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Updated: Dec 12, 2025

Using Multi-fluorinated Bile Acids and In Vivo Magnetic Resonance Imaging to Measure Bile Acid Transport
Published on: November 27, 2016
[A case of fibrinogen storage disease with primary biliary cholangitis]
Hiroko Tsuchiya1, Kyoko Hoshikawa1, Taketo Nishina1
1Department of Gastroenterology, Faculty of Medicine, Yamagata University.
Abstract:
A 66-year-old woman was diagnosed as primary biliary cholangitis (PBC) and was previously hospitalized for ascites and jaundice. She came to our hospital for further examination of the liver by needle biopsy, which showed interface hepatitis that mainly comprised lymphocytes and inflammatory infiltrates in the bile duct in the portal area. On the other hand, numerous intracytoplasmic inclusions that were positive for fibrinogen immunostaining were seen in the lobular area. Finally, we histologically diagnosed as PBC with fibrinogen storage disease (FSD). FSD is rare disease that leads to liver damage caused by abnormal fibrinogen storage in the endoplasmic reticulum of hepatocytes, with only four cases reported in Japan until now.
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