Normal Response of Factor VIII and von Willebrand Factor to 1-Deamino-8 d -Arginine Vasopressin in Nephrogenic Diabetes Insipidus

The effects of 1-deamino-8D-arginine vasopressin (DDAVP) on plasma factor VIII and von Willebrand factor (vWF) levels were studied in four patients with nephrogenic diabetes insipidus (NDI) from two unrelated families. While the urine osmolality remained low, the plasma levels of factor VIII and vWF rose 2- to 3-fold after infusion of 0.3 micrograms/kg DDAVP, compared to preinfusion levels. The degree of increase was similar in both NDI patients and normal subjects. We conclude that in NDI patients the end-organ resistance to DDAVP is confined to the kidneys and does not involve the sites from which factor VIII and vWF are released.