Browsing by Author "Vučelić, Dragica (19934507000)"

Introduction The significance of ADAMTS13 (a disintegrin and metalloproteinase with thrombospondin motif-13) activity for diagnosis and therapy of thrombotic thrombocytopenic purpura (TTP) and hemolytic uremic syndrome (HUS) is still a controversial issue. Objective The aim of this report was to analyze the value of ADAMTS13 measurements in the diagnosis of TTP and HUS. Methods At presentation, we analyzed patients with idiopathic TTP (n=18), secondary TTP (n=4), diarrhea positive HUS (n=3) and diarrhea negative HUS (n=3) treated in Belgrade, Serbia from 2004 to 2010. ADAMTS13 activity from acute phase samples was measured using the residual collagen binding activity assay at the Haemophilia and Thrombosis Centre, Milan, Italy. Results There was a significant correlation between reduced ADAMTS13 activity and idiopathic TTP diagnosis (p=0.000) as well as between lower ADAMTS13 activities and higher reticulocytes (p=0.017) and lactate dehydrogenase levels (p=0.027). Significant correlation was also found between higher pro- tease activity and diagnosis of HUS (p=0.000). There was a statistically significant correlation between higher ADAMTS13 activities and higher platelets count (p=0.002), blood urea nitrogen (p=0.000), and creatinine level (p=0.000). Conclusion Severe ADAMTS13 deficiency points at the diagnosis of idiopathic TTP and it is present in the secondary TTP but not in HUS.

Introduction: Hemostatic abnormalities in liver cirrhosis are complex and multifactorial and may predispose to prolonged hemorrhage following invasive procedures. Due to increased perioperative bleeding risks, patients with cirrhosis should undergo elective surgery after making medical preparations. It has been shown that 1-deamino-8-D-arginine vasopressin (DDAVP), desmopressin, can be used as a safe and effective remedy in preventing and treating bleeding in cirrhotics. However, there is still scarce information of adequate test(s) for assessing effects of DDAVP in platelet dysfunction. The use of platelet function analyzer-100 (PFA-100) allows more reliable assessment of impaired primary hemostasis as well as follow-up of hemostatic changes induced by DDAVP effects. Case Outline: In a 49-year-old male with ethylic liver cirrhosis and prolonged bleeding time scheduled for elective left side inguinal hernia repair, we carried out PFA-100 testing to investigate the patient's platelet functional status. Results were affirmative for the presence of platelet functional problems. By standard coagulation tests the patient was also identified as having secondary hemostasis. Preoperatively, PFA-100 was used to test the patient's response to a standard dose of DDAVP, which was favorable. The patient was operated after medical preparations with DDAVP and vitamin K. Neither bleeding complications nor side effects of DDAVP were recorded in the perioperative period. Conclusion: The PFA-100 is a simple and reliable test for the assessment of primary hemostasis as well as in monitoring of DDAVP therapy.