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원발성 경화성 담관염 : 증례보고 및 국내문헌 고찰

Other Titles
 Primary Sclerosing Cholangitis : Report of a Case with a Clinical Analysis of the Cases Reported in the Korean Literature 
 서정건  ;  정준표  ;  조현근  ;  이귀순  ;  이관식  ;  전재윤  ;  강진경  ;  박인서  ;  김기황  ;  김호근  ;  이상인 
 Korean Journal of Medicine (대한내과학회지), Vol.53(1) : 93-101, 1997 
Journal Title
 Korean Journal of Medicine (대한내과학회지) 
Issue Date
We experienced a case of primary sclerosing cholangitis(PSC) in a 40-year-old female who complained of jaundice and pruritus. Marked elevation of serum alkaline phophatase level, typical beaded appearance and pruned-tree appearance on endoscopic retrograde cholangiography, together with a finding of chronic obliterative fibrosing cholangitis on sono-guided gun biopsy specimen of the liver led to a confirmative diagnosis of PSC. The patient responded to ursodeoxycholic acid (UDCA), but was reluctant to treatment and died of hepatic failure 7 months later. PSC is a very rare disease in Korea. So far, only 5 cases including our present case have been reported in the Korean literature. Male-to-female ratio was 2:3 and the median age was 40(27-80 years old). Ulcerative colitis was associated in one case. Four cases involved both intra, and extrahepatic bile ducts and one case was reported to be confined in the intrahepatic bile ducts. Fatality was in 3 cases, 20 days, 36 days, and 7 months after the initial presentaion, respectively. The causes of death were acute cholangitis and sepsis in two, and hepatic failure in one. We herein report a case of PSC and clinical charateristics of the reported cases in Korea, and review the literature with an emphasis on UDCA treatment in PSC.
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1. College of Medicine (의과대학) > Dept. of Pathology (병리학교실) > 1. Journal Papers
1. College of Medicine (의과대학) > Dept. of Internal Medicine (내과학교실) > 1. Journal Papers
Yonsei Authors
Kim, Hogeun(김호근)
Lee, Kwan Sik(이관식) ORCID logo https://orcid.org/0000-0002-3672-1198
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