由Kerr等于1961年报道一种在本质上不同于肝硬化的先天性肝纤维性变(简称CHF)以来,大约已有100例报道。本病可伴有肾囊肿和肺气肿,但直到5岁或5岁后,当门脉高压导致胃肠道出血时,才出现肝损症状。新生儿的CHF常继发于婴儿多囊性疾病,因尿毒症死亡后经尸检发现。本文首次报道一例婴儿表现为多发性先天性异常及先天性肝纤维性变引起的顽固性腹水。病例报告:新 About 100 cases have been reported by Kerr et al. In 1961 for a report of a congenital hepatic fibrosis (CHF) that is essentially different from cirrhosis. The disease can be associated with renal cysts and emphysema, but until 5 years of age or 5 years of age, when portal hypertension causes gastrointestinal bleeding occurs only when the liver damage symptoms. Infants often newborns CHF secondary to polycystic disease of the baby, died of uremia by autopsy found. This article first reported a case of infants with multiple congenital abnormalities and congenital liver fibrosis caused by refractory ascites. Case Report: New