Smitz S, Legros J J
Acta Endocrinol (Copenh). 1985 Nov;110(3):346-51. doi: 10.1530/acta.0.1100346.
A patient with the chronic hypernatraemia syndrome is described. Using a sensitive and specific radioimmunoassay, the plasma arginine-vasopressin (AVP) level was measured under various conditions. With an unrestricted diet, the plasma AVP level was inappropriately low for the degree of plasma hyperosmolality (0.9 pmol/l and 302 mOsm/kg, respectively). After chronic water loading, plasma osmolality was 271 mOsm/kg, plasma AVP level 1.5 pmol/l, and the urine remained hypertonic with respect to the plasma. During hypertonic saline infusion, plasma osmolality increased from 271 to 294 mOsm/kg without a concomitant increase in the plasma AVP concentration. After sc injection of apomorphine and after haemodynamic stimulation, the plasma AVP concentration increased from 0.8 to 36 pmol/l and from 1.2 to 6.3 pmol/l, respectively. These data demonstrate a selective deficiency in the osmoregulation of the AVP secretion. The observed neuroendocrine abnormalities may be linked to a congenital malformation of the brain.
本文描述了一位患有慢性高钠血症综合征的患者。使用灵敏且特异的放射免疫分析法，在不同条件下测量了血浆精氨酸加压素（AVP）水平。在不限饮食的情况下，血浆AVP水平相对于血浆高渗程度而言过低（分别为0.9 pmol/l和302 mOsm/kg）。慢性水负荷后，血浆渗透压为271 mOsm/kg，血浆AVP水平为1.5 pmol/l，且尿液相对于血浆仍为高渗。在输注高渗盐水期间，血浆渗透压从271升高至294 mOsm/kg，而血浆AVP浓度并未随之增加。皮下注射阿扑吗啡后以及血流动力学刺激后，血浆AVP浓度分别从0.8升高至36 pmol/l以及从1.2升高至6.3 pmol/l。这些数据表明AVP分泌的渗透压调节存在选择性缺陷。观察到的神经内分泌异常可能与大脑先天性畸形有关。
