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

Ataturk University

Status: Completed

Conditions: Cirrhosis, Liver; Coagulation Disorder

Study type: Observational

Funder types: Other

Identifiers

NCT04267406

218S871

Details and patient eligibility

About

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.

Full description

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.

Enrollment: 64 patients

Sex: All

Ages: 3 months to 18 years old

Volunteers: Accepts Healthy Volunteers

Inclusion criteria

• Patients with cirrhosis or portal hypertension aged 3 months to 18 years.

Exclusion criteria

• Being treated with fresh frozen plasma in the recent month.
• Patients previously diagnosed with bleeding diathesis.
• The patients who consumed vitamin K in the recent three weeks.
• Patients previously diagnosed with another chronic disease (such as renal failure, heart failure etc).
• Patients who suffer from acute or chronic infectious diseases.

Trial design

64 participants in 3 patient groups

Cirrhosis

Description:

Patients who diagnosed as cirrhosis. 2 ml EDTA blood sample was taken from patients during their routine controls or at the time of diagnosis. Samples will be tested for ADAMTS-13 and vWF levels and activity at the end of study.

Non-cirrhotic portal hypertension

Description:

Patients who diagnosed as extrahepatic portal hypertension (due to portal vein thrombosis). 2 ml EDTA blood sample was taken from patients during their routine controls or at the time of diagnosis. Samples will be tested for ADAMTS-13 and vWF levels and activity at the end of study.

Control

Description:

Healthy volunteers, who admitted to our hospital for any complaints, but was not determined any organic disease. 2 ml EDTA blood sample was taken from healthy volunteers, during their hospital admition. Samples will be tested for ADAMTS-13 and vWF levels and activity at the end of study.