A case of nodular regenerative hyperplasia (NRH) of the liver with CRST syndrome complicated by recurrent rupture of esophageal varices.

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A 71-year-old female was admitted to Tokyo Metropolitan Hiroo General Hospital for hematemesis. Emergency endoscopy revealed ruptured esophageal varices. Liver function tests, however, were almost within normal limit. Percutaneous needle biopsy of the liver was performed, which revealed no significant changes except for a slight round cell infiltration in the portal area.<BR>During her 2nd admission hematemesis happened again. Exploratory laparotomy with wedge biopsy of the liver was done, revealing multiple small-sized hepatocytic nodules without encapsulation scattered throughout the liver, the findings compatible with nodular regenerative hyperplasia of the liver. In addition, the patient showed the physical signs of CRST syndrome with positive serological tests for antinuclear and anti-centromete antibody.<BR>4 cases of NRH with CRST syndrome have been found in the world literature, but none in Japanese.<BR>It was suggested hepatocytic nodules caused portal hypertension by increasing pre- and postsinusoidal pressure, imparing sinusoidal blood flow and compressing tributaries of hepatic veins. Immunological abnormalities may be concerned with the etiology of NRH.
社団法人日本肝臓学会の論文