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Congenital Hepatic Fibrosis: A Case Report
先天性肝纖維化：病例報告
摘要
由於臺灣地區慢性B型肝炎合併肝硬化是造成門靜脈高壓及其併發症最常見的原因。因此不論病患是屬於何種年齡層，肝硬化常被視為門靜脈高壓合併食道靜脈曲張出血的當然原因。使少數原本可以用更積極方法延長生命的病例因而延誤治療時機。本文所報告的病例為一位22歲年輕男子因吐血入院。患者無C型肝炎病史亦非B型肝炎帶原者。理學檢查顯示肝脾腫大；檢驗發現除血小板數目偏低及血清鹼性磷酸酶值過高外，肝功能指數，藍胞漿素（ceruolplasmin）及抗核抗體（antinuclear artibody）皆正常。腹部超音波、電腦斷層以及上胃腸道泛內視鏡檢證實病患具肝脾腫大合併門靜脈高壓現象。為達診斷目的，病人接受超音波指引下之肝臟活體切片，經病理證實為罕見的先天性肝纖維化，且無合併肝內、外或腎臟囊腫。由於臨床上病人對食道靜脈出血耐受性極佳與一般肝硬化的出血現象大異其趣，故提出報告。更希望由此突顯肝臟活體切片對不明原因肝臟疾病診斷的必要性。
並列摘要
Hepatitis B infection with liver cirrhosis, an endemic disease in Taiwan, has been implicated as the most common cause of portal hypertension. Irregardless of the age group, young or old alike, most postnecrotic liver cirrhosis had been considered as the leading cause of portal hypertension with gastroesophageal variceal bleeding. For this reason, these groups of patients had been incorrectly managed with conservative means, when in fact, they could have benefited from a more aggressive treatment such as a shunt operation or liver transplantation, thereby giving them a better chance at survival. We hereby report a case of a 22-year-old man who presented with hematemesis, thrombocytopenia, and markedly elevated alkaline phosphatase, despite normal liver function tests, without a previous history of hepatitis B nor C. Further investigation showed hepatosplenomegaly with portal hypertension, well-tolerated variceal bleeding, and absence of hepatic or renal cysts. Clinical dilemma forced us to perform liver biopsy which revealed pure congenital hepatic fibrosis, a very rare cause of portal hypertension. This case emphasizes the importance of liver biopsy in the diagnosis of liver disease of unknown etiology.