Variceal Bleeding in an Adolescent With HIV Diagnosed With Hepatoportal Sclerosis and Nodular Regenerative Hyperplasia

Abstract

340 due to elevated portal cirrhotic and noncirrh V ariceal bleeding venous pressure may result from both otic processes. Etiologies of portal hypertension in the absence of cirrhosis include portal vein thrombosis, nodular regenerative hyperplasia (NRH), hepatoportal sclerosis (HPS), incomplete septal cirrhosis, schistosomiasis, and vasculitides (1). HPS is also known as Banti syndrome (2), idiopathic portal hypertension, and noncirrhotic portal fibrosis. Characteristic histological findings are narrowed and/or obliterated portal veins, often with dilated periportal megasinusoids (1,3). Patients with HPS often present with variceal bleeding, splenomegaly, thrombocytopenia, and only mildly abnormal liver function tests (1,3). NRH is often the result of disturbed portal venous circulation and is characterized histologically by multiple small nodules composed of hyperplastic hepatocytes often arranged in cords more than 2 cells thick, typically surrounding portal regions. Hepatocytes located between the nodules are frequently atrophic and compressed but without significant fibrosis (4). NRH may be associated with medications (6-thioguanine, highly active antiretroviral therapy [HAART] with nucleoside reverse transcriptase inhibitors) and viral infections, including hepatitis C and HIV (1,4). NRH may also be seen in the setting of HPS and recently 4 adults with HIV on HAART with noncirrhotic portal hypertension were found to have HPS (1). We describe the first pediatric case of a 15-year-old boy with HIV on HAART with HPS and NRH.