加压素调节正常的慢性高钠血症2例报告及文献复习

Chronic hypodipsic hypernatremia with normal osmoregulation of arginine vasopressin report of two cases and related literature review

目的探讨渴感减退而加压素调节正常的慢性高钠血症患者的临床特征。方法回顾性分析北京协和医院2例及联机医学文献检索系统(MEDLINE)收录的3例患者的临床资料。结果5例患者的临床特征是:①15岁前起病,慢性病程;②无渴感,意识清楚伴生长发育迟滞;③血钠持续重度升高并间歇性加重;④血浆加压素(AVP)水平及其对血浆渗透压刺激反应正常;⑤单纯饮水治疗即可纠正高钠血症。结论人类司渴感渗透压调节和AVP渗透压调节的神经通路是独立的。此类慢性高钠血症的治疗以单纯补水为主。

Objective To investigate the clinical characters of chronic hypodipsic hypernatremia with normal osmoregulation of arginine vasopressin （AVP）. Methods The clinical data of two cases from the PUMC Hospital and three cases from MEDLINE were retrospectively analyzed. Results The following clinical characters of the five cases were discovered： ① the age of onset was before 15 and the course was chronic; ② all the patients suffered from hypodipsia with normal consciousness and retardation of growth and development; ③ hypernatremia was persistent and severe; ④ the level of plasma AVP was normal and the response of AVP to the change of plasma osmotic pressure was normal; ⑤ the hypernatremia could be corrected simply by drinking water. Conclusions The cases of hypodipsic and normal AVP regulation indicate that the neuronal pathways of thirsty regulation and AVP secretion are separated from each other. Drinking water is the major therapy for this subset of chronic hypernatremia.