Is there a higher incidence of Budd-Chiari syndrome in the Dominican population?

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Budd-Chiari Syndrome and Dominican Population

There is no evidence of a higher incidence of Budd-Chiari syndrome specifically in the Dominican population. The available clinical guidelines and research do not identify Dominican ethnicity or geographic origin as a risk factor for this condition.

Established Risk Factors for Budd-Chiari Syndrome

The etiology of Budd-Chiari syndrome (BCS) is well-characterized in the literature, and myeloproliferative neoplasms are the most common underlying cause, present in approximately 49% of cases 1. The condition is fundamentally a thrombotic disorder affecting hepatic venous outflow, and its risk factors are related to prothrombotic states rather than specific ethnic or geographic populations 2.

Primary Thrombotic Risk Factors

The major etiological factors include:

  • Myeloproliferative neoplasms (49% of BCS cases), with JAK2 mutation positivity in 29% 1
  • Inherited thrombophilias (21% of cases) including Factor V Leiden mutation (7-32% prevalence), prothrombin G20210A gene variant, protein C deficiency, protein S deficiency, and antithrombin deficiency 1
  • Acquired thrombophilias (44% of cases) 1
  • Hormonal factors (38% of cases), particularly oral contraceptives (33%) and pregnancy (6%) 1
  • Paroxysmal nocturnal hemoglobinuria (19% of cases) 1

Multiple Risk Factors Are Common

Most patients (84%) have at least one identifiable thrombotic risk factor, and nearly half (46%) have more than one 2. This emphasizes that BCS results from underlying prothrombotic conditions rather than ethnic predisposition.

Geographic and Ethnic Considerations

The available evidence base comes primarily from European cohorts 2 and does not identify any specific ethnic or geographic populations—including Dominican—as having increased susceptibility. BCS is a rare disease with an incidence of 0.1 to 10 per million inhabitants per year globally 3, and this rarity is consistent across populations.

Clinical Implication

If you are evaluating a patient of Dominican origin for possible BCS, focus your workup on the established thrombotic risk factors rather than ethnicity 4. The diagnostic approach should include:

  • Doppler ultrasound as first-line imaging (sensitivity >75%) 4
  • Thrombophilia screening including Factor V Leiden, prothrombin G20210A, protein C/S, and antithrombin levels 4
  • JAK2V617F mutation testing and evaluation for myeloproliferative neoplasms 4
  • Assessment for antiphospholipid antibodies 1

Initiate anticoagulation as soon as the diagnosis is confirmed and continue indefinitely 5, as this is the cornerstone of management regardless of the patient's ethnic background.

References

Guideline

Guideline Directed Topic Overview

Dr.Oracle Medical Advisory Board & Editors, 2025

Research

Budd-Chiari Syndrome.

Prague medical report, 2017

Guideline

Budd-Chiari Syndrome: Pathophysiology, Risk Factors, and Management

Praxis Medical Insights: Practical Summaries of Clinical Guidelines, 2025

Guideline

Treatment of Budd-Chiari Syndrome

Praxis Medical Insights: Practical Summaries of Clinical Guidelines, 2025

Professional Medical Disclaimer

This information is intended for healthcare professionals. Any medical decision-making should rely on clinical judgment and independently verified information. The content provided herein does not replace professional discretion and should be considered supplementary to established clinical guidelines. Healthcare providers should verify all information against primary literature and current practice standards before application in patient care. Dr.Oracle assumes no liability for clinical decisions based on this content.

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