Published online Jun 14, 2013. doi: 10.3748/wjg.v19.i22.3502
Revised: January 30, 2013
Accepted: February 5, 2013
Published online: June 14, 2013
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.
Core tip: Nodular regenerative hyperplasia (NRH) is still an evolving concept. Although a rarely identified liver disorder, it is a frequent cause of noncirrhotic portal hypertension. Liver involvement in primary hypogammaglobulinemia mainly consists of NRH leading to chronic cholestasis and portal hypertension. Optimal management of gastric variceal bleed remains unclear. Histoacryl injection is the endoscopic method of choice for gastric variceal bleed but one should keep a lower threshold for transjugular intrahepatic portosystemic shunt procedure for recurrent gastric variceal bleed.