DISEASE SCANNER

Global Incurable Diseases Tracker

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Hepatologic Disease

Budd-Chiari Syndrome

HIGH SEVERITY

A rare condition caused by obstruction of hepatic venous outflow from thrombosis of hepatic veins or inferior vena cava. Acute form is life-threatening; chronic form causes cirrhosis. Associated with hypercoagulable states.

Global Affected

200.0K

Countries

18

Symptoms

Abdominal pain
Ascites
Hepatomegaly
Jaundice
Lower extremity edema
Fatigue
Nausea/vomiting
Hepatic encephalopathy

Treatment Options

Anticoagulation
Thrombolysis
Angioplasty/stenting
TIPS procedure
Liver transplantation
Diuretics
Treatment of underlying cause

Risk Factors

1Myeloproliferative neoplasms
2Factor V Leiden
3Prothrombin G20210A
4Antiphospholipid syndrome
5Oral contraceptives
6Pregnancy
7Paroxysmal nocturnal hemoglobinuria

Diagnostic Methods

  • 1Doppler ultrasound
  • 2CT/MR venography
  • 3Liver biopsy
  • 4Hypercoagulability workup
  • 5JAK2 mutation testing
  • 6Bone marrow biopsy

Prognosis

Variable; 5-year survival 50-90% depending on severity and treatment. Transplant-free survival improved with TIPS.

Prevention

  • Anticoagulation (if prothrombotic)
  • Management of underlying conditions
  • Avoidance of oral contraceptives (high-risk)
  • Early diagnosis

Research Status

Anticoagulation and Thrombolysis are primary treatments. Research focuses on improved therapies and outcomes.

Sources

  • https://www.niddk.nih.gov/health-information/liver-disease/budd-chiari-syndrome
  • https://www.liverfoundation.org/for-patients/about-the-liver/diseases-of-the-liver
  • https://www.ncbi.nlm.nih.gov/books/NBK470433

Medical Disclaimer

This information is for educational purposes only. Always consult healthcare professionals for medical advice, diagnosis, and treatment.