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

Cirrhosis, Liver Clinical Trial

Official title:

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

Clinical Trial Details — Status: Completed

Administrative data

• NCT number: NCT04267406
• Other study ID #: 218S871
• Status: Completed
• Start date: January 2, 2019
• Est. completion date: December 30, 2019

Study information

• Verified date: February 2020
• Source: Ataturk University
• Contact: n/a
• Is FDA regulated: No
• Study type: Observational

Clinical Trial 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.

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.

Recruitment information / eligibility

• Status: Completed
• Enrollment: 64
• Est. completion date: December 30, 2019
• Est. primary completion date: October 30, 2019
• Accepts healthy volunteers: Accepts Healthy Volunteers
• Gender: All
• Age group: 3 Months to 18 Years

Eligibility

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.

Related Conditions & MeSH terms

• Blood Coagulation Disorders
• Cirrhosis, Liver
• Coagulation Disorder
• Fibrosis
• Hemostatic Disorders
• Hypertension
• Hypertension, Portal
• Liver Cirrhosis

Locations

Country	Name	City	State
Turkey	Ataturk University Hospital	Erzurum

Sponsors (2)

Lead Sponsor	Collaborator
Ataturk University	The Scientific and Technological Research Council of Turkey

Country where clinical trial is conducted

Turkey, 

References & Publications (6)

Goel A, Alagammai PL, Nair SC, Mackie I, Ramakrishna B, Muliyil J, Keshava SN, Eapen CE, Elias E. ADAMTS13 deficiency, despite well-compensated liver functions in patients with noncirrhotic portal hypertension. Indian J Gastroenterol. 2014 Jul;33(4):355-6 — View Citation

Lisman T, Bongers TN, Adelmeijer J, Janssen HL, de Maat MP, de Groot PG, Leebeek FW. Elevated levels of von Willebrand Factor in cirrhosis support platelet adhesion despite reduced functional capacity. Hepatology. 2006 Jul;44(1):53-61. — View Citation

Lisman T, Caldwell SH, Burroughs AK, Northup PG, Senzolo M, Stravitz RT, Tripodi A, Trotter JF, Valla DC, Porte RJ; Coagulation in Liver Disease Study Group. Hemostasis and thrombosis in patients with liver disease: the ups and downs. J Hepatol. 2010 Aug;53(2):362-71. doi: 10.1016/j.jhep.2010.01.042. Epub 2010 May 12. — View Citation

Plaimauer B, Zimmermann K, Völkel D, Antoine G, Kerschbaumer R, Jenab P, Furlan M, Gerritsen H, Lämmle B, Schwarz HP, Scheiflinger F. Cloning, expression, and functional characterization of the von Willebrand factor-cleaving protease (ADAMTS13). Blood. 2002 Nov 15;100(10):3626-32. Epub 2002 Jul 12. — View Citation

Turner NA, Nolasco L, Ruggeri ZM, Moake JL. Endothelial cell ADAMTS-13 and VWF: production, release, and VWF string cleavage. Blood. 2009 Dec 3;114(24):5102-11. doi: 10.1182/blood-2009-07-231597. Epub 2009 Oct 12. — View Citation

Uemura M, Fujimura Y, Matsumoto M, Ishizashi H, Kato S, Matsuyama T, Isonishi A, Ishikawa M, Yagita M, Morioka C, Yoshiji H, Tsujimoto T, Kurumatani N, Fukui H. Comprehensive analysis of ADAMTS13 in patients with liver cirrhosis. Thromb Haemost. 2008 Jun; — View Citation

Outcome

Type	Measure	Description	Time frame	Safety issue
Primary	Measuring ADAMTS-13 enzyme levels in collected EDTA bloods.	Measuring ADAMTS-13 antigen levels using commercial ELISA kits (IU/mL) in all groups.	First day
Primary	Measuring ADAMTS-13 activities in collected EDTA bloods.	Measuring ADAMTS-13 activities using commercial ELISA kits (as a percentage) in all groups.	First day
Primary	Measuring vWF antigen levels in collected EDTA bloods.	Measuring vWF antigen levels using immunoturbidimetric assay (as a percentage) in all groups.	First day
Primary	Measuring vWF activities in collected EDTA bloods.	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).	First day