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Two Distinct Pathophysiological Mechanisms in Congenital Nephrogenic Diabetes Insipidus^*

Departments of Medicine and Pathology, State University of New York, Health Science Center, and Veterans Administration Medical Center Syracuse, New York 13210
The Division of Endocrinology and Metabolism, Johns Hopkins University School of Medicine Baltimore, Maryland 21205

Address all correspondence and requests for reprints to: Dr. Arnold M. Moses, University Hospital, 750 East Adams Street, Syracuse, New York 13210.

V₁ and V₂ vasopressin receptor functions were studied in 2 patients with congenital nephrogenic diabetes insipidus. V₁ receptor-mediated functions (increase in urinary prostaglandin E₂ excretion and plasma cortisol levels) and G₈ (guanine nucleotide-binding stimulatory protein) activity of erythrocyte membranes were normal in both patients. After infusion of 0.4 µg/kg dDAVP, a 57-yr-old male patient had no increase in plasma factor VIII coagulant, ristocetin cofactor, or fibrinolytic activity or change in von Willebrand factor multimers. In addition, he had no vasodilatory response to dDAVP, a response that occurs in normal subjects and patients with central diabetes insipidus. In contrast, a 25-yr-old female patient had normal hemostatic and vasodilatory responses to the infusion of dDAVP. These observations indicate that the cellular abnormalities in patients with congenital nephrogenic diabetes insipidus may be either at the V₂ receptor or in the postreceptor (and G₈ activity) cascade of events that mediate vasopressin-induced antidiuresis. Therefore, heterogeneity exists in the biochemical cause(s) of congenital nephrogenic diabetes insipidus in man.

^* This work was supported in part by V.A. research funds and grants RR-229 and RR-35 from the GCRC program of the Division of Research Resources, and Grants HL-32853 and DK-34281 from the NIH (Bethesda, MD).

Received December 3, 1987.

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