Abstract
ABSTRACT: Fibrinolytic parameters and von Willebrand factor (vWF) antigen were measured in the plasma of 10 patients with hemolytic uremic syndrome (HUS). Samples were taken at presentation and again 2 wk later, before and after infusion of l-desamino-8-arginine vasopressin. Compared with the plasma values of healthy control children, levels of tissue-plasminogen activator (t-PA) antigen, plasminogen activator inhibitor type I (PAI-1) activity, and vWF as well as fibrin(ogen) degradation products were significantly elevated in the plasma of HUS patients on admission. No response of the fibrinolytic parameters and vWF were seen when l-desamino-8-arginine vasopressin infusion was given on admission. After 2 wk, t-PA antigen and vWF had partially returned to basal values, and t-PA antigen increased rapidly again after l-desamino-8-arginine vasopressin infusion. To investigate whether verocytotoxin contributes to the alteration of the fibrinolytic system found in HUS patients, purified verocytotoxin-1 (VT-1) was added to the media of cultured human endothelial cells. Addition of VT-1 alone did not change the production of t-PA, plasminogen activator inhibitor type I, and vWF antigen in these cells. However, when the endothelial cells were preincubated with tumor necrosis factor-a to increase the number of VT-1 receptors, VT-1 induced a marked decrease of the synthesis of t-PA, plasminogen activator inhibitor type I, and vWF. This was caused by a decrease in overall protein synthesis in the tumor necrosis factor-α-and VT-1-treated endothelial cells. We conclude from this study that the systemic fibrinolytic parameters measured in the plasma of HUS patients are probably not a direct effect of VT-1 on the endothelium but are sequelae of the disease in which the intestine and the kidney are predominantly affected.
Similar content being viewed by others
Article PDF
Author information
Authors and Affiliations
Rights and permissions
About this article
Cite this article
Van De Kar, N., Van Hinsbergh, V., Brommer, E. et al. The Fibrinolytic System in the Hemolytic Uremic Syndrome: In Vivo and In Vitro Studies. Pediatr Res 36, 257–264 (1994). https://doi.org/10.1203/00006450-199408000-00019
Received:
Accepted:
Issue Date:
DOI: https://doi.org/10.1203/00006450-199408000-00019
This article is cited by
-
Cardiac ischemia during hemolytic uremic syndrome
Pediatric Nephrology (2003)
-
Hemostatic complications in renal disorders of the young
Pediatric Nephrology (1996)