Cronkhite-Canada综合征三例报道并文献复习

doi:10.12114/j.issn.1007-9572.2022.0244

中国全科医学 ›› 2022, Vol. 25 ›› Issue (33): 4210-4216.DOI: 10.12114/j.issn.1007-9572.2022.0244

Cronkhite-Canada综合征三例报道并文献复习

李淑英¹, 林颖敏², 王敏¹^,³^,^*()

1.250000　山东省济南市，山东大学齐鲁医院消化内科
2.250000　山东省济南市，山东大学齐鲁医院全科医学科
3.250000　山东省济南市，山东大学齐鲁医院老年医学科　山东大学第一临床学院全科医学教研室

收稿日期:2022-02-14 修回日期:2022-04-16 出版日期:2022-11-20 发布日期:2022-05-27
通讯作者: 王敏
李淑英，林颖敏，王敏. Cronkhite-Canada综合征三例报道并文献复习[J].中国全科医学，2022，25（33）：4210-4216. [www.chinagp.net]
作者贡献：李淑英进行文章的构思与设计，文章的可行性分析，文献/资料收集、整理，撰写论文；林颖敏进行论文的修订；王敏负责文章的质量控制及审校，对文章整体负责，监督管理。

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

LI Shuying¹, LIN Yingmin², WANG Min¹^,³^,^*()

1.Department of Gastroenterology, Qilu Hospital of Shandong University, Ji'nan 250000, China
2.Department of General Practice, Qilu Hospital of Shandong University, Ji'nan 250000, China
3.Department of Geriatrics, Qilu Hospital of Shandong University, Department of General Practice, First Clinical College of Shandong University, Ji'nan 250000, China

Received:2022-02-14 Revised:2022-04-16 Published:2022-11-20 Online:2022-05-27
Contact: WANG Min
About author:
LI S Y, LIN Y M, WANG M. Cronkhite-Canada syndrome: report of three cases and literature review[J]. Chinese General Practice, 2022, 25 (33) : 4210-4216.

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摘要/Abstract

摘要： 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。

关键词: 肠息肉病, 疾病特征, 治疗, 病例报告

Abstract:

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.

Key words: Intestinal polyposis, Disease attributes, Therapy, Case reports

李淑英,林颖敏,王敏. Cronkhite-Canada综合征三例报道并文献复习[J]. 中国全科医学, 2022, 25(33): 4210-4216. DOI: 10.12114/j.issn.1007-9572.2022.0244.
LI Shuying,LIN Yingmin,WANG Min. Cronkhite-Canada Syndrome: Report of Three Cases and Literature Review[J]. Chinese General Practice, 2022, 25(33): 4210-4216. DOI: 10.12114/j.issn.1007-9572.2022.0244.

图/表 4

图1 病例1治疗前内镜及病理表现注：A表示胃镜示胃弥漫黏膜隆起呈大小不一不规则息肉样，B表示超声胃镜示胃壁各层次正常结构消失，C表示肠镜示乙状结肠多发息肉样黏膜隆起，表面充血水肿，D表示胃窦胃黏膜息肉（HE染色，×100），E表示降结肠增生性息肉，伴慢性炎症（HE染色，×100），F表示胃低分化腺癌伴印戒细胞癌

Figure 1 Pre-treatment endoscopic and pathological presentations of case 1

图2 病例2治疗前内镜及病理表现注：A表示胃镜示大量息肉样隆起，B表示肠镜示多发结节样增生，C表示胶囊内镜示可见息肉样黏膜隆起，D表示胃窦增生性息肉（HE染色，×40），E表示胃窦炎性增生性息肉（HE染色，×100），F表示横结肠黏膜急慢性炎症伴息肉样增生（HE染色，×100）

Figure 2 Pre-treatment endoscopic and pathological presentations of case 2

图3 病例2治疗5个月后内镜及病理表现注：A表示胃镜示大量息肉样隆起，B表示肠镜示弥漫分布息肉样隆起，C表示胶囊内镜示可见息肉样黏膜隆起，D表示胃窦增生性息肉（HE染色，×100），E表示升结肠炎性息肉，幼年型（HE染色，×100）

Figure 3 Endoscopic and pathological presentations of case 2 after 5 months of treatment

图4 病例3治疗前内镜及病理表现注：A表示经口小肠镜示弥漫结节样隆起，B表示经口小肠镜示回肠上段黏膜水肿粗糙，进镜无效后结晶紫标记，C表示经肛小肠镜示回肠下段黏膜粗糙水肿，部分呈结节样隆起，D表示经肛小肠镜示结肠见多枚亚蒂及广基息肉，E表示空肠黏膜中度慢性炎症（HE染色，×40），F表示回肠黏膜重度活动性慢性炎症（HE染色，×40）

Figure 4 Pre-treatment endoscopic and pathological presentations of case 3

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Cronkhite-Canada综合征三例报道并文献复习

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

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