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| Name | Class |
|---|---|
| The Scientific and Technological Research Council of Turkey | OTHER |
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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.
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.
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| Label | Type | Description | Intervention Names |
|---|---|---|---|
| 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. |
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| 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 |
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Inclusion Criteria:
• Patients with cirrhosis or portal hypertension aged 3 months to 18 years.
Exclusion Criteria:
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Patients with cirrhosis and/or portal hypertension who were treated in Pediatric Gastroenterology Department of Ataturk University Hospital included to the study.
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| Name | Affiliation | Role |
|---|---|---|
| Ali Islek, MD | Ataturk University School of Medicine | Principal Investigator |
| Facility | Status | City | State | ZIP | Country | Contacts |
|---|---|---|---|---|---|---|
| Ataturk University Hospital | Erzurum | 25240 | Turkey (Türkiye) |
| PubMed Identifier | Type | Citation | Retractions |
|---|---|---|---|
| 20546962 | Background | 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. | |
| 12393399 |
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| ID | Term |
|---|---|
| D020141 | Hemostatic Disorders |
| D008103 | Liver Cirrhosis |
| D005355 | Fibrosis |
| D006975 | Hypertension, Portal |
| ID | Term |
|---|---|
| D014652 | Vascular Diseases |
| D002318 | Cardiovascular Diseases |
| D006474 | Hemorrhagic Disorders |
| D006402 | Hematologic Diseases |
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Blood product
| 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. |
| 19822897 | 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. |
| 24756423 | Result | 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. |
| 18521503 | 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. |
| 16799972 | 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. |
| D006425 |
| Hemic and Lymphatic Diseases |
| D008107 | Liver Diseases |
| D004066 | Digestive System Diseases |
| D010335 | Pathologic Processes |
| D013568 | Pathological Conditions, Signs and Symptoms |