Current knowledge in pathophysiology and management of Budd-Chiari syndrome and non-cirrhotic non-tumoral splanchnic vein thrombosis

Virginia Hernández-Gea, Andrea De Gottardi, Frank W.G. Leebeek, Pierre Emmanuel Rautou, Riad Salem, Juan Carlos Garcia-Pagan*

*Corresponding author for this work

Research output: Contribution to journalReview article

2 Citations (Scopus)

Abstract

Budd-Chiari syndrome and non-cirrhotic non-tumoral portal vein thrombosis are 2 rare disorders, with several similarities that are categorized under the term splanchnic vein thrombosis. Both disorders are frequently associated with an underlying prothrombotic disorder. They can cause severe portal hypertension and usually affect young patients, negatively influencing life expectancy when the diagnosis and treatment are not performed at an early stage. Yet, they have specific features that require individual consideration. The current review will focus on the available knowledge on pathophysiology, diagnosis and management of both entities.

Original languageEnglish (US)
Pages (from-to)175-199
Number of pages25
JournalJournal of Hepatology
Volume71
Issue number1
DOIs
StatePublished - Jul 1 2019

Fingerprint

Budd-Chiari Syndrome
Viscera
Veins
Thrombosis
Portal Hypertension
Portal Vein
Life Expectancy
Therapeutics

Keywords

  • Angioplasty
  • Coagulopathy
  • Portal hypertension
  • Portal vein thrombosis
  • Thrombophilia
  • TIPS
  • Transplant

ASJC Scopus subject areas

  • Hepatology

Cite this

Hernández-Gea, Virginia ; De Gottardi, Andrea ; Leebeek, Frank W.G. ; Rautou, Pierre Emmanuel ; Salem, Riad ; Garcia-Pagan, Juan Carlos. / Current knowledge in pathophysiology and management of Budd-Chiari syndrome and non-cirrhotic non-tumoral splanchnic vein thrombosis. In: Journal of Hepatology. 2019 ; Vol. 71, No. 1. pp. 175-199.
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abstract = "Budd-Chiari syndrome and non-cirrhotic non-tumoral portal vein thrombosis are 2 rare disorders, with several similarities that are categorized under the term splanchnic vein thrombosis. Both disorders are frequently associated with an underlying prothrombotic disorder. They can cause severe portal hypertension and usually affect young patients, negatively influencing life expectancy when the diagnosis and treatment are not performed at an early stage. Yet, they have specific features that require individual consideration. The current review will focus on the available knowledge on pathophysiology, diagnosis and management of both entities.",
keywords = "Angioplasty, Coagulopathy, Portal hypertension, Portal vein thrombosis, Thrombophilia, TIPS, Transplant",
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Current knowledge in pathophysiology and management of Budd-Chiari syndrome and non-cirrhotic non-tumoral splanchnic vein thrombosis. / Hernández-Gea, Virginia; De Gottardi, Andrea; Leebeek, Frank W.G.; Rautou, Pierre Emmanuel; Salem, Riad; Garcia-Pagan, Juan Carlos.

In: Journal of Hepatology, Vol. 71, No. 1, 01.07.2019, p. 175-199.

Research output: Contribution to journalReview article

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AU - Hernández-Gea, Virginia

AU - De Gottardi, Andrea

AU - Leebeek, Frank W.G.

AU - Rautou, Pierre Emmanuel

AU - Salem, Riad

AU - Garcia-Pagan, Juan Carlos

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AB - Budd-Chiari syndrome and non-cirrhotic non-tumoral portal vein thrombosis are 2 rare disorders, with several similarities that are categorized under the term splanchnic vein thrombosis. Both disorders are frequently associated with an underlying prothrombotic disorder. They can cause severe portal hypertension and usually affect young patients, negatively influencing life expectancy when the diagnosis and treatment are not performed at an early stage. Yet, they have specific features that require individual consideration. The current review will focus on the available knowledge on pathophysiology, diagnosis and management of both entities.

KW - Angioplasty

KW - Coagulopathy

KW - Portal hypertension

KW - Portal vein thrombosis

KW - Thrombophilia

KW - TIPS

KW - Transplant

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