中国人肝淀粉样变性的临床特点  

Clinical characteristics of hepatic amyloidosis in Chinese patients

作　　者：王慧慧[1] 田字彬[1] 丁雪丽[1] 荆雪[1] 孔心娟[1] 张翠萍[1] 魏良洲[1] 赵清喜[1] 

机构地区：[1]青岛大学医学院附属医院消化科,山东省青岛市266003

出　　处：《世界华人消化杂志》2013年第13期1261-1265,共5页World Chinese Journal of Digestology

摘　　要：目的:分析中国人肝淀粉样变性的临床特点,提高临床诊断率.方法:在中国医学文献数据库(CNKI)中检索1981-2012年中国人肝淀粉样变性临床资料并进行回顾性分析.结果:临床表现主要为乏力、腹胀、纳差、消瘦等非特异性症状.体格检查中肝大(95.5%),脾大(34.8%),移动性浊音(42.4%)多发.实验室检查中碱性磷酸酶明显升高,平均662.94 U/L±620.86 U/L(80.3%),谷氨酰转肽酶升高,平均687.34 U/L±873.91U/L(71.2%).轻链检测阳性率为76.2%.活检病理检查是唯一确诊手段,刚果红染色阳性,淀粉样物质沉积主要部位在窦状隙和间质(54.8%).66例患者中,误诊率达45.5%,死亡原因主要是心、肝、肾多脏器功能衰竭.结论:肝淀粉样变性临床表现、辅助检查缺乏特异性,临床误诊率高.肝脏明显肿大伴碱性磷酸酶、谷氨酰转肽酶明显升高,其他肝功指标轻度异常时,应考虑肝淀粉样变性,及时肝活检可以避免误诊.AIM：To investigate the clinical characteristics of hepatic amyloidosis in Chinese patients.METHODS：Relevant data for Chinese patients with hepatic amyloidosis diagnosed from 1981 to 2012 were retrieved from a Chinese biomedical database and analyzed.RESULTS：Major clinical manifestations of hepatic amyloidosis were unspecific,including fatigue,abdominal distension,anorexia and weight loss.Hepatomegaly（95.5%）,splenomegaly（34.8%） and shifting dullness（42.4%） were common in physical examination.Laboratory tests often revealed apparently elevated serum alkaline phosphatase（662.94 U/L±620.86 U/L;80.3%） and glutamyltranspeptidase（687.34 U/L±873.91 U/L;71.2%）.Approximately 76.2% of patients had either a serum or urine monoclonal protein.Liver biopsy is the golden standard for the diagnosis of this disease.The Congo red staining was often positive.Amyloid deposition was mainly located in the sinusoid and interstitium（54.8%）.The misdiagnosis rate was 45.5%.The major cause of death was multiple organic failure.CONCLUSION：Patients with hepatic amyloidosis often show no specific clinical manifestations or laboratory and imaging features,which results in a high misdiagnosis rate.When a patient has hepatomegaly and elevated alkaline phosphatase and glutamyltranspeptidase,hepatic amyloidosis should be considered even if other liver function parameters are almost normal.Liver biopsy has been suggested to be the only means to confirm the diagnosis.

关 键 词：肝脏 淀粉样变性 误诊 活检 

分 类 号：R575.5[医药卫生—消化系统]