Nonomura A, Ohta G, Yoshida K, Kurachi M, Matsubara F, Takazakura E
Acta Pathol Jpn. 1978 Nov;28(6):949-56.
A patient who developed pancytopenia and hepatosplenomegaly with portal hypertension is described. Liver wedge biopsy at the time of operation showed typical histology of congenital hepatic fibrosis. Renal surgical biopsy revealed tubular ectasia. A continuous anastomosing network of the biliary tree was observed by reconstruction of serial sections of the liver. Some parts of the network were dilated and no isolated bile ducts were present.
描述了一名出现全血细胞减少、肝脾肿大伴门静脉高压的患者。手术时肝脏楔形活检显示为先天性肝纤维化的典型组织学表现。肾脏手术活检显示肾小管扩张。通过肝脏连续切片重建观察到胆管树有连续的吻合网络。该网络的一些部分扩张，未见孤立的胆管。
