误诊疾病数据库单病种误诊文献研究:POEMS综合征  

A Study on Misdiagnosis Literature of Single Disease of Misdiagnosed Disease Database: POEMS Syndrome

作　　者：徐依成[1] 杜继臣[1] 

机构地区：[1]航天中心医院神经内科,北京100049

出　　处：《临床误诊误治》2016年第9期32-35,共4页Clinical Misdiagnosis & Mistherapy

摘　　要：POEMS综合征为临床少见疾病,表现多样,可累及多个系统,加之其缺乏特异性症状和体征,误诊率高。误诊疾病数据库收录2004—2013年发表在中文医学期刊上的POEMS综合征误诊文献共31篇,累计误诊病例140例,误诊时间1个月~6年,其中6篇涉及误诊率,误诊率高达85.9%。共误诊152例次36种疾病,其中误诊例次居前3位的是慢性炎性脱髓鞘性多发性神经病（chronic inflammatory demyelinating polyneuropathy,CIDP）、Guillain-Barre综合征及多发性周围神经病。129例描述了误诊与疾病转归的关联,3.1%的患者造成Ⅲ级后果,86.82%的患者造成Ⅱ级后果,10.08%的患者造成Ⅰ级后果。诊治经验不足、缺乏对该病认识是导致误诊的最常见原因。提高对该病的认识,建立整体性、全面性诊断思维,进行适当的实验室筛查（血清、尿免疫固定电泳及血管内皮生长因子测定）和必要的医技检查,是减少和避免POEMS综合征、误漏诊的关键。POEMS syndrome is a rare disease in clinical practice. Due to its complicated clinical manifestations,multiple systems are involved and lack specific symptoms and signs,and therefore,and therefore POEMS tends to be misdiagnosed. During 2004 and 2013,a total of 31 papers related to the misdiagnosis of POEMS syndrome had been published in Chinese medical journals,and all these articles had been included in the misdiagnosed disease database. Through the analysis of these databases,we found that the number of cumulative misdiagnosis cases was 140,and the time of misdiagnosis duration differed from 1 month to 6 years. 6 papers referred to the misdiagnosis rate,which accounted for 85. 9%. There were 152 cases of POMES syndrome misdiagnosed as other disease. The range of POEMS misdiagnosed as other diseases had a very wide scope with a total of 36 kinds. And it mainly concentrated in chronic inflammatory demyelization polyneuropathy（ CIDP）,Gillian-Bare syndrome,and polyneuropathy. 129 cases described the association between the misdiagnosis and the outcome.3. 1% of patients had grade Ⅲ consequences,86. 82% of the patients had grade II consequences,10. 08% of the patients had grade I consequences. Inadequate experience and lack of understanding of POEMS is the most common cause of misdiagnosis. In order to reduce the rate of misdiagnosis,clinicians should have a comprehensive clinical thinking style as a whole and master the relevant knowledge of the disease,perform the proper laboratory examination as serum（ urine） immune-fixed electrophoresis and VEGF determination,and necessary auxiliary examination.

关 键 词：POEMS综合征 误诊 多发性神经病 GUILLAIN-BARRE综合征 

分 类 号：R442.8[医药卫生—诊断学]