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

NCT04267406 | Completed

• 1 other identifier: 218S871
• Study Type: observational
• Target: 64
• Locations: 1 country
• Sites: 1

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.

Conditions

Coagulation Disorder; Cirrhosis, Liver

Keywords

Von willebrand factor; ADAMTS-13; Children; Cirrhosis; Portal hypertension

Outcome Measures

Primary Outcomes (4)

• 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

• Measuring ADAMTS-13 activities in collected EDTA bloods.

  Measuring ADAMTS-13 activities using commercial ELISA kits (as a percentage) in all groups.

  First day

• Measuring vWF antigen levels in collected EDTA bloods.

  Measuring vWF antigen levels using immunoturbidimetric assay (as a percentage) in all groups.

  First day

• 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

Study Arms (3)

Cirrhosis

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

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

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.

Eligibility Criteria

• Age: 3 Months - 18 Years
• Sex: all
• Healthy Volunteers: Yes
• Age Groups: Child (0-17), Adult (18-64)
• Sampling Method: Probability Sample

Study Population

Patients with cirrhosis and/or portal hypertension who were treated in Pediatric Gastroenterology Department of Ataturk University Hospital included to the study.

You may qualify if:

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

You may not qualify if:

• 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.

Sponsors & Collaborators

• Ataturk University: lead
• The Scientific and Technological Research Council of Turkey: collaborator

Study Sites (1)

Ataturk University Hospital

Erzurum, 25240, Turkey (Türkiye)

Location

Related Publications (6)

• 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.

  PMID: 20546962 BACKGROUND

• Plaimauer B, Zimmermann K, Volkel D, Antoine G, Kerschbaumer R, Jenab P, Furlan M, Gerritsen H, Lammle 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. doi: 10.1182/blood-2002-05-1397. Epub 2002 Jul 12.

  PMID: 12393399 BACKGROUND

• 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.

  PMID: 19822897 BACKGROUND

• 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):355-63. doi: 10.1007/s12664-014-0460-4. Epub 2014 Apr 24.

  PMID: 24756423 RESULT

• 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;99(6):1019-29. doi: 10.1160/TH08-01-0006.

  PMID: 18521503 RESULT

• 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. doi: 10.1002/hep.21231.

  PMID: 16799972 RESULT

Biospecimen

Retention: SAMPLES WITHOUT DNA

Blood product

MeSH Terms

Conditions

Hemostatic Disorders; Liver Cirrhosis; Fibrosis; Hypertension, Portal

Condition Hierarchy (Ancestors)

Vascular Diseases; Cardiovascular Diseases; Hemorrhagic Disorders; Hematologic Diseases; Hemic and Lymphatic Diseases; Liver Diseases; Digestive System Diseases; Pathologic Processes; Pathological Conditions, Signs and Symptoms

Study Officials

• Ali Islek, MD

  Ataturk University School of Medicine

  PRINCIPAL INVESTIGATOR

Study Design

• Study Type: observational
• Observational Model: CASE CONTROL
• Time Perspective: PROSPECTIVE
• Sponsor Type: OTHER
• Responsible Party: PRINCIPAL INVESTIGATOR
• PI Title: Associate Professor

Study Record Dates

• First Submitted: February 7, 2020
• First Posted: February 12, 2020
• Study Start: January 2, 2019
• Primary Completion: October 30, 2019
• Study Completion: December 30, 2019
• Last Updated: February 12, 2020

Record last verified: 2020-02

Data Sharing

• IPD Sharing: Will not share

Locations

TU (1)