Yamamoto T, Shimizu M, Fukuyama J, Yamaji T
Department of Medicine, Kishiwada Tokushukai Hospital, Osaka, Japan.
Endocrinol Jpn. 1988 Dec;35(6):915-24. doi: 10.1507/endocrj1954.35.915.
A 26-year-old man with hypothalamic hypodipsia-hypernatremia syndrome is reported, who presented with adipsia, hypernatremia, and impaired osmolality-mediated arginine vassopressin (AVP) secretion. A chorionic gonadotropin-secreting tumor was detected in the anterior hypothalamus and treated with external irradiation. After the treatment, hypernatremia persisted and was not corrected by fluid loading, osmolality-mediated AVP secretion remained impaired. Despite the absence of signs of hydropenia, hypovolemia was suggested by low blood pressure and elevated plasma indices of the renin-angiotensin system, and supported by blood volume determination. The plasma aldosterone concentrations were inappropriately low for the renin-angiotensin status. The plasma atrial natriuretic polypeptide (ANP) level was normal in spite of hypovolemia and increased more than double after fluid loading. Hypernatremia, primarily caused by hypodipsia and impaired osmolality-mediated AVP secretion, secondarily sustained ANP secretion and suppressed aldosterone release, which conceivably contributed to the development and perpetuation of hypovolemia in this patient.
报告了一名26岁患有下丘脑性低渴-高钠血症综合征的男性，其表现为无渴感、高钠血症以及渗透压介导的精氨酸加压素（AVP）分泌受损。在前下丘脑检测到分泌绒毛膜促性腺激素的肿瘤，并接受了外照射治疗。治疗后，高钠血症持续存在，且通过补液未得到纠正，渗透压介导的AVP分泌仍受损。尽管没有脱水迹象，但低血压和肾素-血管紧张素系统血浆指标升高提示存在低血容量，血容量测定也支持这一点。血浆醛固酮浓度相对于肾素-血管紧张素状态而言不适当降低。尽管存在低血容量，但血浆心钠素（ANP）水平正常，补液后升高超过两倍。高钠血症主要由低渴感和渗透压介导的AVP分泌受损引起，继而持续刺激ANP分泌并抑制醛固酮释放，这可能导致了该患者低血容量的发生和持续。
