Idiopathic adulthood ductopenia |
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| Authors: | ISAO NAKANO YOSHIHIDE FUKUDA YASUO KOYAMA FUMIHIRO URANO MASAHIKO YAMADA YOSHIAKI KATANO AKIKO MARUI KAZUMI IMADA TETSUO HAYAKAWA MASAFUMI ITO YORIKO YAMASHITA MASAMI IMOTO YASUNI NAKANUMA |
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| Affiliation: | *Second Department of Internal Medicine, School of Medicine, Kanazawa University, Kanazawa, Japan;†First Department of Pathology, Nagoya University School of Medicine, Nagoya, School of Medicine, Kanazawa University, Kanazawa, Japan;‡Department of Internal Medicine, Fujita Health University School of Medicine, Toyoake, School of Medicine, Kanazawa University, Kanazawa, Japan;§Second Department of Pathology, School of Medicine, Kanazawa University, Kanazawa, Japan |
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| Abstract: | In 1981, a 26 year old man occasionally demonstrated elevated serum transaminase concentrations. He had no history of medication, or a personal or family history of jaundice, except for prolonged physiological jaundice as a neonate. Serum hepatitis B surface antigen, hepatitis C virus antibody and anti-mitochondrial antibody were absent. A wedge biopsy specimen revealed ductular proliferation, mild inflammation of the portal area and disappearance of bile ducts from 80% of the portal tracts. Serial sections demonstrated a vanishing bile duct. Endoscopic retrograde choledo-chopancreatography, portography and arteriography demonstrated no abnormalities. In 1994, the patient died of hepatic failure following a 12 year observation period. He was subsequently diagnosed with idiopathic adulthood ductopenia on the basis of the criteria proposed by Ludwig. |
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| Keywords: | Key words cholestasis ductular proliferation idiopathic adulthood ductopenia intrahepatic bile ducts primary biliary cirrhosis primary sclerosing cholangitis vanishing bile duct syndrome |
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