Clinical characteristics of primary biliary cirrhosis based on the analysis of 16 patients.
- Author:
Dong Il PARK
1
;
Kwang Cheol KOH
;
Jun Hyek LEE
;
Moon Seok CHOI
;
Tae Wook KANG
;
Won MOON
;
Min Kyoo RYU
;
Yong Wook LEE
;
Jee Eun KIM
;
Jae Geun HYUN
;
Jae Kwon JANG
;
Seung Woon PAIK
;
Jong Chul RHEE
;
Kyoo Wan CHOI
;
Cheol Geun PARK
Author Information
1. Department of Internal Medicine, Samsung Medical Center, Sungkyunkwan University School of Medicine, Seoul, Korea.
- Publication Type:Original Article
- Keywords:
Hemolytic uremic syndrome;
Microinfarcts;
Pancreatitis
- MeSH:
Antibodies, Antinuclear;
Fatigue;
Female;
Hemolytic-Uremic Syndrome;
Humans;
Jaundice;
Korea;
Liver;
Liver Cirrhosis, Biliary*;
Liver Diseases;
Male;
Melena;
Pancreatitis;
Prospective Studies;
Pruritus;
Rheumatoid Factor;
Xerophthalmia;
Xerostomia
- From:Korean Journal of Medicine
2000;59(6):634-640
- CountryRepublic of Korea
- Language:Korean
-
Abstract:
BACKGROUND: Primary biliary cirrhosis (PBC) is a chronic cholestatic liver disease of unknown etiology. The disease is relatively common in western countries, but so far, only about 20 cases have been reported in Korea. Thus, the purpose of our prospective study was to evaluate the clinical characteristics of PBC in Korea. METHODS: Between October 1994 and February 1999, 16 patients diagnosed as PBC at our department were enrolled in this study. We analyzed these patients for the distribution of age and sex, initial symptoms and signs, associated disorders, laboratory, endoscopic, and radiologic and histologic findings. RESULTS: Of the 16 patients, the ratio of male to female was 1:7 and the average age was 57.5 years. Pruritus (37.5%) was the most frequent presenting symptom followed by xerostomia and xerophthalmia (12.5%), jaundice (6.3%), chronic fatigue (6.3%), melena (6.3%). Associated disorders were Sj gren's syndrome (25%), arthropathy (12.5%), Raynaud's phenomenon (6.3%) and diabetes (6.3%). Abnormalities of liver function were found frequently. Antimitochondrial antibody (100%), antinuclear antibody (31.25%) and rheumatoid factor (31.25%) were found. Long-term administration of UDCA improved both clinical and biochemical signs in most patients, however, 2 patients experienced exacerbation during UDCA therapy. CONCLUSIONS: We experienced 16 cases of PBC within a short period. It is expected that we can find these patients more frequently if we suspect this disease in patients with pruritus and unexplained obstructive pattern of liver function abnormality.
