An Unusual Case of Idiopathic Noncirrhotic Portal Hypertension Complicated by Hepatic Encephalopathy and Hepatocellular Carcinoma
Ahmed Lutful Moben
Department of Hepatology, Kurmitola General Hospital, Dhaka, Bangladesh
Rokshana Begum
Department of Hepatology, Shaheed Suhrawardy Medical College, Dhaka, Bangladesh.
Sheikh Mohammad Noor E Alam
Department of Hepatology, Bangladesh Medical University, Dhaka, Bangladesh.
Md. Abdur Rahim
Department of Hepatology, International Medical College, Gazipur, Bangladesh.
Bishnu Pada Dey
Department of Pathology, Bangladesh Medical University, Dhaka, Bangladesh.
Musarrat Mahtab
Clinical Research Organization Ltd., Dhaka, Bangladesh.
Sheikh Mohammad Fazle Akbar
Clinical Research Organization Ltd., Dhaka, Bangladesh., Department of Gastroenterology and Metabolism, Ehime University Graduate School of Medicine, Ehime, Japan., Research Center for Global and Local Infectious Diseases, Oita University, Yufu, Oita, Japan. and Miyakawa Memorial Research Foundation, Tokyo, Japan.
Mamun Al Mahtab *
Department of Hepatology, Bangladesh Medical University, Dhaka, Bangladesh.
*Author to whom correspondence should be addressed.
Abstract
Idiopathic Noncirrhotic Portal Hypertension (INCPH) is a rare disease entity which is characterized by intrahepatic portal hypertension in the absence of cirrhosis and other liver diseases and splanchnic venous thrombosis. Etiology of INCPH remains unknown and immunological disorders, chronic infections, exposure to medicines and toxins, genetic disorders and prothrombotic conditions have been implicated with the development of INCPH. The diagnosis is by exclusion and depends on clinical and histopathological features. Here we present a case of INCPH with rather unusual clinical course from Bangladesh. This young female presented with repeated bouts of hematemesis and melaena. She had features resembling decompensated liver cirrhosis and eventually developed hepatic encephalopathy and hepatocellular carcinoma.
Keywords: Idiopathic noncirrhotic portal hypertension, hepatic decompensation, hepatocellular carcinoma, Bangladesh