Department of Gastroenterology, Glasgow Royal Infirmary, Glasgow G4 0SF, United Kingdom.
World J Gastroenterol. 2013 Jun 14;19(22):3502-4. doi: 10.3748/wjg.v19.i22.3502.
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.
肝结节性再生性增生（NRH）是一种相对罕见的肝脏疾病，但却是非肝硬化性门静脉高压症的常见原因。我们介绍一位患有普通可变免疫缺陷的女性，她以上消化道出血和肝功能异常为表现，但合成功能正常。上消化道内镜显示出血性胃静脉曲张和非出血性食管静脉曲张。尽管她的食管静脉曲张通过反复内镜套扎得以消除，但胃静脉曲张在反复内镜组织胶注射后仍未缓解，最终需要进行经颈静脉肝内门体分流术。肝脏活检显示 NRH。我们回顾了低丙种球蛋白血症与 NRH 的关联，并讨论了 NRH 门静脉高压症的适当治疗方法。
