title: Normal homeostasis of fibrinolysis in nephrogenic diabetes insipidus in spite of defective V2- receptor-mediated responses of tissue plasminogen activator release.
abstract: Three patients with congenital, nephrogenic diabetes insipidus (NDI) from two unrelated families were tested for haemostatic and fibrinolytic responses to DDAVP infusion and venous occlusion. None of the three patients showed a response of factor VIII:C, vWF:Ag or t-PA to DDAVP, a V2-agonist. However, the baseline levels of these factors in the patients' plasma were normal and during venous occlusion a rise in t-PA antigen and t-PA activity was observed in all patients. One patient showed a definite response of the t-PA antigen level to exercise. It is concluded that (extrarenal) V2-receptor-mediated responses are absent in these patients, but that baseline homeostasis and the response to venous occlusion and physical exertion are intact. Presumably, these depend on other mechanisms. This observation denies a central role for vasopressin receptors in the on-demand regulation of clotting and clot dissolving properties of the blood.
authors: Gaubius Institute TNO, Leiden, The Netherlands.
authors: E J
authors: EJ
authors: Brommer
authors: H
authors: H
authors: Brink
authors: F H
authors: FH
authors: Derkx
authors: M A
authors: MA
authors: Schalekamp
authors: J
authors: J
authors: Stibbe
mesh_terms: D000328:Adult; D003894:Deamino Arginine Vasopressin / Q000494:pharmacology; D003919:Diabetes Insipidus / Q000150:complications / Q000151:congenital / Q000503:physiopathology*; D005342:Fibrinolysis / Q000187:drug effects / Q000502:physiology*; D006706:Homeostasis; D006801:Humans; D007674:Kidney Diseases / Q000150:complications / Q000151:congenital / Q000503:physiopathology*; D008297:Male; D015849:Plasminogen Inactivators / Q000097:blood; D011945:Receptors, Angiotensin / Q000187:drug effects / Q000502:physiology; D017483:Receptors, Vasopressin; D010959:Tissue Plasminogen Activator / Q000378:metabolism
chemical_list: D015849:Plasminogen Inactivators; D011945:Receptors, Angiotensin; D017483:Receptors, Vasopressin; D010959:Tissue Plasminogen Activator; D003894:Deamino Arginine Vasopressin