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[Liver cirrhosis in childhood. Considerations on 22 cases with different etiology]
R Giacchino1, C Navone, B Ciravegna
1Clinica Malattie Infettive dell'Università, Istituto Giannina Gaslini, Genova, Italia.
Insights
Pediatric liver cirrhosis, though rare, is serious. Early diagnosis and identifying causes like HBV infection or Wilson's disease are crucial for effective treatment and improved outcomes in children.
Area of Science:
- Pediatrics
- Hepatology
- Internal Medicine
Background:
- Liver cirrhosis in children is uncommon but severe.
- Etiology is multifactorial, often leading to rapid fatality.
- This study reviews 22 pediatric cases over 15 years.
Purpose of the Study:
- To analyze the clinical characteristics, etiology, and outcomes of pediatric liver cirrhosis.
- To emphasize the importance of early diagnosis and etiological definition for treatment.
Main Methods:
- Retrospective review of 22 pediatric patients diagnosed with liver cirrhosis.
- Analysis of predisposing conditions, clinical presentation, diagnostic methods (liver biopsy), and follow-up data.
- Evaluation of treatment responses and patient outcomes.
Main Results:
- Common etiologies included Hepatitis B Virus (HBV) infection, Wilson's disease, and perinatal Cytomegalovirus (CMV) infection.
- Mean age at diagnosis was 6 years 8 months; 10 cases lacked prior chronic liver disease history.
- Hepatomegaly was universal; 13 patients had signs of hepatic failure. 8 out of 22 patients died during follow-up.
Conclusions:
- Early diagnosis and etiological clarification are vital for managing pediatric liver cirrhosis.
- Timely intervention can potentially alter the disease course and improve patient prognosis.
- Further research into specific pediatric liver cirrhosis subtypes is warranted.
Abstract:
Although rather uncommon and multifactorial in etiology, liver cirrhosis is a severe and often rapidly fatal disease in pediatrics. In our institution, during the last 15 years, 22 children with liver cirrhosis have been followed. The underlying predisposing condition was HBV infection (8 cases), CMV perinatal infection (2 cases), Wilson's disease (4 cases), chronic cholestasis (2 cases) and alcohol abuse (2 cases); in 4 cases no predisposing condition was evident. In all cases the histological examination of the liver was the diagnostic cornerstone. The mean age at diagnosis was 6 years and 8 months, with an early onset especially in the posthepatitis cirrhosis. In 10 out of 22 patients, cirrhosis was not preceded by an history of chronic liver disease. Poor subjective symptomatology was present in 13 of the cases, hepatomegaly in all, splenomegaly in 18 cases, signs of hepatic failure in 13 cases. In all patients various impairments of hepatocellular synthesis were detectable, especially during the period preceding the development of hepatic insufficiency. The mean time to cirrhosis was 5 years. The average duration of the follow up was 3 years and 4 months: during the follow up 6 patients improved, 5 patients showed no clinical or functional modifications of their hepatic disease, 3 patients worsened and 8 died. In order to perform suitable treatment of liver cirrhosis the need of early diagnosis and etiological definition should be emphasized.