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원발성 담즙성 간경변증-자가면역성 간염의 중복증후군 1예
안상준,권병표,이상화,이태영,장진석,정진숙,한상영 대한내과학회 2004 대한내과학회지 Vol.66 No.2
저자들은 지속적으로 간기능 악화를 보이는 환자에서 간조직 검사를 통해 원발성 담증성 간경변증-자가면역성간염의 중복증후군 환자를 진단하였기에 문헌고찰과 함께 보고하는 바이다. Primary biliary cirrhosis and autoimmune hepatitis are the two main immune-mediated liver diseases. They are generally differentiated easily on the basis of clinical, biochemical, serological, and histological findings. Occasionally, the normally distinctive features overlap and classification of a given patient's chronic liver disease is difficult. Series of such patients with Primary biliary cirrhosis-autoimmune hepatitis overlap syndrome were reported. We report a case of primary biliary cirrhosis-autoimmune hepatitis overlap syndrome proven by clinicopathology. The patient showed hepatitis features including easy fatigability, icteric sclera. Markers of hepatitis B and C virus were absent. The liver biopsy showed chronic hepatitis with piecemeal necrosis, infiltration of inflammatory cell surrounding bile duct. The clinical data of high titers of antinuclear antibodies and antimitochondral antibodies, elevated serum IgM, suggest mixed features of primary biliary cirrhosis-autoimmune hepatitis. The patient as given only ursodeoxycholic acid (600 ㎎/day), then was observed follow-up regularly.
원발성 담즙성 간경변증의 임상적 특성과 UDCA 치료반응
김주영,김태헌,유권,한예지,최정은,김지윤,조민선 이화여자대학교 의과학연구소 2015 EMJ (Ewha medical journal) Vol.38 No.3
Objectives: Primary biliary cirrhosis (PBC) is a chronic cholestatic liver disease that may progress to end stage liver cirrhosis. Benefits of ursodeoxycholic acid (UDCA) treatment has been investigated through large clinical studies. However, most of the studies were done in western countries and recent increase in prevalence of this relatively uncommon chronic liver disease draws attention in Korea. As early UDCA treatment effectively prevent the grave consequences of PBC progression, early diagnosis and lifelong management with UDCA is important. This study was designed to investigate the clinical features of PBC and response rates of UDCA treatments in Ewha Womans University Medical Center. Methods: Clinical data of PBC patients diagnosed between 2001 and 2014 at Ewha Womans University Medical Center were analyzed retrospectively. Results: A total of 35 patients with mean follow-up duration of 42 months were enrolled. At the diagnosis, 72.7% of the patients were asymptomatic, 5.7% had decompensated liver cirrhosis. The mean serum alkaline phosphate (ALP) level was 2.65 times upper limit of normal . UDCA was prescribed in 91.4% of the patients (n=32), among which 77.4% exhibited biochemical responses defined as serum ALP less than 2 upper limit of normal at 6 months (Mayo criteria). Conclusion: Most PBC patients were asymptomatic at the time of diagnosis and the average biochemical responses rate to UDCA treatment were ranged from 60.0% to 78.9% according to various response criteria. To elucidate the clinical features and courses of Korean PBC patients in detail, larger scale investigations and longer clinical follow up studies are warranted.
이준수,홍상훈,김강산,박상은,Lee, Jun-su,Hong, Sang-hoon,Kim, Gang-san,Park, Sang-eun 대한한방내과학회 2016 大韓韓方內科學會誌 Vol.37 No.2
Objective: This study was designed to assess the clinical effect of traditional Korean medicine on primary biliary cirrhosis.Method: We provided herbal medicine and acupuncture therapy to a 38-year-old male patient who complained about jaundice, itching, and fatigue. During a six-day hospital stay, we checked any changes in symptoms and conducted liver function tests (such as aspartate aminotransferase [AST], alanine aminotransferase [ALT], alkaline phosphatase [ALP], and gamma-glutamyl transferase [GGT], among others) to evaluate any improvement.Results: We observed that herbal medicine and acupuncture therapy appeared to decrease the symptoms of primary biliary cirrhosis. During each hospital stay, the symptoms and liver function test both showed improvement, especially in ALP.Conclusion: Based on the therapeutic results of the six-day hospital stay, we report improved primary biliary cirrhosis symptoms and a decreasing tendency in ALP and GGP levels following treatment with traditional Korean medicine.
