Luciani J C, Conte-Devolx B, Fourcade J C, Barjon P
Clin Nephrol. 1980 May;13(5):242-7.
A syndrome of chronic hypernatremia (range 148 to 161 mmoles/l) and partial hypopituitarism (growth hormone and gonadotropin deficiencies) is reported in a 27 year-old man with sarcoid hypothalamic involvement. The patient did not complain of thirst and spontaneous fluid intake was not sufficient to restore the serum sodium to normal. However, when larger amounts of water were given (50 ml/kg for 180 min), the plasma osmolality returned to normal values in 3 hours. Blood volume values were found subnormal on two occasions on free diet (63 and 74% of the theorical normal values) and plasma renin activity was elevated (22 ng/ml/hour). Plasma vasopressin (AVP) concentrations (range < 1 to 1.9 pg/ml) were inappropriately low for the degree of plasma osmolality and remained markedly subnormal when hypertonic saline was infused (NaCl 5%, 10 ml/min for 60 min). However, the secretory stores and hemodynamic control of AVP release were intact since a rise in plasma AVP to 10.8 pg/ml was observed after induction of arterial hypotension with sodium nitroprusside infusion. These results provide further direct evidence fo the dysfunction of the thirst mechanism and the osmotic contol of AVP release. They support the concept that osmoreceptor areas are anatomically distinct from the neurohypophyseal AVP secretory system and that neural inputs from baroreceptor and osmoreceptor cells are completely separated.
一名27岁患有结节病累及下丘脑的男性患者,被报道患有慢性高钠血症(范围为148至161毫摩尔/升)和部分垂体功能减退(生长激素和促性腺激素缺乏)综合征。患者没有口渴主诉,自发饮水量不足以使血清钠恢复正常。然而,当给予大量水时(50毫升/千克,持续180分钟),血浆渗透压在3小时内恢复到正常水平。在两次自由饮食情况下发现血容量值低于正常(理论正常值的63%和74%),血浆肾素活性升高(22纳克/毫升/小时)。血浆血管加压素(AVP)浓度(范围<1至1.9皮克/毫升)相对于血浆渗透压程度而言不适当降低,在输注高渗盐水(5%氯化钠,10毫升/分钟,持续60分钟)时仍明显低于正常。然而,AVP释放的分泌储备和血流动力学控制是完整的,因为在用硝普钠输注诱导动脉低血压后,观察到血浆AVP升高至10.8皮克/毫升。这些结果为口渴机制功能障碍和AVP释放的渗透控制提供了进一步的直接证据。它们支持这样的概念,即渗透压感受器区域在解剖学上与神经垂体AVP分泌系统不同,并且来自压力感受器和渗透压感受器细胞的神经输入是完全分开的。