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

Completed

• Conditions: Coagulation Disorder; Cirrhosis, Liver

• Registration Number: NCT04267406
• Lead Sponsor: Ataturk University

Brief Summary

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.

Detailed 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.

Study Timeline

• Study Start: 2019-01-02
• Primary Completion: 2019-10-30
• Study Completion: 2019-12-30
• Status Verified: 2020-02
• Study First Submitted: 2020-02-07
• Study First Submitted QC: 2020-02-11
• Last Update Submitted: 2020-02-11
• Study First Posted: 2020-02-12
• Last Update Posted: 2020-02-12

Recruitment & Eligibility

• Status: COMPLETED
• Sex: All
• Target Recruitment: 64

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.

Study & Design

• Study Type: OBSERVATIONAL
• Study Design: Not specified

Primary Outcome Measures

Name	Time	Method
Measuring ADAMTS-13 activities in collected EDTA bloods.	First day	Measuring ADAMTS-13 activities using commercial ELISA kits (as a percentage) in all groups.
Measuring ADAMTS-13 enzyme levels in collected EDTA bloods.	First day	Measuring ADAMTS-13 antigen levels using commercial ELISA kits (IU/mL) in all groups.
Measuring vWF antigen levels in collected EDTA bloods.	First day	Measuring vWF antigen levels using immunoturbidimetric assay (as a percentage) in all groups.
Measuring vWF activities in collected EDTA bloods.	First day	Measuring vWF activities \[von Willebrand factor ristocetin cofactor (vWF:RCo)\] by the aggregation of platelets in the presence of ristocetin using immunoturbidimetric assay (as a percentage).

Trial Locations

Locations (1)

Ataturk University Hospital; 🇹🇷 Erzurum, Turkey