Budd-Chiari Syndrome Clinical Presentation

1. Khuroo MS, Al-Suhabani H, Al-Sebayel M, et al. Budd-Chiari syndrome: long-term effect on outcome with transjugular intrahepatic portosystemic shunt. J Gastroenterol Hepatol. 2005 Oct. 20(10):1494-502. [Medline].

2. Montano-Loza AJ, Tandon P, Kneteman N, Bailey R, Bain VG. Rotterdam score predicts early mortality in Budd-Chiari syndrome, and surgical shunting prolongs transplant-free survival. Aliment Pharmacol Ther. 2009 Nov 15. 30(10):1060-9. [Medline].

3. Valla DC. Primary Budd-Chiari syndrome. J Hepatol. 2009 Jan. 50(1):195-203. [Medline].

4. Segev DL, Nguyen GC, Locke JE, et al. Twenty years of liver transplantation for Budd-Chiari syndrome: a national registry analysis. Liver Transpl. 2007 Sep. 13(9):1285-94. [Medline].

5. Aydinli M, Bayraktar Y. Budd-Chiari syndrome: etiology, pathogenesis and diagnosis. World J Gastroenterol. 2007 May 21. 13(19):2693-6. [Medline].

6. Darwish Murad S, Plessier A, Hernandez-Guerra M, et al. Etiology, management, and outcome of the Budd-Chiari syndrome. Ann Intern Med. 2009 Aug 4. 151(3):167-75. [Medline].

7. Marudanayagam R, Shanmugam V, Gunson B, et al. Aetiology and outcome of acute liver failure. HPB (Oxford). 2009. 11(5):429-34. [Medline]. [Full Text].

8. Chaudhuri M, Jayaranganath M, Chandra VS. Percutaneous recanalization of an occluded hepatic vein in a difficult subset of pediatric Budd-Chiari syndrome. Pediatr Cardiol. 2012 Jun. 33(5):806-10. [Medline].

9. Paradis V, Bieche I, Dargere D, et al. Quantitative gene expression in Budd-Chiari syndrome: a molecular approach to the pathogenesis of the disease. Gut. 2005 Dec. 54(12):1776-81. [Medline]. [Full Text].

10. Tian ZL, Jia GL, Xi HL, Feng S, Wang XK, Li R. Investigation on etiology of hepatic venous obstruction Budd-Chiari syndrome. Cell Biochem Biophys. 2014 Dec. 70(3):1803-6. [Medline].

11. Rautou PE, Angermayr B, Garcia-Pagan JC, et al. Pregnancy in women with known and treated Budd-Chiari syndrome: maternal and fetal outcomes. J Hepatol. 2009 Jul. 51(1):47-54. [Medline].

12. Moucari R, Rautou PE, Cazals-Hatem D, et al. Hepatocellular carcinoma in Budd-Chiari syndrome: characteristics and risk factors. Gut. 2008 Jun. 57(6):828-35. [Medline].

13. Rajani R, Melin T, Bjornsson E, et al. Budd-Chiari syndrome in Sweden: epidemiology, clinical characteristics and survival - an 18-year experience. Liver Int. 2009 Feb. 29(2):253-9. [Medline].

14. Ki M, Choi HY, Kim KA, Kim BH, Jang ES, Jeong SH. Incidence, prevalence and complications of Budd-Chiari syndrome in South Korea: a nationwide, population-based study. Liver Int. 2016 Jul. 36(7):1067-73. [Medline].

15. Qi X, Ren W, Wang Y, Guo X, Fan D. Survival and prognostic indicators of Budd-Chiari syndrome: a systematic review of 79 studies. Expert Rev Gastroenterol Hepatol. 2015 Jun. 9 (6):865-75. [Medline].

16. Pavri TM, Herbst A, Reddy R, Forde KA. Budd-Chiari syndrome: A single-center experience. World J Gastroenterol. 2014 Nov 21. 20(43):16236-44. [Medline]. [Full Text].

17. Liu FY, Wang MQ, Duan F, Fan QS, Song P, Wang Y. Hepatocellular carcinoma associated with Budd-Chiari syndrome: imaging features and transcatheter arterial chemoembolization. BMC Gastroenterol. 2013 Jun 24. 13:105. [Medline]. [Full Text].

18. Goel RM, Johnston EL, Patel KV, Wong T. Budd-Chiari syndrome: investigation, treatment and outcomes. Postgrad Med J. 2015 Dec. 91(1082):692-7. [Medline].

