Coagulation DisorderNCT04267406
ADAMTS-13 and Von Willebrand Factor Levels and Activities in Children With Cirrhosis and/or Portal Hypertension
An observational study of Coagulation Disorder and Cirrhosis, Liver, run by Ataturk University at 1 site in Turkey (Türkiye).
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. Read more about this study
- Who is it for?
- Ages 3 months to 18 yearsHealthy volunteers can take part
- Conditions the study is for
- Coagulation Disorder, Cirrhosis, Liver
- How many people
- 64 participants
- Where
- 1 siteTurkey (Türkiye)
- Study dates
- 2 Jan 2019to 30 Dec 2019
- Phase
- Observational
- Are there results?
- None posted in the registry
Key requirements
Who can take part, as listed by the sponsor. Only the study team can confirm whether you are eligible, so take this list to your doctor.
- Ages 3 months to 18 years
- All sexes
- Healthy volunteers accepted
- Child, Adult
Study population: Patients with cirrhosis and/or portal hypertension who were treated in Pediatric Gastroenterology Department of Ataturk University Hospital included to the study.
Who may be able to join
- Patients with cirrhosis or portal hypertension aged 3 months to 18 years.
Who cannot join
- 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 locations and contacts
1 site in 1 country.
About this study
Official title: Investigation of Changes in ADAMTS-13 and Von Willebrand Factor Levels and Activities in Children With Cirrhosis and/or Portal Hypertension
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.
Read the 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.
- Conditions
- Coagulation Disorder, Cirrhosis, Liver
- Keywords
- Von willebrand factor, ADAMTS-13, Children, Cirrhosis, Portal hypertension
- Medical subject headings
- Hemostatic Disorders, Liver Cirrhosis, Fibrosis, Hypertension, Portal
What participants receive
What participants receive. In a randomised study each person is assigned to one group by chance.
| Study group | Role | What they receive |
|---|---|---|
| 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. |
What is being measured
The primary outcome is the main question the study is designed to answer.
Measuring ADAMTS-13 enzyme levels in collected EDTA bloods.
Measuring ADAMTS-13 antigen levels using commercial ELISA kits (IU/mL) in all groups.
Measured: First day
Measuring ADAMTS-13 activities in collected EDTA bloods.
Measuring ADAMTS-13 activities using commercial ELISA kits (as a percentage) in all groups.
Measured: First day
Measuring vWF antigen levels in collected EDTA bloods.
Measuring vWF antigen levels using immunoturbidimetric assay (as a percentage) in all groups.
Measured: 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).
Measured: First day
Timeline
From the start of the study to its completion; the mark is today. Estimated dates are the sponsor’s plan.
- First posted12 Feb 2020
- Study start2 Jan 2019
- Primary completion30 Oct 2019
- Study completion30 Dec 2019
Resources
- Official record on ClinicalTrials.govThe registry entry this page is built from, with its full change history.
- Publications (6)Papers the sponsor has linked to this study.
- Other trials for Coagulation DisorderStudies of the same condition, open and under way.
- More trials run by Ataturk UniversityThe sponsor's other registered studies.
- What the trial phases meanHow a treatment moves from first tests in people to approval.
More details
For doctors and researchers: how the study is designed, who runs it, and its registry IDs.
Study design
- Study type
- Observational
- Observational model
- Case-control
- Time perspective
- Prospective
- Enrolment
- 64 participants (actual)
Sponsor and investigators
- Lead sponsor
- Ataturk University
- Sponsor type
- University, hospital or other
- Collaborators
- The Scientific and Technological Research Council of Turkey
- Responsible party
- Principal investigator, Ali Islek, Ataturk University
- Investigators
- Ali Islek, MD, Principal investigator, Ataturk University School of Medicine
IDs, dates and publications
- ClinicalTrials.gov ID
- NCT04267406
- Sponsor's protocol ID
- 218S871
- Status last verified
- Feb 2020
- First submitted
- 7 Feb 2020
- Last updated
- 12 Feb 2020
- Results posted
- No
- Data monitoring committee
- No
- FDA-regulated drug
- No
- FDA-regulated device
- No
- Individual data sharing
- No
Publications
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.
PubMed 20546962Plaimauer 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.
PubMed 12393399Turner 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.
PubMed 19822897Goel 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.
PubMed 24756423Uemura 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.
PubMed 18521503Lisman 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.
PubMed 16799972
Study at a glance
From the registry record, last updated by the sponsor on 12 Feb 2020. Recruitment status is as the sponsor reported it: confirm with the study team.
- Official title
- Investigation of Changes in ADAMTS-13 and Von Willebrand Factor Levels and Activities in Children With Cirrhosis and/or Portal Hypertension
- ClinicalTrials.gov ID
- NCT04267406
- Acronym / sponsor ID
- 218S871
- Conditions
- Coagulation Disorder, Cirrhosis, Liver
- Keywords
- Von willebrand factor, ADAMTS-13, Children, Cirrhosis, Portal hypertension
- Study type
- Observational
- Recruitment status
- Completed
- Enrolment
- 64 participants (actual)
- Lead sponsor
- Ataturk UniversityUniversity, hospital or other
- Collaborators
- The Scientific and Technological Research Council of Turkey
- Study start
- 2 Jan 2019 (actual)
- Primary completion
- 30 Oct 2019 (actual)
- Study completion
- 30 Dec 2019 (actual)
- First posted
- 12 Feb 2020 (actual)
- Results
- Not posted in the source record
Clinical summary
The research question as the sponsor registered it. This describes what the study plans to test, not what it found.
