Hashida Y, Yunis E J
Children's Hospital of Pittsburgh, University of Pittsburgh, Pennsylvania.
Pediatr Pathol. 1988;8(1):1-15. doi: 10.3109/15513818809022275.
Morphologic findings of the liver in syndromatic paucity of intrahepatic bile ducts (SPIHBD) during infancy include paucity of interlobular bile ducts, features of "giant cell hepatitis," dilated lymphatics and veins in the portal tract, perisinusoidal fibrosis, and bile duct epithelial changes with a concentric layering of mesenchymal cells around bile ducts reminiscent of renal dysplasia. The latter change is characteristic of SPIHBD. Although the disease is characterized by paucity of bile ducts, morphometric studies show paucity of interlobular bile ducts in less than half of the patients during infancy. Reduced numbers of portal tracts and increased percentage of portal tracts devoid of bile ducts are more constant findings. It was impossible to predict from the early biopsy which patients would develop more severe portal fibrosis. Later in the disease portal fibrosis is variable and unevenly distributed, being more severe near the hilum regardless of the prior performance of a Kasai-type operation or the state of patency of the extrahepatic bile ducts. Hypoplasia of the extrahepatic bile ducts is the usual finding in SPIHBD, but if atresia of extrahepatic bile ducts is associated with intrahepatic paucity of bile ducts, the hepatic histopathology is that of PIHBD. Recognition of PIHBD would avoid unwarranted surgical procedures.
婴儿期综合征性肝内胆管减少症（SPIHBD）的肝脏形态学表现包括小叶间胆管减少、“巨细胞肝炎”特征、门静脉区淋巴管和静脉扩张、窦周纤维化以及胆管上皮改变，胆管周围有间充质细胞同心层状排列，类似于肾发育异常。后一种改变是SPIHBD的特征。尽管该疾病以胆管减少为特征，但形态计量学研究显示，婴儿期不到一半的患者小叶间胆管减少。门静脉数量减少以及无胆管门静脉的百分比增加是更常见的表现。早期活检无法预测哪些患者会发展为更严重的门静脉纤维化。在疾病后期，门静脉纤维化程度不一且分布不均，无论之前是否进行过Kasai型手术或肝外胆管是否通畅，在肝门附近更为严重。肝外胆管发育不全是SPIHBD的常见表现，但如果肝外胆管闭锁与肝内胆管减少相关，则肝脏组织病理学表现为PIHBD。认识PIHBD可避免不必要的手术。