19. [Guideline] Lee WM, Larson AM, Stravitz T. AASLD position paper: the management of acute liver failure: update 2011. National Guideline Clearinghouse. September 2011. Available at http://guideline.gov/content.aspx?id=36894&search=liver+failure. Accessed: Nov 3, 2014.

20. Copelan A, Remer EM, Sands M, Nghiem H, Kapoor B. Diagnosis and management of Budd Chiari syndrome: an update. Cardiovasc Intervent Radiol. 2015 Feb. 38(1):1-12. [Medline].

21. Hwang HJ, Kim KW, Jeong WK, et al. Hepatic outflow obstruction at middle hepatic vein tributaries or inferior right hepatic veins after living donor liver transplantation with modified right lobe graft: comparison of CT and Doppler ultrasound. AJR Am J Roentgenol. 2009 Sep. 193(3):745-51. [Medline].

22. Song HP, Yu M, Zhang M, et al. Diagnosis of active hemorrhage from the liver with contrast-enhanced ultrasonography after percutaneous transhepatic angioplasty and stent placement for Budd-Chiari syndrome. J Ultrasound Med. 2009 Jul. 28(7):955-8. [Medline].

23. Virmani V, Khandelwal N, Kang M, Gulati M, Chawla Y. MDCT venography in the evaluation of inferior vena cava in Budd-Chiari syndrome. Indian J Gastroenterol. 2009 Jan-Feb. 28(1):17-23. [Medline].

24. Cheng D, Xu H, Hua R, et al. Comparative study of MRI manifestations of acute and chronic Budd-Chiari syndrome. Abdom Imaging. 2015 Jan. 40(1):76-84. [Medline].

25. Garcia-Pagan JC, Heydtmann M, Raffa S, et al. TIPS for Budd-Chiari syndrome: long-term results and prognostics factors in 124 patients. Gastroenterology. 2008 Sep. 135(3):808-15. [Medline].

26. Hasija RP, Nagral A, Marar S, Bavdekar AR. Transjugular intrahepatic portosystemic shunt (TIPSS) for Budd Chiari syndrome. Indian Pediatr. 2010 Jun. 47(6):527-8. [Medline].

27. Fitsiori K, Tsitskari M, Kelekis A, Filippiadis D, Triantafyllou K, Brountzos E. Transjugular intrahepatic portosystemic shunt for the treatment of Budd-Chiari syndrome patients: results from a single center. Cardiovasc Intervent Radiol. 2014 Jun. 37(3):691-7. [Medline].

28. Van Brusselen D, Janssen CE, Scott C, et al. Budd-Chiari syndrome as presenting symptom of hepatic sarcoidosis in a child, with recurrence after liver transplantation. Pediatr Transplant. 2012 Mar. 16(2):E58-62. [Medline].

29. Tang TJ, Batts KP, de Groen PC, et al. The prognostic value of histology in the assessment of patients with Budd-Chiari syndrome. J Hepatol. 2001 Sep. 35(3):338-43. [Medline].

30. Sun YL, Fu Y, Zhou L, Ma XX, Wang ZW, Wu Y. Staged management of Budd-Chiari syndrome caused by co-obstruction of the inferior vena cava and main hepatic veins. Hepatobiliary Pancreat Dis Int. 2013 Jun. 12(3):278-85. [Medline].

31. Horton JD, San Miguel FL, Ortiz JA. Budd-Chiari syndrome: illustrated review of current management. Liver Int. 2008 Apr. 28(4):455-66. [Medline].

32. Copelan A, Remer EM, Sands M, Nghiem H, Kapoor B. Diagnosis and management of Budd Chiari syndrome: an update. Cardiovasc Intervent Radiol. 2015 Feb. 38(1):1-12. [Medline].

33. Mancuso A. An update on the management of Budd-Chiari syndrome: the issues of timing and choice of treatment. Eur J Gastroenterol Hepatol. 2015 Mar. 27(3):200-3. [Medline].

34. Tripathi D, Macnicholas R, Kothari C, et al. Good clinical outcomes following transjugular intrahepatic portosystemic stent-shunts in Budd-Chiari syndrome. Aliment Pharmacol Ther. 2014 Apr. 39(8):864-72. [Medline].

35. Rathod K, Deshmukh H, Shukla A, et al. Endovascular treatment of Budd-Chiari syndrome: single center experience. J Gastroenterol Hepatol. 2017 Jan. 32(1):237-43. [Medline].

36. Tripathi D, Sunderraj L, Vemala V, et al. Long-term outcomes following percutaneous hepatic vein recanalization for Budd-Chiari syndrome. Liver Int. 2017 Jan. 37(1):111-20. [Medline].

