Donhuijsen K, Leder L D
Z Gastroenterol. 1978 Jun;16(6):387-94.
A case of congenital hepatic fibrosis (CHF) is described. CHF is characterized by hepatomegaly, portal hypertension, extensive portal fibrosis, ectatic bile ducts, and hypoplasia of terminal portal vein branches. In contrast to the severe portal hypertension liver function tests are largely normal. In our case the disease was first detected when the patient was 7 years old. During the following 9 1/2 years three sequential liver biopsies were performed. Each of them showed the same picture and no progression occurred. The characteristic histological picture of CHF includes mature bile ducts without epithelial proliferation, absence of significant intraportal or interlobular inflammatory infiltrates, and small or hypoplastic distal portal vein branches. On the basis of these features the disease can easily be separated from other forms of liver cirrhosis.
本文描述了一例先天性肝纤维化（CHF）病例。CHF的特征为肝肿大、门静脉高压、广泛的门静脉纤维化、胆管扩张以及终末门静脉分支发育不全。与严重门静脉高压形成对比的是，肝功能检查基本正常。在我们的病例中，该疾病首次被发现时患者7岁。在接下来的9年半时间里，相继进行了三次肝脏活检。每次活检结果均相同，且病情无进展。CHF的典型组织学表现包括成熟的胆管且无上皮细胞增殖、门静脉内或小叶间无明显炎性浸润以及小的或发育不全的远端门静脉分支。基于这些特征，该疾病可轻易与其他形式的肝硬化相鉴别。
