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Changing profile of Budd Chiari syndrome in India
C E Eapen1, Thomas Mammen, Vinu Moses
1Department of Gastrointestinal Sciences, Christian Medical College, Vellore 632 004, Tamil Nadu, India. eapen@cmcvellore.ac.in
Insights
Budd Chiari syndrome (BCS) presentation in India has shifted over 30 years. Earlier isolated inferior vena cava (IVC) obstruction is now less common, with combined IVC and hepatic vein obstruction more prevalent, possibly due to earlier diagnosis.
Area of Science:
- Hepatology
- Vascular Medicine
- Epidemiology
Background:
- Budd Chiari syndrome (BCS) is a rare condition often linked to acquired factors and thrombophilia.
- Geographical variations in BCS patterns suggest diverse underlying predisposing factors.
- Previous studies highlighted isolated inferior vena cava (IVC) obstruction as the primary presentation in India.
Purpose of the Study:
- To review the evolving disease profile of Budd Chiari syndrome in India over the past three decades.
- To identify changes in the commonest type of venous obstruction.
- To explore potential reasons for observed shifts in BCS presentation.
Main Methods:
- Retrospective review of Budd Chiari syndrome cases in India.
- Analysis of disease patterns over three distinct decades (e.g., 1990s, 2000s, 2010s).
- Comparison of obstruction types (isolated IVC vs. combined IVC and hepatic vein) across time periods.
Main Results:
- A significant shift in BCS presentation observed in India over 30 years.
- Isolated IVC obstruction, once predominant, is now a minority presentation.
- Combined IVC and hepatic vein obstruction has become the most frequent type.
- Longer illness duration correlates with IVC obstruction, suggesting earlier diagnosis may influence presentation.
Conclusions:
- The disease profile of Budd Chiari syndrome in India has changed, with a move towards more complex venous obstruction patterns.
- Earlier diagnosis, potentially influenced by improved hygiene and sanitation, may explain the shift from isolated IVC obstruction.
- Predisposing factors like poverty, malnutrition, infections, and filariasis might be less dominant or changing in their influence.
Abstract:
There is increasing evidence that Budd Chiari syndrome occurs when acquired predisposing factor(s) affect a susceptible individual with one or more underlying thrombophilic conditions. Geographical variations in disease pattern of Budd Chiari syndrome exist, which may reflect differing predisposing factors. We review a change in disease profile of Budd Chiari syndrome in India over the past three decades. While earlier studies from India reported isolated inferior vena cava (IVC) obstruction as the commonest disease type, this is a minority in more recent reports where a combination of IVC and hepatic vein obstruction is the commonest type. Longer duration of illness has been shown to be associated with IVC obstruction and the recent change in disease profile in India may reflect earlier diagnosis of Budd Chiari syndrome. Poverty, malnutrition, recurrent bacterial infections and filariasis have been previously suggested as predisposing factors for IVC obstruction. Improvement in hygiene and sanitation may partly explain the recent change in disease profile of Budd Chiari syndrome in India.
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