Budd-Chiari syndrome risk factors

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Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1]; Associate Editor(s)-in-Chief:

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Risk Factors

  • Approximately 80 percent of patients with the Budd-Chiari syndrome have an underlying disorder.Many patients may have more than one risk factor. Same patient may have multiple causes that predispose to the development of Budd-Chiari Syndrome.

Associated risk factors include:

  • Hematologic disorders including:
    • Polycythemia rubra vera
    • Paroxysmal nocturnal hemoglobinuria
    • myeloproliferative disorder
    • Antiphospholipid antibody syndrome
    • Essential thrombocytosis
    • Inherited thrombotic diathesis
  • Coagulopathies include the following:
    • Protein C deficiency
    • Protein S deficiency
    • Antithrombin III deficiency
    • Factor V Leiden deficiency
  • Chronic infections like:
    • Hydatid cysts
    • Aspergillosis
    • Amebic abscess
    • Syphilis
    • Tuberculosis
  • Chronic inflammatory diseases such as:
    • Behçet disease
    • Inflammatory bowel disease
    • Sarcoidosis
    • Systemic lupus erythematosus
    • Sjögren syndrome
    • Mixed connective-tissue disease
  • Tumors such as
    • Hepatocellular carcinoma (HCC)
    • Renal cell carcinoma
    • Leiomyosarcoma
    • Adrenal carcinoma
    • Wilms tumor
    • Right atrial myxoma
  • Congenital membranous obstructions that includes the following:
    • Type I: Thin membrane is present in the vena cava or the atrium
    • Type II: A part of the vena cava is absent
    • Type III: The inferior vena cava (IVC) cannot be filled, and collaterals have developed
  • Miscellaneous risk factors of Budd-Chiari syndrome include the following:
    • Alpha1-antitrypsin deficiency
    • Dacarbazine
    • Urethane
    • Hypoplasia of the suprahepatic veins
    • Postsurgical obstruction
    • Posttraumatic obstruction
    • Total parenteral nutrition (TPN): Budd-Chiari syndrome can be a complication of TPN via an IVC catheter in a neonate

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