deamino-arginine-vasopressin and Hemolytic-Uremic-Syndrome

Gastroenteritis due to Escherichia coli O157:H7 occurs in young children and is associated with consumption of under cooked beef. Approximately 5-10% of patients will develop hemolytic uremic syndrome (HUS): renal failure, microangiopathic hemolytic anemia, and thrombocytopenia. A 6-year-old boy was admitted with abdominal pain, guaiac positive stool, decreased urine output and elevated creatinine levels. Hemodialysis was initiated upon rapid progression to anuria. On hospital day # 5 he developed acute abdominal pain, which was different from his initial assessment. Exam revealed focal tenderness in the right lower quadrant with localized guarding and rebound. Ultrasound demonstrated a dilated, fluid filled tubular structure in the RLQ concerning for appendicitis. Based on these findings the patient was taken to the operating room for a laparoscopic appendectomy. The patient had undergone dialysis the previous day and was preoperatively treated with DDAVP to minimize the risk of bleeding. The procedure occurred without complication and final pathology confirmed acute appendicitis. This case highlights the unique clinical scenario in which patients with HUS require operative intervention. Surgical procedures can be performed on these patients, however, all precautions should be taken to minimize the risk of bleeding, including the use of preoperative DDAVP.

Topics: Acute Disease; Appendicitis; Child; Deamino Arginine Vasopressin; Escherichia coli Infections; Escherichia coli O157; Hemolytic-Uremic Syndrome; Hemostatics; Humans; Male; Shiga Toxins; Treatment Outcome

Topics: Anemia, Hemolytic; Antineoplastic Combined Chemotherapy Protocols; Breast Neoplasms; Deamino Arginine Vasopressin; Female; Hemolytic-Uremic Syndrome; Hemostatics; Humans; Middle Aged; Myocardial Infarction; Thrombosis

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 1-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 1-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 1-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-alpha 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-alpha- and VT-1-treated endothelial cells.(ABSTRACT TRUNCATED AT 250 WORDS)

Topics: Bacterial Toxins; Cells, Cultured; Child, Preschool; Deamino Arginine Vasopressin; Endothelium, Vascular; Female; Fibrin Fibrinogen Degradation Products; Fibrinolysis; Hemolytic-Uremic Syndrome; Humans; In Vitro Techniques; Infant; Male; Plasminogen Activator Inhibitor 1; Shiga Toxin 1; Tissue Plasminogen Activator; Tumor Necrosis Factor-alpha; von Willebrand Factor

Thrombotic obstruction of glomerular capillaries causes acute renal failure in patients with hemolytic-uremic syndrome (HUS). Recanalization of occluded vessels normally occurs by activation of the endogenous fibrinolytic system, mediated by plasminogen activators, which are stored and synthesized in the endothelial cells. However, endothelial injury is considered the primary event in the pathogenesis of HUS, and this may result in impaired fibrinolysis. In five children with HUS we performed a prospective study of plasminogen activator activity and two plasminogen activator antigens: tissue-type plasminogen activator and urokinase-type plasminogen activator before and after intravenous desmopressin. Plasminogen activator inhibitor type-1 antigen was also studied. In the acute stage of HUS plasminogen activating activity was low, in spite of elevated levels of total plasminogen activator antigens. This decrease of plasminogen activating activity was due to high levels of the plasminogen activator inhibitor. Improvement of fibrinolysis paralleled recovery from HUS. We conclude that decreased fibrinolysis is an important pathophysiologic feature of HUS.

Topics: Adolescent; Adult; Child; Child, Preschool; Deamino Arginine Vasopressin; Female; Fibrinolysis; Follow-Up Studies; Hemolytic-Uremic Syndrome; Humans; Infant; Injections, Intravenous; Male; Plasminogen Inactivators; Prospective Studies; Tissue Plasminogen Activator; Urokinase-Type Plasminogen Activator