Cronkhite-Canada综合征三例报道并文献复习

Cronkhite-Canada综合征(CCS)目前被认为是一种非遗传性疾病，临床较为罕见，内镜下多表现为胃肠道多发息肉，临床上以消化道症状、皮肤色素沉着、脱发、脱甲等为主要表现。本文分析了2012年8月至2021年9月于山东大学齐鲁医院确诊为CCS的3例患者的临床特点及诊疗过程并作文献复习。CCS的诊断基于病史、体格检查、胃肠息肉的内镜检查和组织病理学结果，消化内镜是其最直接的检查手段。3例CCS患者内镜下均表现为胃肠道弥漫性多发息肉，均存在脱发、脱甲表现；病例1和病例3白蛋白水平均降低，病例2和病例3抗核抗体(ANA)阴性，病例1未查ANA，3例均无幽门螺杆菌(HP)感染。糖皮质激素及质子泵抑制剂(PPI)为CCS的主要治疗药物，病例1未使用糖皮质激素治疗，仅给予PPI及支持治疗，后期出现血便、肠坏死，起病6年后因胃癌行全胃切除术；病例2和病例3确诊CCS后给予泼尼松、PPI等治疗后好转。早期诊断、以糖皮质激素为中心的药物治疗、定期内镜检查和新的治疗方案可能为未来更好的预后提供更多实践经验，可提高临床医师对该病的认识和理解、减少漏诊和误诊。

Cronkhite-Canada Syndrome: Report of Three Cases and Literature Review

Cronkhite-Canada syndrome (CCS) is currently considered to be a non-genetic disorder and a relatively rare clinical condition that is characterized endoscopically by multiple polyps in the gastrointestinal tract and clinically manifested by gastrointestinal symptoms, skin pigmentation, hair loss, and nail loss. In this paper, we analyzed the clinical characteristics and treatment procedures of three patients diagnosed with CCS at Qilu Hospital of Shandong University from August 2012 to September 2021 and reviewed the relevant literature. The diagnosis of CCS is based on medical history, physical examination, endoscopic findings of gastrointestinal polyps, and histopathology, with gastrointestinal endoscopy being the most direct means of examination. All three patients presented endoscopically with diffuse multiple polyps in the gastrointestinal tract, had hair and nail loss and were free of HP infection. Moreover, case 1 and case 3 both had reduced serum albumin levels; case 2 and case 3 were negative for antinuclear antibodies (ANA) , case 1 was not checked for ANA. Glucocorticoids and proton pump inhibitors (PPI) were the main pharmacological treatments for CCS. Case 1 was not treated with glucocorticoids, but only PPI and supportive treatment, and later developed bloody stools and intestinal necrosis and underwent total gastrectomy for gastric cancer 6 years after the onset of the disease. Cases 2 and 3 were improved after being treated with prednisone and PPI. Early diagnosis, glucocorticoid-based pharmacotherapy, regular gastrointestinal endoscopy, and new treatment options may contribute to a better prognosis, and the reduction in possibilities of missed diagnosis or misdiagnosis of CCS via improving clinicians' knowledge and understanding of the disease.