渴感减退性高钠血症病因及治疗探讨——5例报道及去氨加压素疗效观察

目的 渴感减退性高钠血症,又称特发性高钠血症.临床罕见,国内外报道有限,发病机制不详,尚无有效安全的治疗手段.本研究根据本组先前对渴感减退性高钠血症发病机制和治疗的探索,对5例渴感减退性高钠血症患者进行了醋酸去氨加压素的实验性临床治疗,观察对该病的临床缓解效果.方法 5例高钠血症伴有渴感缺失或减退的患者根据Halter等提出的参考标准诊断.对该5例患者进行血尿渗透压、血钠、尿比重、自由水清除率测定和禁水加压实验及水负荷实验,以视觉模拟刻度对渴感等级进行评估,并进行垂体前叶功能评价,所有患者进行强制性每日饮水2 000～2 500 ml,有垂体前叶功能减退者给予相应激素替代补充治疗,1周后在患者高血钠没有缓解的情况下给予醋酸去氨加压素治疗,观察高钠血症和渴感减退的缓解情况.结果 5例患者的血钠水平为160～190 mmol/L,血浆渗透压为330～370 mmol/L,而患者的渴感等级为0～2级.患者均存在部分性中枢性尿崩症,渗透压阈值升高为330～360 mmol/L.强制性每日饮水和垂体前叶激素替代治疗1周后高血钠仍然不能缓解.通过治疗尿崩症的药物醋酸去氨加压素0.05～0.2 mg/d对该5例患者进行试验性治疗5～7天后,发现患者血钠下降,渴感减退也有明显缓解,渴感等级上升为5～7级,且未见明显不良反应.结论 渴感减退性高钠血症存在中枢性部分性尿崩症,醋酸去氨加压素对渴感减退性高钠血症的治疗有效而且安全方便.

Clinical study on desmopressin acetate therapy in patients with adipsic hypernatremia: Five cases report

Objective Adipsic hypernatremia is very rare in clinical practice and only a few cases have been reported so far. Since the pathogenesis of adipsic hypernatremia is unclear, therapeutic method is very limited and uncertain. We previously found the existence of partial central diabetes insipidus in the patient with adipsic hypernatremia according to a series of clinical studies. In this clinical study, we observed the response of vasopressin in the treatment of adipsic hypernatremia as a routinely used agent in treating central diabetes insipidus.Methods Five patients with chronic sustained hypernatremia and hypodipsia were diagnosed as adipsic hypernatremia according to the criteria by Halter. After testing plasma electrolytes, urinary osmolality, plasma osmolality, and free water clearance (CH2O);with evaluating thirsty grade and anterior pituitary function, patients were forced to drink water 2 000-2 500 ml each day combined with prednisone and/or levethyroxin supplementation therapy if hypopituitarsm was revealed. One week later, since the hypernatremia was unrelieved, desmopressin acetate (0.05-0.2 mg/d)was administered to those 5 patients. About 5-7 days later, plasma electrolytes, urinary and plasma osmolality, and free water clearance ( CH2O ) were reevaluated. Osmolality was detected by the method of freezing point depression and thirst grade by the method of visual analogue scales. Results The plasma sodium was 160-190 mmol/L and plasma osmolality was 330-370 mmoL/L without thirsty perception during the hospitalization. Four of the five patients were revealed to be suffering from hypopituitarism, but the hypernatremia could not be corrected by intentional water intake combined with prednisone treatment. After administering vasopressin 0.05-0.2 mg/d to these patients, that hypernatremia was relieved, and hyperosmolality and hypodypsia were improved effectively. During this period of time, no side effects were detected in these patients. Conclusion We testified the existence of central partial diabetes insipidus in the patients with adipsic hypernatremia. Desmopressin acetate in the treatment of adipsic hypernatremia is proved to be effective, simple, and safe.