【作者】 白静；

【导师】 钱家鸣；

【作者基本信息】 中国协和医科大学 ， 内科学， 2007， 硕士

【摘要】 1.目的对肠白塞病(Intestinal Behcet’s disease)患者资料进行分析,旨在探讨本病临床特点,加深对本病的认识。2.材料与方法参照1990年国际白塞病研究组(International Study Group forBehcet’S Disease,BD-ISG)制定的白塞病(Behcet’s disease,BD)诊断标准及分类方案,回顾性分析北京协和医院1986年1月～2006年12月收治29例肠白塞病患者临床资料,并应用SPSS 13.0统计软件进行统计。3.结果①一般情况:中位发病年龄26岁,病程较长,男女之比为1:1.23。②临床表现:93.1%的患者消化道症状晚于BD系统表现出现;腹痛最常见,占93.1%,多位于右下腹(15/27);消化道出血、肠梗阻、穿孔和瘘管形成为肠白塞病常见并发症,它们的发生率依次为41.4%、24.1%、17.2%和10.3%。③辅助检查:病情活动时常见实验室检查异常为血沉(Erythrocytesedimentation rate,ESR)(92.3%)和C-反应蛋白(C-reactive protein,CRP)(92.0%)升高;人白细胞抗原(Human leucocyte antigen,HLA)-B5检查阳性率为30.8%(4/13)。④造影检查:病变以胃肠粘膜皱襞粗大不规则最多见,占54.5%。⑤内镜检查:回盲部受累最为常见,占69.6%(16/23),单一部位受累者占34.8%(8),上下消化道均受累3例。最常见的镜下表现为溃疡,占95.7%(22/23),包括孤立深大溃疡14例(60.8%)及多发或单发不规则浅溃疡9例(39.1%)。⑥病理:内镜活检标本(25)均无血管炎表现,7例术后病理中3例存在血管炎表现,另有1例镜下可见管壁不规则增厚的肌性血管,对诊断有帮助。⑦诊断:首诊误诊率高,多诊为阑尾炎(7例,24.1%)。⑧治疗:以激素(28)、免疫抑制剂(20)为主,依从性差(78.6%自行调整用药),激素减量或停药易复发;深大溃疡较浅小溃疡药物治疗疗效差且复发率高,复发及单用激素治疗无效者选择联合免疫抑制剂治疗有效;手术率为31.0%(9),包括由于溃疡穿孔行急诊手术5例、单纯阑尾切除术2例,因回盲部巨大溃疡出血内科保守治疗无效及腹痛持续不缓解手术各1例;术式以回盲部及部分小肠切除(4)、右半结肠及部分回肠切除(3)为主。⑨完全型与不完全型肠白塞病临床特点无显著差异。4.结论①肠白塞病以青年患者多见,病程迁延,首诊误诊率高。②消化道症状多晚于BD系统病变出现,腹痛最常见。③病变主要累及回盲部,以深大溃疡为主。④深大溃疡较浅小溃疡药物治疗疗效差且复发率高。⑤复发及单用激素治疗无效者联合免疫抑制剂有效。⑥完全型肠白塞病与不完全型肠白塞病临床特点无明显差异。更多还原

【Abstract】 1.Objective:To analyze the clinical characteristics of intestinal Behcet’s disease, and provide the knowledge of its diagnosis and therapy.2.Methods:According to the International Study Group for Behcet’s Disease diagnostic criter and classification of Behcet’s disease(BD),29 patients,who involved definite gastrointestinal system manifestations,were diagnosed as Behcet’s disease from January 1986 to December 2006 in Peking Union Medical College Hospital.Their clinical data were analyzed retrospectively by SPSS 13.0.3.Results:①The median age at onset of the patients with intestinal Bechet’s was 26 years old with long clinical courses.Ratio of male to female was 1:1.23.②Clinical symptoms and signs of gastrointestinal were appearance later than that of systemic manifestation of BD in 93.1%cases.Abdominal pain was the most common symptom,the rate was 93.1%,and frequently represented on right-lower abdomen(15/27).The chief complaints of the patients with intestinal Behcet’s disease were gastrointestinal bleeding(41.4%),obstruction(24.1%),perforation (17.2%) and intestinal fistula(10.3%) in turn.③Both ESR(92.3%) and CRP (92.0%) were increased which were common abnormity of laboratory test.The positive rate of HLA-B5 was 30.8%(4/13).④54.5%of cases showed the gastrointestinal mucous membranes were thick by X ray.⑤By means of the endoscopy,69.6%(16/23)of the patients with intestinal Behcet’s disease had ulcers located in the ileocecal.34.8%(8) only had one ulcer.Whole gastrointestinal tract involved only seen in 3 cases.95.7%(22/23) had ulcer by endoscopic in which 14 cases(60.8%) with solitary large shape ulcers and 9 cases (39.1%) with single or multiple irregular geographic ulcers.⑥Nether of the 25 endoscopic biopsy samples were found the inflammatory vasculitis performance, but 3 of 7 surgical-pathologic samples were positive.⑦The rate of misdiagnosis when the patients first visited was high,and most of them were diagnosed as appendicitis(7 cases,24.1%).⑧The main therapy of intestinal Behcet’s disease included glucocorticoid hormone(28) and immunosuppressant(20),but the compliance of patients was poor,and it’s easy to recurrence in the course of reducing the dose of glucocorticoid hormone or stopping using the medicine. Larger shape ulcers in intestinal Behcet’s disease showed a less favorable response to medical treatment but a more frequent rate of recurrence.The patients who had recurrence or useless therapy with glucocorticoid hormone only, combined immunosuppressant had got an approving curative effect.9 cases had a history of operation,including 5 emergency surgeries for perforation;the operation procedures including the resection of ileocecal(4) and right hemicolectomy(3).⑨There’s no significant difference between the complete BD and incomplete BD.4.Conclusion:①Most of the patients of intestinal Behcet’s disease were young people,the clinic course was long and it’s difficult to diagnose.②The clinical symptoms and signs of gastrointestinal often appearance later than the systemic manifestation of BD.Abdominal pain was the most common symptom.③The patients with intestinal Behcet’s disease had ulcer which often located in the ileocecal in large shape ulcers.④The larger shape ulcers in intestinal Behcet’s disease showed a less favorable response to medical treatment but a more frequent rate of recurrence.⑤The patients who had recurrence or useless therapy with glucocorticoid hormone only,combined immunosuppressant had get a approving curative effect.⑥There’s no significant difference between the complete BD and incomplete BD.更多还原