Korean J Pediatr Gastroenterol Nutr.  2001 Apr;4(1):120-124.

A Case of Secondary Sclerosing Cholangitis in Langerhans Cell Histiocytosis

• Kim JH
• Choi BH
• Kim KM
• Moon HN

Abstract

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.

Keyword

Secondary sclerosing cholangitis; Langerhans cell histiocytosis

MeSH Terms

Bile Ducts
Child
Child, Preschool
Cholangitis
Cholangitis, Sclerosing*
Cholestasis
Cystic Fibrosis
Esophageal and Gastric Varices
Fibrosis
Histiocytosis, Langerhans-Cell*
Humans
Hypertension, Portal
Inflammation
Liver Cirrhosis, Biliary
Liver Diseases
Liver Failure
Liver Transplantation
Male
Prognosis