肝结节性再生性增生18例诊治分析

目的 分析总结肝结节性再生性增生(nodular regenerative hyperplasia of the liver,NRH)的临床诊治经验,以提高临床医师对本病的认识.方法 回顾性分析我院近26年连续收治的18例:NRH的临床表现、影像学及实验室检查、诊治及预后资料.结果 本组18例NRH患者中有15例表现为门静脉高压,4例表现为肝脏单发、多发占位,8例合并自身免疫疾病,3例可疑合并血液系统疾病.本组患者中术前13例被诊断为肝硬化,2例诊断为肝癌或局灶性结节性增生(focal nodular hyperplasia FNH).所有18例患者均行肝楔形活检,并且3例行脾切除,4例行断流术/Phemister术,3例行肝占位/肝叶切除术,1例行部分小肠切除术,1例行脾动脉缩窄限流手术.术后门静脉高压症状明显缓解.随访多数患者症状稳定,说明肝脏占位的:NRH患者预后良好.结论 NRH可能与肝脏血供紊乱有关,临床最常表现为门静脉高压,并可伴发免疫、血液系统性疾病.临床表现为单发、或多发性肝脏占位,应注意与肝硬化、局灶性结节性增生、特发性门静脉高压等鉴别,诊断依靠肝楔形活检.手术对于治疗门静脉高压疗效确切.

Abstract：

Objective To summarize the clinical diagnosis and treatment of nodular regenerative hyperplasia of the liver. Methods Retrospective analysis was made on the clinical manifestations,imagings, laboratory tests, diagnosis, treatment and prognosis of 18 consecutive cases finally established as NRH during the past 26 years. Results 15 of the 18 cases showed portal hypertension, 4 cases showed mono or multiple occupations of the liver, 8 cases suffered from concurrent autoimmune diseases, 3 cases were suspected of blood diseases. Preoperatively, 13 cases were diagnosed as cirrhosis, 2 cases were diagnosed as liver cancer or focal nodular hyperplasia ( FNH). All cases were diagnosed by operative wedging biopsy. 3 cases received splenectomy, 4 cases received disconnection /Phemister surgery, 3 cases received liver occupation/liver lobe resection, 1 case received partial small bowel resection, and 1 case received spleen artery restrictive surgery. Postoperatively, symptoms of portal hypertension relieved obviously. Follow-up study showed most of the patients were stable and prognosis of the NRH was good.Conclusions NRH may relate to the disturbance of liver blood supply, and most common clinical manifestation is portal hypertension, and can combine with immune diseases, hematopathy also can present single or multiple liver occupations. Differential diagnoses include liver cirrhosis, FNH, idiopathic portal hypertension. Diagnosis of NRH relies on liver wedging biopsy. Surgery can relive concurrent portal hypertension.

Nodular regenerative hyperplasia of the liver: a report of 18 cases

Objective To summarize the clinical diagnosis and treatment of nodular regenerative hyperplasia of the liver. Methods Retrospective analysis was made on the clinical manifestations,imagings, laboratory tests, diagnosis, treatment and prognosis of 18 consecutive cases finally established as NRH during the past 26 years. Results 15 of the 18 cases showed portal hypertension, 4 cases showed mono or multiple occupations of the liver, 8 cases suffered from concurrent autoimmune diseases, 3 cases were suspected of blood diseases. Preoperatively, 13 cases were diagnosed as cirrhosis, 2 cases were diagnosed as liver cancer or focal nodular hyperplasia ( FNH). All cases were diagnosed by operative wedging biopsy. 3 cases received splenectomy, 4 cases received disconnection /Phemister surgery, 3 cases received liver occupation/liver lobe resection, 1 case received partial small bowel resection, and 1 case received spleen artery restrictive surgery. Postoperatively, symptoms of portal hypertension relieved obviously. Follow-up study showed most of the patients were stable and prognosis of the NRH was good.Conclusions NRH may relate to the disturbance of liver blood supply, and most common clinical manifestation is portal hypertension, and can combine with immune diseases, hematopathy also can present single or multiple liver occupations. Differential diagnoses include liver cirrhosis, FNH, idiopathic portal hypertension. Diagnosis of NRH relies on liver wedging biopsy. Surgery can relive concurrent portal hypertension.