Nodular regenerative hyperplasia of the liver. A review of 14 cases

Background/Aims: Nodular regenerative hyperplasia of the liver, is a noncir rhotic liver disease, characterized by nodules in the hepatic parenchyma, w hich clinically presents primarily with manifestations of portal hypertensi on. The aims of this study are i) to review the clinical, histological and diagnostic aspects of 14 documented cases of NRHL, and ii) to assess the ev olution and management of this condition in the cases reviewed. Methodology: The diagnosis of nodular regenerative hyperplasia of the liver was based on liver biopsy in all cases. Imaging studies (ultrasonography, computed tomography scan and magnetic resonance imaging scan) were performe d as part of the diagnostic evaluation. Clinical manifestations and biochem ical tests were recorded at the time of diagnosis. Management and prognosis were also reviewed. Results: The most common clinical manifestations were those of portal hyper tension, namely splenomegaly, esophageal varices and variceal bleeding. The histological findings were nodules in the hepatic parenchyma, the typical histologic feature of nodular regenerative hyperplasia of the liver, with m ild periportal fibrosis and intraportal lymphocytic infiltration. Biochemic al tests showed normal synthetic liver function, as evidenced by normal ser um albumin, bilirubin and prothrombin time. Elevation of gamma -glutamyl tr anspeptidase and alkaline phosphatase due to cholestasis was noted. Managem ent was directed to portal hypertension and variceal bleeding, with beta-bl ockers, sclerotherapy, mesenteric-caval shunt and transjugular intrahepatic portosystemic shunt with satisfactory results. Conclusions: Nodular regenerative hyperplasia of the liver is an uncommon c ondition but it should be considered in patients with unexplained portal hy pertension and distinguished from liver cirrhosis, in view of the differenc es in the natural history and prognosis. Liver biopsy confirms the diagnosi s. Management is directed primarily to portal hypertension and variceal ble eding, which is the main source of mortality. Liver failure is uncommon due to satisfactory preservation of liver function.