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Current spectrum of hepatobiliary disorders in northern India
S K Yachha1, B C Sharma, A Khanduri
1Department of Gastroenterology (Pediatric GE), Sanjay Gandhi Postgraduate Institute of Medical Sciences, Lucknow.
Insights
This study evaluated pediatric hepatobiliary disorders in Northern India, finding chronic liver disease and neonatal cholestasis syndrome to be common. Post-hepatitic and metabolic causes were prevalent in chronic liver disease cases.
Area of Science:
- Pediatric Hepatology
- Gastroenterology
- Internal Medicine
Background:
- Hepatobiliary disorders in children present a significant health challenge.
- Understanding the spectrum of these diseases is crucial for effective management and treatment strategies.
- Northern India represents a unique demographic for studying pediatric liver diseases.
Purpose of the Study:
- To determine the current prevalence and etiological spectrum of hepatobiliary disorders in pediatric patients in Northern India.
- To analyze the common patterns of liver diseases, including acute hepatitis, chronic liver disease, and neonatal cholestasis syndrome.
- To identify the primary causes of chronic liver disease in this population.
Main Methods:
- A retrospective analysis of 235 children with hepatobiliary disorders treated at a tertiary referral hospital.
- Data collection included clinical assessment, liver function tests, viral and autoimmune markers, liver biopsy, and copper studies.
- Evaluation period spanned from January 1992 to July 1995.
Main Results:
- Acute hepatitis (28%), chronic liver disease (36%), and neonatal cholestasis syndrome (NCS) (26%) were the most frequent diagnoses.
- In chronic liver disease, Wilson's disease (21%) and non-Wilsonian metabolic diseases (16%) were significant contributors, alongside post-hepatitic (13%) and cryptogenic etiologies.
- Cirrhosis was observed in 71% of chronic liver disease cases, with fulminant hepatic failure presenting in 4% of all liver disease cases.
Conclusions:
- Chronic liver diseases in Northern Indian children are predominantly post-hepatitic, metabolic, or cryptogenic, with Indian childhood cirrhosis (ICC) being rare.
- Neonatal cholestasis syndrome is a major etiological subgroup requiring attention.
- The findings highlight the need for targeted diagnostic and therapeutic approaches for pediatric liver diseases in the region.
Objective:
To evaluate the current spectrum of hepatobiliary disorders in children in Northern India.
Setting:
Tertiary level referral hospital.
Methods:
All children with hepatobiliary disorders presenting between January 1992 through July 1995 were evaluated by clinical assessment, liver function tests, viral and autoimmune markers, liver biopsy, copper studies and other relevant investigations.
Results:
Two hundred and thirty five children with hepatobiliary disorders were seen over three and a half years period (67 cases per year). Acute hepatitis (28%), chronic liver disease (36%) and neonatal cholestasis syndrome (NCS) (26%) were the most common patterns of liver diseases. Chronic liver diseases were constituted by ICC (2%), post-hepatitic etiology (13%), Wilson's disease (21%), autoimmune (4%), non-Wilsonian metabolic diseases (16%), hepatic venous outflow obstruction (2%) and non-cirrhotic portal fibrosis (1%). Cirrhosis was documented in 71% and chronic hepatitis in 12% of cases with chronic liver disease. Fulminant hepatic failure was the presentation in 4% of children with liver diseases.
Conclusion:
Chronic liver diseases in Northern India are mainly constituted by post hepatitic, metabolic and cryptogenic etiology and ICC is rarely encountered. NCS is also one of the major subgroups of liver diseases in children.