关键词: 先天性肝纤维化, 肝硬化, 肾囊肿, 上消化道出血, 门静脉高压

Abstract: Objective: To analyze the clinical characteristics,the typical pathological features and treatment methods of the patients with congenital hepatic fibrosis (CHF),and to improve the prognosis of the patients. Methods: The clinical data of a patient with CHF underwent liver transplantation for liver function failure were collected,and the relevant literatures were reviewed; the classification, clinical characteristics, diagnosis and treatment of CHF were analyzed. Results: The patient was a 39-year-old man and prensented with upper gastrointestinal bleeding. The results of gastroscope examination showed the severe varicose veins of the esophagus,which was marked by portal hypertension. The imaging examination after admission showed liver cirrhosis, splenomegaly and right renal cyst; the patient had mild liver function change, poor coagulation function, and liver function failure. After medical treatment, the condition was not improved, and the patient underwent allogeneic orthotopic liver transplantation. The postoperative liver pathology indicated CHF; all the laboratory indicators were normal after 1 month of transplantation. The patient recovered better 1 year after operation. Conclusion: CHF is characterized by liver fibrosis, portal hypertension and renal cystic lesions. Liver biopsy is the gold standard for its diagnosis, clinical treatment is mainly for the complications, and liver transplantation is the fundamental treatment.

Key words: congential hepatic fibrosis, liver cirrhosis, renal cyst, upper gastrointestinal bleeding, portal hypertension