Congenital Hepatic Fibrosis in Children: A Report of Three Cases

孩童先天性肝纖化症：三病例報告

翁哲寬(Che-Kuan Weng) ； 張美惠(Mei-Hwei Chang) ； 陳志榮(Chi-Long Chen)

《Acta Paediatrica Sinica》 29卷1期 (1988/02) Pp. 40-48

摘要

本文提出三例經病理組織切片證實爲先天性肝纖維化症的孩童。第一例是一位12歲的男孩，臨床上表現出因肝門脈高血壓所引起的嘔血症狀。第二例是一位11歲的男孩，臨床上的表現和第一個病例相似。第三例是一位4歲的男孩，臨床上的表現是由於膽管炎所引起的反覆性發燒。雖然沒有嘔血的症狀，但如同前面兩例，可見到食道靜脈曲張的現象及由病理組織切片所見證明有肝纖維化現象。因此在有肝門脈高血壓症狀或徵象的孩童應懷疑是否有先天性肝纖維化症，而且先天性肝纖維化症也可以表現出反覆性的發燒而沒有嘔血的症狀。

並列摘要

Three pediatric patients with congenital hapatic fibrosis histologically proven were presented. The first patient was a 12-year-old boy who presented with hematemesis due to portal hypertension. The second patient was a 11-year-old boy who had similar clinical manifestations as in the first case. The third patient was a 4-year-old boy who presented with recurrent fever due to cholangitis. Although no hematemesis occurred, esophageal varices was also present as in the first two cases. It suggested that congenital hepatic fibrosis should be suspected in patients with symptoms or signs of portal hypertension; but could also masquerade in as recurrent fever without hematemesis.

並列關鍵字

congenital hepatic fibrosis ； portal hypertension ； cholangitis ； polycystic kidneys