62例蓝色橡皮大疱痣综合征临床特征分析  

Clinical analysis of sixty-two blue rubber bleb nevus syndrome cases

作　　者：崔洪涛[1] 许帅[1] 刘浩润[1] 李亚男[2] 李为民[1] 

机构地区：[1]中国人民解放军309医院肝胆外科,北京100091 [2]北京航天总医院,北京100076

出　　处：《临床消化病杂志》2016年第2期110-112,共3页Chinese Journal of Clinical Gastroenterology

摘　　要：[目的]分析国内蓝色橡皮大疱痣综合征(BRBNS)的文献报道,探讨中国BRBNS患者的临床特征。[方法]回顾性分析62例BRBNS患者(包括中国人民解放军309医院收治的4例、国内文献报道的58例)的临床资料,总结BRBNS的临床特征、病变特点、诊治方法及预后。[结果]62例BRBNS患者中男41例,女21例,发病年龄中位数为16.2岁。所有患者均有皮肤病变,61例(97.1%)患者有消化道出血表现。以黑便为主诉者32例(51.6%),贫血为主诉者15例(24.2%),皮肤血管瘤为主诉者7例(11.3%)。病变可累及多部位多脏器。消化道病变中,胃受累最多(74.2%,46/62),小肠其次62.9%(39/62)。[结论]BRBNS是一种罕见的先天性疾病,皮肤和消化道是常累及的部位,多并发消化道出血,有效地治疗方法尚需要进一步研究探讨。[Objective] To analyze the clinical characteristics of blue rubber bleb nevus syndrome （BRBNS）from the published articles in China. [Methods]The clinical manifestation, characteristic of le- sions,diagnosis,treatment and prognosis of 62 BRBNS cases reported（including 4 cases treated in the hos- pital and 58 cases reported by domestic literature）were summarized. [Results]Among the 62 patients,41 ca- ses were male and 21 cases were female,and median age was 16.2 years. All patients had the venous mal- formations of the skin, 97. 1 % （61 cases） presented with gastrointestinal bleeding. 32 （51. 6 % ） cases com- plained of melena, 15 cases （24.2 % ） complained of anemia, and 7 cases （ 11.3 % ） complained of cutaneous he- mangiomas. For the digestive tract lesions,the position of the lesions were located in stomach（46,74.2%）, small intestine（62.9% ,39/62）. [Conclusion]Blue rubber bleb nevus syndrome is a rare disease which main- ly involve skin and digestive tract. BRBNS mostly complicated with gastrointestinal bleeding. The effective treatment still need further study.

关 键 词：蓝色橡皮大疱痣综合征 血管瘤 消化道 内镜检查 治疗 

分 类 号：R758.5[医药卫生—皮肤病学与性病学]