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Korean J Pancreas Biliary Tract > Volume 2(1):1997 > Article
The Korean Journal of Pancreas and Biliary Tract 1997;2(1):51-59.
원발성 경화성 담관염 3예
조주영1, 함정식1, 장재영1, 김연수1, 이준성1, 이문성1, 황성규1, 심찬섭1, 김동원2, 이동화2
1순천향대학교 의과대학 내과학교실, 소화기연구소
2순천향대학교 의과대학 해부병리과
Cases Reports of Primary ScIerosing Cholangitis
Joo Young Cho1, Jeong Sik Ham1, Yun Soo Kim1, Jung Sung Lee1, Moon Sung Lee1, Seong Gyu Hwang1, Chan Sup Shim1, Dong Won Kim2, Dong Wha Lee2
1Institute for Digestive Research, Department of Internal Medicine, College of Medicine, Soon Chun Hyang University, Seoul, Korea
2Department of Pathology, College of Medicine, Soon Chun Hyang University, Seoul, Korea
Corresponding author:  Chan Sup Shim,
ABSTRACT
Primary sclerosing cholangitis is a disease in which the bile ducts inside and outside the liver become narrowed due to inflammation and scarring. This causes bile to accumulate in the liver and can result in damage to liver cells. Although the exact cause of primary sclerosing cholangitits is unknown, genetic and immunologic factors appear to play a role. Primary sclerosing cholangitis has been considered a rare disease, but recent studies suggest that it is more common than previously throught. It may occur alone, but approximatedly 70% of patients have associated inflammatory bowel disease, particularly ulcerative colitis. Initially, many individuals have no symptoms and the disease is detected because of abnormal laboratory test results, an enzyme test called alkaline phosphatase. Criteria for diagnosis include: 1) absence of previous biliary surgery 2) abnormal serum alkaline phosphatase level 3) extrahepatic and intrahepatic bile ducts become diffuse or focal irregular narrowed by cholangiogram 4) pathologic finding shows obstructive and fibrous cholangitis. The course of the disease is unpredictabe for the patient, but is generally slowly progressive. Liver failure may occur after 7-15 years of disease or even longer. Approximated 10% of patients who have the disease on a longstanding basis may develop a superimposed tumor of the bile ducts called cholangiocarcinoma. There is currently no specific treatment for primary sclerosing cholangitis. In some instances, endoscopic, radiologic, or surgical techiques may be employed to open major blockages in the common bile duct and improve bile flow. When progressive liver failure occurs in spite of these measures, liver transplantation may be indicated. We report 3 cases of primary sclerosing cholangitis which were diagonised by endoscopic retrograde cholangiography and pathological finding.
Keywords: Primary sclerosing cholangitis, ERCP
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