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

Study Overview
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Brief Summary
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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
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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.

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

Conditions
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Coagulation Disorder, Cirrhosis, Liver

Participation Criteria
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Eligibility Criteria
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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.

Ages Eligible for Study
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Minimum Age: 3 Months
Maximum Age: 18 Years

Sexes Eligible for Study
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All

Accepts Healthy Volunteers
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Accepts Healthy Volunteers

Study Plan
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How is the study designed?
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Arms and Interventions

| Participant Group/Arm | Intervention/Treatment |
| --- | --- |
| Cirrhosis<br>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<br>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<br>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. | |

What is the study measuring?
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Primary Outcome Measures

| Outcome Measure | Measure Description | Time Frame |
| --- | --- | --- |
| 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 | 

 Terms related to the study
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Keywords Provided by Centre Hospitalier Valida
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ADAMTS-13, Von willebrand factor, Children, Cirrhosis, Portal hypertension