The Indonesian Journal of Gastroenterology, Hepatology, and Digestive Endoscopy 60 C ASE REPO RT Non Cirrhotic Portal Fibrosis Ralph Girson *, Andri Sanityoso **, Rino A Gani ** Wirasmi Marwoto ***, Murdani Abdullah****, Ari F Syam **** *Department of Internal medicine, Faculty of medicine University of Indonesia/ Dr. Cipto Mangunkusumo General National Hospital ** Division of Hepatology, Department of Internal Medicine, Faculty of Medicine, University of Indonesia/Dr. Cipto Mangunkusumo General National Hospital ***Department of Pathology Anatomy, Faculty of Medicine, University of Indonesia/Dr. Cipto Mangunkusumo General National Hospital **** Division of Gastroenterology, Department of Internal Medicine, Faculty of Medicine University of Indonesia/Dr. Cipto Mangunkusumo General National Hospital ABSTRACT Diagnosis of non cirrhotic portal fibrosis was considered when the following criteria were fulfilled evidence of portal hypertension (oesophageal varices, hypersplenism, ascites, or increased hepatic venous pressure gradient), Doppler ultrasound showing patent portal and hepatic veins, and liver biopsy showing sign of cirrhosis. Non cirrhotic portal fibrosis clinically characterized by splenomegaly, anemia, portal hypertension, and histopathological examination portal tract showing fibrosis and sclerosis. Portal hypertension are most caused by a cirrhotic liver (85%), there are only a few reports on non cirrhotic portal hypertension, mostly in Japan and India. We reported a case of non cirrhotic portal fibrosis in young male. The clinical complications of portal hypertension are variceal bleeding and pancytopenia due to hypersplenism. Variceal band ligation and splenectomy were performed. The patient showed good clinical response. Keywords: portal hypertension, non cirrhotic portal fibrosis, young male INTRODUCTION The standard of normal portal venous pressure is 5 - 10 mmHg. Portal hypertension is defined as pressure that exceeds 10 mmHg. 1-4 Cirrhosis is the most common cause of portal hypertension in the United States (85%). Other causes of portal hypertension are portal vein obstruction, infection, pancreatitis, abdominal trauma, portal vein thrombosis or idiopathic. Idiopathic portal vein thrombosis may develop in a variety of hypercoagulable states, including polycthemia vera, essential trombocythemia, deficiency of protein C, protein S or antithrombin III. 3 Hepatic vein thrombosis and hepatic venoocclusive disease are relatively infrequent cause of portal hypertension. Non cirrhotic portal fibrosis accounts for only a few cases of portal hypertension. There are reports on non-cirrhotic portal hypertension in India and Japan, and in Indonesia it is presumed to be less than 5%. 2, 3 Causes of portal hypertension can be classified as increased resistance to flow and increase portal blood flow. Increasing resistance can cause by at pre-hepatic obstruction (portal vein obstruction), intrahepatic obstruction, and posthepatic obstruction. Non cirrhotic fibrosis of the liver can be distinguished from cirrhosis by the absence of hepatocellular damage and lack of nodular regenerative activity, but there are still manifestations of portal hypertension. There are three variants of Idiopathic Portal Hypertension or Non Cirrhotic Portal Fibrosis (NCPF) as follows: Diagnosis of NCPF was considered when the following criteria were fulfilled: evidence of portal hypertension (oesophageal varices, hypersplenism, ascites, or increased hepatic venous pressure gradient). Doppler ultrasound showing patent portal and hepatic veins, liver biopsy showing no cirrhosis. 6 Non cirrhotic portal fibrosis (NCPF) clinically characterized by