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目的分析北京协和医院CCS患者流行病学及临床病理特点、治疗策略及预后。方法回顾性分析,非参数Spearman相关分析判断各治疗方法的短期及远期疗效。结果 12例CCS患者均存在上消化道和下消化道息肉,以及至少1项外胚层异常。1/3患者尿蛋白阳性,1例合并肾病综合征。若联合腹痛、腹泻及便潜血阳性3项指标,则灵敏度为100%。胃镜下可见多发黏膜下黑褐色素斑,息肉病理类型以增生性息肉及腺管状腺瘤最常见。平均随访44.7月,存活率100%;10例在初始或后续治疗中加用糖皮质激素,均获不同程度缓解。糖皮质激素停药后复发者再次使用仍然有效。泼尼松≥40 mg/d较≤40 mg/d预后更佳(n=10,r=-0.655,P<0.05);单用氨基水杨酸制剂、胃黏膜保护剂、肠道益生菌、息肉电切术及肠内外营养支持均无明显疗效;氨基水杨酸制剂与较差预后相关(r=0.598,P<0.05)。结论 CCS患者可见黏膜下色素沉着及尿蛋白阳性,糖皮质激素为基础的综合治疗临床缓解率较高,提示其发病机制可能有免疫因素参与。
Objective To analyze the epidemiological, clinical and pathological features, treatment strategies and prognosis of CCS patients in Peking Union Medical College Hospital. Methods The retrospective analysis and nonparametric Spearman correlation analysis were used to determine the short-term and long-term effects of each treatment. Results All 12 CCS patients had upper gastrointestinal and lower gastrointestinal polyps, and at least 1 ectodermal abnormality. One third of patients had positive urinary protein and one had nephrotic syndrome. If combined with abdominal pain, diarrhea and occult blood positive three indicators, the sensitivity was 100%. Gastroscopic multiple submucosal melanin spots visible polypoid pathological type to proliferative polyps and ductular adenoma most common. With an average follow-up of 44.7 months, the survival rate was 100%. 10 cases were treated with glucocorticoid in initial or follow-up treatment, and all were relieved to varying degrees. Re-use of glucocorticoid after stopping relapse is still valid. Prednisone ≥40 mg / d vs ≤40 mg / d had a better prognosis (n = 10, r = -0.655, P <0.05); with aminosalicylic acid alone, gastric mucosal protective agent, There was no significant effect of polyp resection and enteral and parenteral nutrition support; aminosalicylate preparation was associated with poor prognosis (r = 0.598, P <0.05). Conclusion CCS patients with submucosal pigmentation and urinary protein positive, glucocorticoid-based comprehensive treatment of clinical response rate is high, suggesting that its pathogenesis may have immune factors involved.