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 design
- Observational model
- Case-control
- Time perspective
- Prospective
- Number of arms
- 3
| Arm | Type | Receives |
|---|---|---|
| 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. |
Who may qualify
- Ages
- 3 months to 18 years
- Sex
- All
- Healthy volunteers
- Accepted
- Age groups
- Child, Adult
- Study population
- Patients with cirrhosis and/or portal hypertension who were treated in Pediatric Gastroenterology Department of Ataturk University Hospital included to the study.
Key inclusion criteria (1 listed)
- Patients with cirrhosis or portal hypertension aged 3 months to 18 years.
Key exclusion criteria (5 listed)
- 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.
Eligibility is decided by the study team after screening; this overview does not establish that a patient qualifies. Full inclusion and exclusion criteria.
Primary outcome
Measuring ADAMTS-13 enzyme levels in collected EDTA bloods.
Time frame: First day
Measuring ADAMTS-13 antigen levels using commercial ELISA kits (IU/mL) in all groups.
Measuring ADAMTS-13 activities in collected EDTA bloods.
Time frame: First day
Measuring ADAMTS-13 activities using commercial ELISA kits (as a percentage) in all groups.
Measuring vWF antigen levels in collected EDTA bloods.
Time frame: First day
Measuring vWF antigen levels using immunoturbidimetric assay (as a percentage) in all groups.
Measuring vWF activities in collected EDTA bloods.
Time frame: 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).
Results and safety
The study is completed, but results are not available in the source record. That does not mean the treatment did or did not work.
Research links
- Official record on ClinicalTrials.govLast updated by the sponsor 12 Feb 2020.
- Background: PubMed 20546962Lisman 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.
- Background: PubMed 12393399Plaimauer 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.
- Background: PubMed 19822897Turner 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.
- Results publication: PubMed 24756423Goel 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.
- Results publication: PubMed 18521503Uemura 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.
- Results publication: PubMed 16799972Lisman 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.
- Individual participant dataSharing planned: No
Related trials for Coagulation Disorder
RecruitingObservational study
A Study in Children and, Adults With Congenital Thrombotic Thrombocytopenic Purpura (cTTP) Treated With Adzynma
Thrombotic Thrombocytopenic Purpura (TTP)
RecruitingPhase 1/2
Harmony-HHT: ATV-1601 in Participants With Hereditary Hemorrhagic Telangiectasia (HHT)
Hereditary Hemorrhagic Telangiectasia (HHT), Osler Weber Rendu Disease
RecruitingObservational study
The CACHE Study: Coronary Artery Care in HaEmophilia
Haemophilia, Cardiovascular Diseases
Active, not recruitingObservational study
An Observational Study of Participants With Type 3 Von Willebrand Disease on Prophylactic Standard-of-Care Treatment
Von Willebrand Disease, Type 3
RecruitingPhase 2
A Study to Assess the Tolerability of Ianalumab (VAY736) With Investigator's Choice Thrombopoietin Receptor Agonist (IC TPO-RA) in Patients With Primary Immune Thrombocytopenia (ITP)
Primary Immune Thrombocytopenia (ITP), Primary Evans Syndrome (ES)
Referral and contact
AzTrials does not enrol participants or take referrals. Contact the study team or a site listed below; they decide who can take part. This study is not recruiting at the moment.
Ali Islek, MD principal investigator
Ataturk University School of Medicine
1 site in 1 country: see every site and its status.
Questions about this trial
What is this clinical trial studying?
An observational study of Coagulation Disorder and Cirrhosis, Liver, run by Ataturk University at 1 site in Turkey (Türkiye).
The full registered description is in the About this study section and the official record.
Who may be eligible to take part?
The sponsor lists 1 inclusion and 5 exclusion criteria. Eligibility is decided by the study team after screening, so read the full criteria and contact a listed site.
Is this clinical trial currently recruiting?
The record says: finished, as of the sponsor's last update on 12 Feb 2020. Status can change between updates, so confirm with the study contact before making plans.
Where is the study taking place?
The record lists 1 site in Turkey (Türkiye). Whether a site is taking part right now should be confirmed with that site. See every site.
What are the possible benefits and risks?
They depend on the study and the treatment, and no study can promise a benefit. The study team explains the known risks during informed consent, before anyone joins; the record itself does not list them.
Are results available for this study?
The study is completed, but results are not available in the source record. That does not mean the treatment did or did not work.
How can a doctor refer a patient or contact the research team?
The record lists no contact. The official record names the sponsor, who can be asked directly. AzTrials does not enrol participants or take referrals.
What does the primary outcome measure?
Measuring ADAMTS-13 enzyme levels in collected EDTA bloods. (time frame: First day). The study lists 4 primary outcomes in all. It is what the study was designed to measure, not a finding.
Where can I read the original study information?
On the ClinicalTrials.gov record for NCT04267406, which also lists 6 linked publications.
Does a listing mean the treatment is approved or proven to work?
No. A listing means the study is registered. It does not show that the treatment is safe, works, or is approved by a regulator.
How often is this information updated?
AzTrials copies changed records from ClinicalTrials.gov every night and reloads the whole registry every week. The sponsor last updated this record on 12 Feb 2020.