김평남 ( Pyoung Nam Kim ),김태환 ( Tae Hwan Kim ),김승영 ( Seung Young Kim ),전재범 ( Jae Bum Jun ),한동수 ( Dong Soo Han ),정성수 ( Sung Soo Jung ),이인홍 ( In Hong Lee ),배상철 ( Sang Cheol Bae ),유대현 ( Dae Hyun Yoo ),주경빈 ( 대한류마티스학회 1995 대한류마티스학회지 Vol.2 No.1
Primary biliary cirrhosis is a chronic, progressive liver disease characterized by inflammatory destruction of septal and intralobular bile ducts which results in intrahepatic cholestasis. Although the cause remains obscure, it is frequently associated with a variety of disorders presumed to be autoimmune in nature. We report a case of early primary biliary cirrhosis which was anteceded by rheumatoid arthritis. The patient was a 54-year-old female who was admitted due to arthralgia and joint deformity. She had been diagnosed as having rheumatoid arthritis 10 years before. On admission, she had elevated serum ALT, AST, alkaline phosphatase, gamma-GTP and positive serum antimitochondrial antibody test. Microscopic findings of the liver were consistent with the early stage of primary biliary cirrhosis.
Review : The diagnosis and treatment of primary biliary cirrhosis
( Kyung Ah Kim ),( Sook Hyang Jeong ) 대한간학회 2011 Clinical and Molecular Hepatology(대한간학회지) Vol.17 No.3
Primary biliary cirrhosis (PBC) is a slowly progressive cholestatic liver disease of autoimmune etiology. The initial presentation of PBC is various from asymptomatic, abnormal liver biochemical tests to overt cirrhosis. The diagnosis of PBC is based on cholestatic biochemical liver tests, presence of antimitochondrial antibody and histologic findings of nonsuppurative destructive cholangitis. Although the diagnosis is straightforward, it could be underdiagnosed because of its asymptomatic presentation, or underrecognition of the disease. UDCA in a dose of 13-15 mg/kg is the widely approved therapy which can improve the prognosis of patients with PBC. However, one-third of patients does not respond to UDCA therapy and may require liver transplantation. Every effort to diagnose PBC in earlier stage and to develop new therapeutic drugs and clinical trials should be made. (Korean J Hepatol 2011;17:173-179)
김호철,장윤식,이연재,이상혁,설상영,정정명,최하진,양영일 인제대학교 1996 仁濟醫學 Vol.17 No.2
원발성 담즙성 간경변증은 원인 미상의 담즙정체 질환으로 만성적인 간내 담관의 염증을 보이면서 간경변증으로 진행하는 드문 질환이다. 저자들은 황달과 소양증을 주소로 내원한 55세의 여자 환자에서 원발성 담즙성 간경변증 1례를 경험하였기에 문헌고찰과 함께 보고하는 바이다. Primary biliary cirrhosis is rare and a disease of unknown origin. It is a chronic cholestatic liver disease characterized by the presence of chronic inflammation, necrosis of hepatic bile duct and progression to liver crirhosis. We experienced a 55 year- old woman complained of jaundice and itching for 3 years. Her clinical symptoms, biochemistry, liver function test and liver biopsy were compatible with primary biliary cirrhosis. Thus we report a case of primary biliary cirrhosis with literature review.
오길찬 ( Kil Chan Oh ),박상훈 ( Sang Hoon Park ),임해성 ( Hae Sung Lim ),송진영 ( Jin Young Song ),박철희 ( Chul Hee Park ),한태호 ( Tae Ho Hahn ),박현주 ( Hyun Ju Park ),이동근 ( Dong Keun Lee ),김종혁 ( Jong Hyeok Kim ),장웅기 ( 대한소화기학회 2003 대한소화기학회지 Vol.41 No.2
Pregnancy in patients with primary biliary cirrhosis (PBC) is uncommon and only a few cases have been reported in the world literature. Thus, there is no report on the outcome and natural history of pregnancy in patients with primary biliary cirrhosis. He