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Sclerosing cholangitis in children
D Debray1, D Pariente, E Urvoas
1Service d'Hépatologie Pédiatrique, Hôpital de Bicêtre, Le Kremlin-Bicêtre, France.
Insights
Sclerosing cholangitis (SC) in children often presents with jaundice and liver enlargement. Prognosis is poor, but liver transplantation offers a chance for survival, especially when SC is not linked to severe immunodeficiency.
Area of Science:
- Pediatric Gastroenterology
- Hepatology
- Immunology
Background:
- Sclerosing cholangitis (SC) is a rare, chronic cholestatic liver disease affecting children.
- Early diagnosis and understanding of associated conditions are crucial for management.
Purpose of the Study:
- To describe the clinical characteristics, histopathology, and outcomes of pediatric sclerosing cholangitis.
- To evaluate the role of liver transplantation in managing severe cases.
Main Methods:
- Retrospective analysis of 56 children diagnosed with SC between 1972 and 1992.
- Clinical data, laboratory findings, histopathology, cholangiography, and treatment outcomes were reviewed.
Main Results:
- SC presented with neonatal cholestasis or later onset associated with conditions like histiocytosis X or immunodeficiency.
- Most children developed biliary cirrhosis; median survival was 10 years.
- Liver transplantation improved survival in 11 of 15 recipients.
Conclusions:
- SC should be suspected in children with chronic cholestatic disease and elevated gamma-glutamyltransferase.
- Prognosis is generally poor, but liver transplantation is a viable option for select patients.
Abstract:
We report on 56 children with sclerosing cholangitis (SC) seen between 1972 and 1992. The first symptoms occurred at a mean age of 3.7 years; 15 infants had neonatal cholestatic jaundice. At diagnosis, cholestatic jaundice was present in 25 children, hepatomegaly in 54, splenomegaly in 41, and ascites in 12. Serum alkaline phosphatase activity was increased in 49 patients and gamma-glutamyltransferase activity in all patients tested. Most often the histopathologic findings were extensive portal fibrosis and neoductular proliferation. Cholangiography showed abnormal intrahepatic bile ducts in all children and abnormal extrahepatic bile ducts in 35 (63%). The children were separated into three groups: (1) those with SC of neonatal onset (27%); (2) those with SC of postneonatal onset associated with another disease (55%)--histiocytosis X in 14 children, immunodeficiency syndromes in 8, chronic inflammatory bowel disease or autoimmune hepatitis in 8, and congenital psoriasis in 1; and (3) those with SC of postneonatal onset without an associated disease (18%). Biliary cirrhosis was present in all but three children after 6 months to 19.3 years of follow-up. Eleven children died of portal hypertension or liver failure, and six died of a complication related to the associated disease. Fifteen children had liver transplantation; 11 of these are alive 6 months to 6 1/2 years later without recurrence of SC. The overall estimated median survival time of children with SC was 10 years from clinical onset. These results indicate that SC should be suspected in all children with a chronic cholestatic disease and increased serum gamma-glutamyl transferase activity, especially when diseases known to be associated with SC are present. The prognosis is poor, but liver transplantation should be considered except in those with severe immunodeficiency syndromes.