37. Hermeziu B, Franchi-Abella S, Plessier A, et al. Budd-Chiari syndrome and essential thrombocythemia in a child: favorable outcome after transjugular intrahepatic portosystemic shunt. J Pediatr Gastroenterol Nutr. 2008 Mar. 46(3):334-7. [Medline].

38. Murad SD, Luong TK, Pattynama PM, et al. Long-term outcome of a covered vs. uncovered transjugular intrahepatic portosystemic shunt in Budd-Chiari syndrome. Liver Int. 2008 Feb. 28(2):249-56. [Medline].

39. Panagiotou I, Kelekis DA, Karatza C, et al. Treatment of Budd-Chiari syndrome by transjugular intrahepatic portosystemic shunt. Hepatogastroenterology. 2007 Sep. 54(78):1813-6. [Medline].

40. Wu X, Ding W, Cao J, Han J, Li J. Modified transjugular intrahepatic portosystemic shunt in the treatment of Budd-Chiari syndrome. Int J Clin Pract. 2010 Mar. 64(4):460-4. [Medline].

41. Ulrich F, Pratschke J, Neumann U, et al. Eighteen years of liver transplantation experience in patients with advanced Budd-Chiari syndrome. Liver Transpl. 2008 Feb. 14(2):144-50. [Medline].

42. Oldakowska-Jedynak U, Ziarkiewicz M, Ziarkiewicz-Wroblewska B, et al. Myeloproliferative neoplasms and recurrent thrombotic events in patients undergoing liver transplantation for Budd-Chiari syndrome: a single-center experience. Ann Transplant. 2014 Nov 14. 19:591-7. [Medline].

43. Pahari H, Chaudhary RJ, Thiagarajan S, et al. Hepatic venous and inferior vena cava morphology no longer a barrier to living donor liver transplantation for Budd-Chiari syndrome: surgical techniques and outcomes. Transplant Proc. 2016 Oct. 48(8):2732-7. [Medline].

44. Cura M, Haskal Z, Lopera J. Diagnostic and interventional radiology for Budd-Chiari syndrome. Radiographics. 2009 May-Jun. 29(3):669-81. [Medline].

45. Ren W, Qi X, Yang Z, Han G, Fan D. Prevalence and risk factors of hepatocellular carcinoma in Budd-Chiari syndrome: a systematic review. Eur J Gastroenterol Hepatol. 2013 Jul. 25(7):830-41. [Medline].

46. Li T, Zhai S, Pang Z, et al. Feasibility and midterm outcomes of percutaneous transhepatic balloon angioplasty for symptomatic Budd-Chiari syndrome secondary to hepatic venous obstruction. J Vasc Surg. 2009 Nov. 50(5):1079-84. [Medline].

47. Qi X, Han G, Guo X, et al. Review article: the aetiology of primary Budd-Chiari syndrome - differences between the West and China. Aliment Pharmacol Ther. 2016 Dec. 44(11-12):1152-67. [Medline].

48. European Association for the Study of the Liver. EASL clinical practice guidelines: vascular diseases of the liver. J Hepatol. 2016 Jan. 64 (1):179-202. [Medline].

49. Mukund A, Sarin SK. Budd-Chiari syndrome: a focussed and collaborative approach. Hepatol Int. 2018 Oct 8. [Medline].

50. Chen ZK, Fan J, Cao C, Li Y. Endovascular treatment for hepatic vein-type Budd-Chiari syndrome: effectiveness and long-term outcome. Radiol Med. 2018 Oct. 123 (10):799-807. [Medline].

51. Ollivier-Hourmand I, Allaire M, Goutte N, et al, for the French Network for Vascular Disorders of the Liver. The epidemiology of Budd-Chiari syndrome in France. Dig Liver Dis. 2018 Sep. 50 (9):931-7. [Medline].

52. Su L, Hu L, Liang P, Wu Y, Sun Q, Gao J. Clinical efficacy of spectral computed tomography for evaluating liver function in patients with Budd-Chiari Syndrome. Acad Radiol. 2018 Aug 9. [Medline].

Praveen K Roy, MD, AGAF  Clinical Assistant Professor of Medicine, University of New Mexico School of Medicine

Praveen K Roy, MD, AGAF is a member of the following medical societies: American Gastroenterological Association, American Society for Gastrointestinal Endoscopy

Disclosure: Nothing to disclose.

Sarah D Komanapalli, MBBS Resident Physician in Internal Medicine, Marshfield Clinic

Sarah D Komanapalli, MBBS is a member of the following medical societies: American College of Physicians

Disclosure: Nothing to disclose.

