POEMS综合征的临床特征研究.pdfVIP

18 Jou rnal of Ch inese Physician, J anuary, 2007 , Vol 9,No1 POEM S综合征的临床特征研究 1 2 1 1 1 1 1 彭杰 ,李萃 ,唐丽安 ,袁伟健 ,阳惠湘 ,刘霆 ,邹益友 ( 1中南大学湘雅医院消化内科 ,湖南 　长沙 　4 10008; 2 卫生部肿瘤蛋白质组学重点实验室 ) 【摘要 】　目的　研究 POEM S综合征的临床特征 ,提高对 POEM S综合征的认识 。方法 　分析 5例确诊的 POEM S综合征临床 表现 、实验室检查结果及治疗经过 。结果 　5例患者中 ,主要临床表现为多发性神经病变 ,肝 、脾肿大 , 内分泌病变 ,皮肤色素沉着及 水肿 ; 1例伴 Castlem an病 ;院外 5例均误诊 ;经治疗后 5例症状均缓解 。结论 　POEM S综合征是一个被人们认识不足的疾病 , 因此 应予以重视 , 以免误诊 、漏诊 。 [关键词 ] 　　PO EM S综合征 C lin ica l Ana ly sis of PO EM S Syn drom e PEN G J ie, L I Cu i, TAN G L ian, YUAN W e ij ian, YAN G H u ix iang, L IU T ing, ZOU Y iy ou. D ep artm ent of Ga stroen tero logy, X iangya Ho s p ital, Cen tral Sou th U n iversity, Changsha 4 10008, Ch ina 【A b stract】　O bjective　To analyze the clin ical characteristic s of POEM S syndrom e and to imp rove the diagno sis of POEM S syn drom e. M ethods　5 ca se sw ith p roved POEM S syndrom e were analyzed, including their clin ical m an ife station s, laboratory te sts and therap eu tic p roce ss. Results　The m ajor clin ical m an ifestation s of the 5 p atien ts were p eripheral polyneurop athy, hegatoand sp lenom egaly, endocri nop athy, p igm en tation and edem a. One case had Ca stlem an disease. The m isdiagno sis rate wa s 100% before adm ission. Symp tom swere im p roved in all ca se s after treatm en t. Conclu sion　POEM S syndrom e, at p resen t, rem ain s to be an underdiagno sed and undertreated disorder and more atten tion shou ld be drawn in ca se of false diagno sis and m isdiagno sis. 【Key words】　　POEM S syndrom e ( ) 　　PO EM S综合征 又称 Crow Fuka se综合征 是一组 大 2例 ,大小约 05 ～2 0 cm ,质硬 ,无压痛 ,活动度差 。 以多发性感觉运动性周围神经病为突出表现 ,常有多