ADAMTS-13 and Von Willebrand Factor Levels and Activities in Children With Cirrhosis and/or Portal Hypertension

Overview

Hemostasis-related disorders are common in cirrhosis and portal hypertension. However, it is not known whether the net effect of changes in hemostasis in the sense of predisposition to hemorrhagic or thrombotic state. It is suggested that increasing the concentration and activities of Von Willebrand factor (vWF) and decline ADAMTS-13 (A Disintegrin and Metalloproteinase with Trombospondin type 1 motif, member 13) may cause thrombophilic changes in cirrhosis and portal hypertension. The aim of this study was to investigate the changes in ADAMTS-13 (A disintegrin and metalloproteinase with thrombospondin motifs 13) and von willebrand factor (vWF) levels and activities in patients with cirrhosis and portal hypertension.

Patients of 3 months to 18 years of age, followed-up or newly diagnosed in pediatric gastroenterology unit, who had cirrhosis or non cirrhotic portal hypertension included to the study. Written informed consent obtained from the parents and/or patients. The subjects was grouped in three. The first group consisted of patients with cirrhosis (with or without portal hypertension). The second group consisted of patients with non-cirrhotic portal hypertension (developed due thrombosis of portal vein). The last group consisted of healthy volunteers. 2 ml of EDTA blood was taken from the patients and healty volunters. Samples will be tested for vWF and ADAMTS-13 levels and activities at the end of the study. The clinical scoring methods, PELD, MELD and Child Pugh scores, treatments received by patients, data from endoscopic, radiological screening, and blood analysis of patients were recorded.

Conditions