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KMID : 0816120010040010120
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Korean Journal of Pediatric Gastroenterolology and Nutrition
2001 Volume.4 No. 1 p.120 ~ p.124
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A Case of Secondary Sclerosing Cholangitis in Langerhans Cell Histiocytosis
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Kim Ja-Hyeong
Choi Bo-Hwa
Kim Kyung-Mo
Moon Hyung-Nam
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Abstract
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Sclerosing cholangitis is a chronic cholestatic disease characterized by inflammation and obliterative fibrosis of the bile ducts, leading to biliary cirrhosis and ultimately to liver failure. In children, sclerosing cholangitis is known to be
associated with Langerhans cell histiocytosis, as well as with congenital immunodeficiencies and cystic fibrosis. Secondary sclerosing cholangitis is suspected in Langerhans cell histiocytosis with chronic cholestasis, liver dysfunction and portal hypertension. Unlike primary sclerosing cholangitis, the cholangitis associated Langerhans cell histiocytosis is destructive in nature and progresses more rapidly to biliary cirrhosis, therefore uniformly the prognosis is poor. In this setting, liver transplantation should be considered early in children with sclerosing cholangitis complicating Langerhans cell histiocytosis before end-stage liver failure and variceal bleeding. We experienced a case of secondary sclerosing cholangitis in Langerhans cell histiocytosis in a 2-year-old boy. We report this case with brief review of the related literatures.
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KEYWORD
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Secondary sclerosing cholangitis, Langerhans cell histiocytosis
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