Homayoun Shojamanesh, MD Former Fellow, Digestive Diseases Branch, National Institutes of Health

Homayoun Shojamanesh, MD is a member of the following medical societies: American College of Gastroenterology, American Gastroenterological Association, American Society for Gastrointestinal Endoscopy

Disclosure: Nothing to disclose.

Jack Bragg, DO Associate Professor, Department of Clinical Medicine, University of Missouri School of Medicine

Jack Bragg, DO is a member of the following medical societies: American College of Osteopathic Internists, American Osteopathic Association

Disclosure: Nothing to disclose.

Gautam Dehadrai, MD Department Chair, Section Chief, Department of Interventional Radiology, Norman Regional Hospital

Gautam Dehadrai, MD is a member of the following medical societies: American College of Radiology, Radiological Society of North America, Medical Council of India

Disclosure: Nothing to disclose.

Showkat Bashir, MD Assistant Professor, Department of Medicine, Division of Gastroenterology, George Washington University, Washington, DC

Showkat Bashir, MD is a member of the following medical societies: American College of Gastroenterology, American College of Physicians, American Gastroenterological Association, American Medical Association

Disclosure: Nothing to disclose.

BS Anand, MD Professor, Department of Internal Medicine, Division of Gastroenterology, Baylor College of Medicine

BS Anand, MD is a member of the following medical societies: American Association for the Study of Liver Diseases, American College of Gastroenterology, American Gastroenterological Association, American Society for Gastrointestinal Endoscopy

Disclosure: Nothing to disclose.

Abhishek Choudhary, MD Resident Physician, Department of Internal Medicine, University Hospital of Missouri-Columbia

Abhishek Choudhary, MD is a member of the following medical societies: American College of Physicians

Disclosure: Nothing to disclose.

Robert Baldassano, MD Director, Center for Pediatric Inflammatory Bowel Disease, Children's Hospital of Philadelphia; Professor, Department of Pediatrics, Division of Gastroenterology and Nutrition, University of Pennsylvania School of Medicine

Robert Baldassano, MD is a member of the following medical societies: Alpha Omega Alpha, American Academy of Pediatrics, American Gastroenterological Association, and North American Society for Pediatric Gastroenterology and Nutrition

Disclosure: Abbott, Inc Consulting fee Consulting

Carmen Cuffari, MD Associate Professor, Department of Pediatrics, Division of Gastroenterology/Nutrition, Johns Hopkins University School of Medicine

Carmen Cuffari, MD is a member of the following medical societies: American College of Gastroenterology, American Gastroenterological Association, North American Society for Pediatric Gastroenterology, Hepatology and Nutrition, and Royal College of Physicians and Surgeons of Canada

Disclosure: Nothing to disclose.

Robert J Fingerote, MD, MSc, FRCPC Consultant, Clinical Evaluation Division, Biologic and Gene Therapies, Directorate Health Canada; Consulting Staff, Department of Medicine, Division of Gastroenterology, York Central Hospital, Ontario

Robert J Fingerote, MD, MSc, FRCPC is a member of the following medical societies: American Association for the Study of Liver Diseases, American Gastroenterological Association, Canadian Medical Association, Ontario Medical Association, and Royal College of Physicians and Surgeons of Canada

Disclosure: Nothing to disclose.

Cass R Smith, MD Pediatric Gastroenterologist, Idaho Pediatric Gastroenterology, PA, and St Luke's Boise Regional Medical Center

Cass R Smith, MD is a member of the following medical societies: North American Society for Pediatric Gastroenterology, Hepatology and Nutrition

Disclosure: Nothing to disclose.

Michael C Stephens, MD, FAAP Assistant Professor of Pediatrics, Section of Pediatric GI and Nutrition, Medical College of Wisconsin; Director of IBD Center, Children's Hospital of Wisconsin

Michael C Stephens, MD, FAAP is a member of the following medical societies: American Academy of Pediatrics, American Gastroenterological Association, and North American Society for Pediatric Gastroenterology and Nutrition

Disclosure: Nothing to disclose.

Francisco Talavera, PharmD, PhD Adjunct Assistant Professor, University of Nebraska Medical Center College of Pharmacy; Editor-in-Chief, Medscape Drug Reference

Disclosure: Medscape Salary Employment

Mary L Windle, PharmD Adjunct Associate Professor, University of Nebraska Medical Center College of Pharmacy; Editor-in-Chief, Medscape Drug Reference

Disclosure: Nothing to disclose.

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