Hemodynamic and Coagulation Responses to 1-Desamino[8-D-Arginine] Vasopressin in Patients with Congenital Nephrogenic Diabetes Insipidus

Abstract The antidiuretic hormone arginine vasopressin interacts with two types of receptors: V1, which mediates the effects of vasopressin on vascular smooth muscle, and V2, which mediates the antidiuretic effects on renal tubules. Resistance of the renal tubules to arginine vasopressin and to the antidiuretic V2-specific agonist 1-desamino[8-D-arginine] vasopressin (dDAVP) occurs in congenital nephrogenic diabetes insipidus, a rare X-linked disease, although the V1-receptor responses remain intact. The extrarenal actions of dDAVP in normal persons are a decrease in blood pressure, an increase in plasma renin activity, and stimulation of the release of factor VIIIc and von Willebrand factor. We measured the response of mean arterial pressure, pulse rate, plasma renin activity, factor VIIIc, and von Willebrand factor to an infusion of dDAVP (0.3 μg per kilogram of body weight) in seven male patients with congenital nephrogenic diabetes insipidus, six obligatory carriers of the gene for nephrogenic diabete...