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克罗恩病-加拿大综合征伴食管受累及六年随访:一例报告。

Cronkhite-Canada syndrome with esophagus involvement and six-year follow-up: A case report.

机构信息

Department of Endocrinology, The First Affiliated Hospital of Ningbo University, Ningbo 315000, Zhejiang Province, China.

出版信息

World J Gastroenterol. 2024 Feb 28;30(8):984-990. doi: 10.3748/wjg.v30.i8.984.

Abstract

BACKGROUND

Cronkhite-Canada syndrome (CCS) is a rare, noninherited disease characterized by gastrointestinal polyposis with diarrhea and ectodermal abnormalities. CCS polyps are distributed through the whole digestive tract, and they are common in the stomach and colon but very uncommon in the esophagus.

CASE SUMMARY

Here, we present a case of a 63-year-old man with skin hyperpigmentation accompanied by diarrhea, alopecia, and loss of his fingernails. Laboratory data indicated anemia, hypoalbuminemia, hypocalcemia, hypokalemia, and positive fecal occult blood. Endoscopy showed numerous polyps scattered throughout the digestive tract, including the esophagus. He was treated with nutritional support and glucocorticoids with remission of his symptoms.

CONCLUSION

Comprehensive treatment led by hormonal therapy can result in partial or full remission of clinical symptoms. Treatment should be individualized for each patient according to their therapy response. Surveillance endoscopy is necessary for assessing mucosal disease activity and detecting malignant transformation.

摘要

背景

Cronkhite-Canada 综合征(CCS)是一种罕见的非遗传性疾病,其特征为胃肠道息肉病伴腹泻和外胚层异常。CCS 息肉分布于整个消化道,常见于胃和结肠,但非常罕见于食管。

病例总结

本案例报道了一位 63 岁男性,其皮肤出现色素沉着,伴有腹泻、脱发和指甲脱落。实验室数据显示贫血、低白蛋白血症、低钙血症、低钾血症和粪便潜血阳性。内镜检查显示消化道内有许多息肉,包括食管。患者接受了营养支持和糖皮质激素治疗,症状缓解。

结论

激素治疗为主的综合治疗可使部分或全部临床症状缓解。应根据患者的治疗反应为每位患者制定个体化治疗方案。监测内镜对于评估黏膜疾病活动度和发现恶性转化非常必要。

https://cdn.ncbi.nlm.nih.gov/pmc/blobs/497e/10950646/61e6ac5e9c94/WJG-30-984-g001.jpg

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