Open AccessNodular regenerative hyperplasia related portal hypertension in a patient with hypogammaglobulinaemia
Author(s) -
Barun Kumar Lal,
Adrian J. Stanley
Publication year - 2013
Publication title -
world journal of gastroenterology
Language(s) - English
Resource type - Journals
SCImago Journal Rank - 1.427
H-Index - 155
eISSN - 2219-2840
pISSN - 1007-9327
DOI - 10.3748/wjg.v19.i22.3502
Subject(s) - nodular regenerative hyperplasia , portal hypertension , medicine , transjugular intrahepatic portosystemic shunt , varices , gastroenterology , gastric varices , upper gastrointestinal bleeding , liver biopsy , liver function , portal venous pressure , endoscope , endoscopy , biopsy , surgery , cirrhosis
Nodular regenerative hyperplasia (NRH) of liver is a relatively rare liver disorder, but a frequent cause of noncirrhotic portal hypertension. We present a lady with common variable immune deficiency who presented with upper gastrointestinal bleeding and deranged liver function tests but preserved synthetic function. Upper gastrointestinal endoscope showed bleeding gastric varices and non-bleeding oesophageal varices. Although her oesophageal varices were eradicated by repeated endoscopic band ligation, the gastric varices failed to resolve after repeated endoscopic histocryl injection and she eventually needed transjugular intrahepatic portosystemic shunt placement. Liver biopsy showed NRH. We review the association of hypogammaglobinaemia and NRH and discuss the appropriate management of portal hypertension in NRH.