24例Cronkhite-Canada综合征患者的内镜特点及临床相关性分析  

Endoscopic features and clinical correlation analysis of 24 patients with Cronkhite-Canada syndrome

作　　者：刘爽[1] 游燕[2] 吴东[1] 万梦瑶 周良锐 周炜洵[2] 张晟瑜[1] 李骥[1] Liu Shuang;You Yan;Wu Dong;Wan Mengyao;Zhou Liangrui;Zhou Weixun;Zhang Shengyu;Li Ji(Department of Gastroenterology,Peking Union Medical College Hospital,Chinese Academy of Medical Sciences,Beijing 100730,China;Department of Pathology,Peking Union Medical College Hospital,Chinese Academy of Medical Sciences,Beijing 100730,China)

机构地区：[1]中国医学科学院北京协和医学院北京协和医院消化内科,北京100730 [2]中国医学科学院北京协和医学院北京协和医院病理科,北京100730

出　　处：《中华医学杂志》2020年第20期1562-1566,共5页National Medical Journal of China

基　　金：国家重点研发计划项目(2016YFC0901500);北京市东城区优秀人才培养资助项目(2018-34);北京市大学生创新项目(2019zlgc0651)。

摘　　要：目的:阐述Cronkhite-Canada综合征(CCS)患者的内镜下特点及其与临床特点的相关性。方法:回顾性纳入1999年1月至2019年6月于北京协和医院住院使用糖皮质激素治疗前接受胃镜及结肠镜检查的CCS患者24例。采集其人口学特点、临床表现、初始内镜下特点及组织病理学资料并分析。结果:24例患者中男15例(62.5%),女9例(37.5%),年龄(59±10)岁,中位病程6(1~36)个月。病变累及胃及结肠100%、十二指肠95.7%、直肠66.7%及末段回肠50.0%。胃病变以胃体下部及胃窦病变尤重,结肠病变以右半结肠为著。所有患者均无食管受累。胃肠镜下病变多表现为弥漫性黏膜充血水肿,伴息肉或结节样改变,病变较重处可有桑葚样或叠瓦状改变,47.8%存在十二指肠淋巴管扩张样改变。息肉多为无蒂或亚蒂,偶见结肠带蒂息肉。11例完整切除、且可清晰解读结肠息肉表面腺管开口的病例中,息肉腺管开口的工藤分型分别为Ⅲs到ⅤⅠ型,其中2例病理为腺管状腺瘤者工藤分型均为ⅢL,其余息肉病理为增生性息肉或CCS息肉。确诊前病程与结肠息肉最大直径正相关(r=0.625,P=0.006),弥漫全胃受累患者的血清白蛋白水平显著低于胃底贲门未受累患者[(29±8)g/L比(37±5)g/L,P=0.034]。结论:CCS的典型内镜下表现是胃、十二指肠、结肠黏膜弥漫充血水肿基础上的多发息肉或结节隆起,可合并十二指肠或末段回肠的淋巴管扩张样改变。部分内镜下特点与疾病的临床特点密切相关。Objective To clarify the endoscopic changes prior to corticosteroid therapy in Cronkhite-Canada syndrome(CCS)patients and to explore the correlation between endoscopic features and clinical characteristics.Methods A total of 24 CCS patients who were hospitalized in Peking Union Medical College Hospital from January 1999 to June 2019 and underwent gastroscopy and colonoscopy before corticosteroid therapy were retrospectively enrolled.The endoscopic images were re-interpreted.The demographic characteristics,clinical manifestations,laboratory tests and histopathological data were collected and analyzed.Results Of all 24 patients,15(62.5%)were male and 9(37.5%)were female,with an average age of(59±10)years and disease course of 6(1~36)months.Based on the endoscopic findings,the percentages of stomach,colon,duodenum,rectum and terminal ileum involvement were 100%,100%,95.7%,66.7%and 50.0%respectively.Gastric involvement was more severe in the lower part of the body and the antrum of the stomach,while the cardia and the fundus were spared in 5(20.8%)cases.Colonic involvement was more severe in the right colon.No patient showed remarkable esophageal involvement.The typical appearance under endoscopy were diffuse mucosal hyperemia and edema with polyps or nodular changes.The lesions may have mulberry-like or imbricate changes in severe cases.Lymphatic dilation in the duodenum was found in 47.8%patients.Most of the polyps were pedunculated or sub-pedunculated,with occasionally seen sessile polyps in the colon.The pit patterns of the 12 resected colon polyps in 11 patients could be classified as Kudo typeⅢS,ⅢL,ⅣandⅤI,among which 2 tubular adenomas had the Kudo typeⅢL.Other resected polyps were hyperplastic polyps or CCS polyps.The disease duration prior to diagnosis was positively correlated with the maximum diameter of colon polyps(r=0.625,P=0.006).Serum albumin levels in patients with whole stomach involvement were significantly lower than those in patients with cardia spared[(29±8)g/L vs(37±5)g/L,P=0.034].C

关 键 词：CRONKHITE-CANADA综合征 内镜 胃肠道息肉 工藤分型 

分 类 号：R57[医药卫生—消化系统]