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The goal of this observational study is to learn about the changes in coagulation factor VIII and IX levels in patients undergoing liver transplantation to help guide future management of coagulation factor replacement in patients with hemophilia and liver disease. The question we aim to answer is: should the recommendations for factor replacement in patients with hereditary bleeding disorders be altered in the setting of end stage liver cirrhosis?
Participants will be asked to provide two blood samples, one at the beginning of their liver transplant, and one after their liver transplant.
Current guidelines for management of hemophilia B suggest replacement of factor IX to 100% prior to major abdominal surgery. However, in patients with concurrent liver cirrhosis where the liver does not produce Factor IX, is it worth considering adjusting the factor replacement strategy? We recently had a case of a patient with Hemophilia B and end-stage liver disease (ESLD) who underwent orthotopic liver transplantation and received the standard pre-operative recombinant factor IX replacement. His case was complicated by intra-cardiac thrombus and hypotension. We conduct a small study to assess the pre-operative thromboelastography (TEG) and factor levels in ESLD patient which we hope will help guide clinical decision making in future hemophilia B patients with cirrhosis.
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| Label | Type | Description | Intervention Names |
|---|---|---|---|
| ESLD | All participants recruited will belong to this group. These participants will all have end stage liver disease and be listed for liver transplant with an accepted organ offer. |
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| Measure | Description | Time Frame |
|---|---|---|
| Factor VIII level | We will collect Factor VIII level pre- and post- transplant | 12 hours |
| Factor IX level | We will collect Factor IX level pre- and post- transplant | 12 hours |
| Thromboelastography (TEG) values | We will collect thromboelastography values pre- and post- transplant | 12 hours |
| Measure | Description | Time Frame |
|---|---|---|
| Complications | We will collect data on bleeding or clotting events during the liver transplant surgery and 24 hours post-operatively | 24 hours |
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Inclusion Criteria:
Exclusion Criteria:
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Participants will be recruited for the study who have end stage liver disease and are undergoing liver transplant at Stanford hospital
| Name | Role | Phone | Extension | |
|---|---|---|---|---|
| Alexandra Ruan, MD | Contact | 650-723-4000 | aruan@stanford.edu |
| Name | Affiliation | Role |
|---|---|---|
| Alexandra Ruan, MD | Stanford University | Study Director |
| Martin Angst, MD | Stanford University | Principal Investigator |
| Facility | Status | City | State | ZIP | Country | Contacts |
|---|---|---|---|---|---|---|
| Stanford Hospital | Palo Alto | California | 94305 | United States |
| PubMed Identifier | Type | Citation | Retractions |
|---|---|---|---|
| 29931985 | Background | Alonso Madrigal C, Dobon Rebollo M, Laredo de la Torre V, Palomera Bernal L, Garcia Gil FA. Liver transplantation in hemophilia A and von Willebrand disease type 3: perioperative management and post-transplant outcome. Rev Esp Enferm Dig. 2018 Aug;110(8):522-526. doi: 10.17235/reed.2018.5204/2017. | |
| 26390184 | Background |
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| ID | Term |
|---|---|
| D008103 | Liver Cirrhosis |
| D006467 | Hemophilia A |
| D002836 | Hemophilia B |
| D017093 | Liver Failure |
| D013927 | Thrombosis |
| D006470 | Hemorrhage |
| D005355 | Fibrosis |
| ID | Term |
|---|---|
| D008107 | Liver Diseases |
| D004066 | Digestive System Diseases |
| D010335 | Pathologic Processes |
| D013568 | Pathological Conditions, Signs and Symptoms |
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| Togashi J, Akamatsu N, Tanaka T, Sugawara Y, Tsukada K, Kaneko J, Arita J, Sakamoto Y, Hasegawa K, Kokudo N. Living donor liver transplantation for hemophilia with special reference to the management of perioperative clotting factor replacement. Liver Transpl. 2016 Mar;22(3):366-70. doi: 10.1002/lt.24341. Epub 2016 Feb 15. No abstract available. |
| 8579741 | Background | Kadry Z, de Moerloose P, Giostra E, Morel P, Huber O, Meili E, Blum HE, Mentha G. Orthotopic liver transplantation in hemophilia B: a case report. Transpl Int. 1995;8(6):485-7. doi: 10.1007/BF00335602. |
| 30328141 | Result | Ramiz S, Hartmann J, Young G, Escobar MA, Chitlur M. Clinical utility of viscoelastic testing (TEG and ROTEM analyzers) in the management of old and new therapies for hemophilia. Am J Hematol. 2019 Feb;94(2):249-256. doi: 10.1002/ajh.25319. Epub 2018 Dec 7. |
| D025861 | Blood Coagulation Disorders, Inherited |
| D001778 | Blood Coagulation Disorders |
| D006402 | Hematologic Diseases |
| D006425 | Hemic and Lymphatic Diseases |
| D020147 | Coagulation Protein Disorders |
| D006474 | Hemorrhagic Disorders |
| D030342 | Genetic Diseases, Inborn |
| D009358 | Congenital, Hereditary, and Neonatal Diseases and Abnormalities |
| D040181 | Genetic Diseases, X-Linked |
| D048550 | Hepatic Insufficiency |
| D016769 | Embolism and Thrombosis |
| D014652 | Vascular Diseases |
| D002318 | Cardiovascular Diseases |