Caroli’s disease in two siblings
نویسندگان
چکیده
We report two cases of Caroli’s disease in a pair of siblings. The first patient, a seven year-old female child, presented with right upper quadrant abdominal pain for one year. Physical examination showed abdominal bulging and hepatosplenomegaly. Laboratory investigations revealed liver function damage. Abdominal ultrasonography and computer tomography showed intrahepatic bile duct dilatation and increased wall thickness. Liver biopsy demonstrated intrahepatic bile duct distension and proliferation, severe portal tracts fibrosis and inflammatory cell infiltration. She was diagnosed with Caroli’s disease. The patient’s 3 year-old brother also presented with similar symptoms, and his laboratory tests, imaging studies and pathological findings were also consistent with the diagnosis of Caroli’s disease. The parents of the siblings showed no abnormalities.
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A Rare Congenital Liver Disorder with Varied Clinical Presentations –report of Two Cases
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