랑게르한스세포 조직구증 환아에서 발생한 속발성 경화성 담관염 1례

A Case of Secondary Sclerosing Cholangitis in Langerhans Cell Histiocytosis

저자들은 복부 팽만과 지속적인 황달을 주소로 내원한 랑게르한스세포 조직구증 환아에서 간조직 검사 및 내시경적 역행성 담도조영술로 진단한 속발성 경화성 담관염 1례를 경험하였기에 문헌 고찰과 함께 보고하는 바이다.

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.