儿童肠型白塞病并发α地中海贫血  

Intestinal Behcet's disease complicated withαthalassemia in a child

作　　者：刘远琴 陆婉秋 贾丽丹 陈桂芳 艾戎 LIU Yuanqin;LU Wanqiu;JIA Lidan;CHEN Guifang;AI Rong(Clinical College of Guizhou Medical University,Guiyang550004,China;Department of Pediatrics,Affiliated Hospital of Guizhou Medical University,Guiyang 550004,China)

机构地区：[1]贵州医科大学临床医学院,贵州贵阳550004 [2]贵州医科大学附属医院儿科,贵州贵阳550004

出　　处：《临床皮肤科杂志》2023年第7期418-421,共4页Journal of Clinical Dermatology

摘　　要：报告1例肠型白塞病并发α地中海贫血。患儿男,9岁。因反复口腔和肛周溃疡3年就诊。皮肤科检查:口腔黏膜见多处大小不等溃疡灶,最大直径约1.0cm,中央凹陷。检查时取截石位,肛周3点、4点及6点各见1处溃疡灶,直径1~1.2cm,均有白色分泌物覆盖。结直肠镜检查示回肠末端和肛周溃疡。回肠末段和回盲瓣组织病理检查示黏膜重度慢性炎症伴溃疡形成。HLA-B51基因检测阳性。地贫基因检测:右缺失型(-α^(3.7)/αα)。诊断:肠型白塞病并发α地中海贫血。A case of intestinal Behcet's disease complicated withαthalassemia is reported.A 9-year-old boy presented with recurrent oral and perianal ulcers for 3 years.Dermatological examination showed multiple ulcers of different sizes with central depression in the oral mucosa,with a maximum diameter of about 1.O cm.Ulcers were found in a lithotomy position at 3,4,and 6 o'clock respectively,with diameters ranging from 1-1.2 cm,and all were covered by white secretion.Colonoscopy showed terminal ileum ulcer and perianal ulcer.Histopathological examination of the terminal ileum and ileocecal valve showed severe chronic mucosal inflammation with ulcer formation.HLA-B51 gene test was positive.A right deletion(-α^(37)/αα)of the thalassemia gene was detected.According to the clinical manifestations,histopathological and genetic results,a diagnosis of intestinal Behcet's disease andαthalassemia was made.

关 键 词：白塞病 肠型 Α地中海贫血 

分 类 号：R597.9[医药卫生—内科学] R556.61[医药卫生—临床医学]