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Chronic Liver Diseases in Children: Clinical Profile and Histology
Sachin Devidas Dhole1, Archana S Kher2, Radha G Ghildiyal3
1Assistant Professor, Department of Pediatrics, A.C.P.M. Medical College , Dhule, Maharashtra, India .
Insights
This study highlights the clinical features of chronic liver disease in children, finding Wilson's disease and metabolic disorders as common causes. Aggressive treatment is crucial for poor prognostic indicators like jaundice and hepatomegaly.
Area of Science:
- Pediatric Gastroenterology and Hepatology
- Clinical Pathology
- Medical Research
Background:
- Chronic liver disease (CLD) in children presents unique diagnostic and management challenges.
- Understanding the clinical profile and histopathological correlations is vital for improving patient outcomes.
Purpose of the Study:
- To elucidate the clinical characteristics of pediatric liver and hepatobiliary disorders.
- To correlate liver biopsy histopathology with clinical findings in chronic liver disease.
- To assess prognosis, outcomes, and treatment effects in pediatric CLD.
Main Methods:
- Prospective study involving 55 pediatric patients with suspected CLD.
- Comprehensive clinical evaluation and relevant investigations.
- Liver biopsy with histopathological analysis for diagnosis and correlation.
Main Results:
- Male predominance (60%) observed, with peak incidence in the 6-12 year age group.
- Jaundice and abdominal distension were most common symptoms; hepatic encephalopathy in 29%.
- Cirrhosis found in 42% of biopsies; Wilson's disease (22%) was the most frequent diagnosis, followed by hepatitis and autoimmune hepatitis. Metabolic liver disease predominated.
Conclusions:
- A high index of suspicion is necessary for diagnosing rare CLD causes in children.
- Jaundice, hepatomegaly, hepatic encephalopathy, and cirrhosis are poor prognostic indicators.
- Expert liver biopsy interpretation is crucial for diagnosis; liver transplantation is key for end-stage disease.
Aim:
The main aim of the study is to study the clinical profile of disorders of the liver and hepatobiliary system in paediatric patients and to correlate the histopathology findings of liver biopsy in chronic liver disease. Another aim being to assess the prognosis and to know the outcome and the effects of treatment in chronic liver diseases in paediatric age group.
Materials And Methods:
It was a prospective study, included the clinical profile of Chronic Liver Diseases (CLD) in children and the histopathological correlation. A total of 55 children were thoroughly investigated by doing relevant investigations and liver biopsy.
Results:
A male predominance (60%) was noted with maximum incidence in the age group of 6-12 years. The incidence of CLD was 1.1% of total admissions. The most common presenting complaint was jaundice and abdominal distension. Hepatic encephalopathy was noted in 29% patients. Hepatomegaly was seen in 63% patients and spleenomegaly was seen in 60% patients. The incidence of cirrhosis on liver biopsy was 42% (23cases) in CLD patients. The most common diagnosis on histopathology was Wilson's disease (22%), followed by hepatitis and autoimmune hepatitis. The predominant spectrum of CLD was metabolic liver disease and also the predominant cause of death.
Conclusion:
As the incidence of CLD is quite low, a very high index of suspicion is required for its diagnosis. Some uncommon causes of CLD in children were seen in our study like neutral lipid storage disease, α1-Antitrypsin deficiency disease, lupus hepatitis, Alagille syndrome and Budd-Chiari syndrome. A patient of CLD with jaundice and hepatomegaly should be treated aggressively as those are the poor prognostic indicators of the disease. Hepatic encephalopathy and cirrhosis are also associated with poor outcome in patients with CLD. Liver biopsy histopathology by an expert and its correlation with laboratory investigations plays an important role in the diagnosis of CLD. The major cause of deaths in patients with CLD is due to end stage liver disease and fulminant hepatic failure and the only way to prevent and treat these patients is by liver transplantation.
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