摘要
目的 探讨自身免疫性疾病相关的非肝硬化性门静脉高压症患者的临床、病理特点。 方法 分析 6例自身免疫性疾病相关的非肝硬化性门静脉高压症患者的资料。患者均为女性 ,无肝炎病史 ,其中 5例发生上消化道出血 ,1例行硬化治疗 ,3例行手术治疗。 4例患者获得了病理诊断。 结果 6例患者均维持稳定的肝功能 ,短期内随诊未再发生上消化道出血。病理检查未见到肝脏纤维间隔形成 ,主要表现为肝结节性再生性增生。 结论 自身免疫性疾病相关的非肝硬化性门静脉高压症是一种少见病症 ,临床上有明确的门静脉高压 ,但缺乏肝硬化的病理表现是其特点。防治上消化道出血是治疗的关键。
Objective To summarize the cases of non cirrhotic portal hypertension associated with autoimmune disease, review the literature and introduce the clinical and pathological features of the syndrome. Methods Six female patients treated, between 1983 and 1998 had no history of hepatitis. Variceal bleeding occurred in 5 patients. One patient received sclerotherapy and operation was performed in 3 patients for variceal bleeding. Four patients were subjected to liver biopsy. Results Liver dysfunction of these patients remained normal and variceal bleeding didn′t reccur after therapy. Liver biopsy showed nodular regenerative hyperplasia without fibrous septa. Conclusions Non cirrhotic portal hypertension associated with autoimmune disease is a rare syndrome, characteriged by significant portal hypertension as well as histological evidence that cirrhosis is absent. Prevention and treatment of variceal bleeding is very important.
出处
《中华外科杂志》
CAS
CSCD
北大核心
2000年第2期101-103,I006,共4页
Chinese Journal of Surgery
