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病例报告——克朗凯特-加拿大综合征息肉的恶性转化

Case report-malignant transformation in Cronkhite-Canada syndrome polyp.

作者信息

Zong Ye, Zhao Haiying, Yu Li, Ji Ming, Wu Yongdong, Zhang Shutian

机构信息

Department of Gastroenterology, Beijing Friendship Hospital, Capital Medical University, Beijing, China.

出版信息

Medicine (Baltimore). 2017 Feb;96(6):e6051. doi: 10.1097/MD.0000000000006051.

DOI:10.1097/MD.0000000000006051
PMID:28178153
原文链接:https://pmc.ncbi.nlm.nih.gov/articles/PMC5313010/
Abstract

RATIONALE

Cronkhite-Canada syndrome (CCS) is a rare disease, the etiology of CCS is currently unknown. Although CCS is widely accepted as a benign disorder, the malignant potential of the polyps in CCS patients is yet controversial.

PATIENT CONCERNS

A 55-year-old Chinese male was first admitted to Beijing Friendship Hospital with a 3-month history of frequent watery diarrhea (10-15 times/day), loss of taste, and a weight loss of 10 kg in August 2010. The left heel bone fracture in the patient occurred about 2 weeks prior to his diarrhea.

DIAGNOSES

He was diagnosed as Cronkhite-Canada syndrome.

INTERVENTIONS

Oral administration of prednisone was initiated at a dosage of 20 mg/day.

OUTCOMES

After 3 months of treatment, the clinical manifestations disappeared, and colonoscopy showed sparsely distributed small polyps in the colon. Consequently, the dose of prednisone was reduced to 10mg. However, after 4 months, his fingernails were again found atrophic along with mild abdominal discomfort without diarrhea. Colonoscopy revealed a recurrence of the polyps in March 2011. The treatment was repeated with prednisone at a dosage of 20 mg/day resulting in subsided symptoms. In September 2011, he underwent colonoscopy although no significant clinical manifestations were observed. In addition, the polyp in the sigmoid colon was cancerated.

LESSONS

The present case indicated that the physical stress was related to CCS and malignant transformation occurred in Cronkhite-Canada syndrome polyp. After the diffused inflammatory polyps have responded to steroid therapy, other existing adenomas require endoscopic treatments, which can decrease the possibility of neoplastic transformation.

摘要

理论依据

克-卡综合征(CCS)是一种罕见疾病,其病因目前尚不清楚。尽管CCS被广泛认为是一种良性疾病,但CCS患者息肉的恶性潜能仍存在争议。

患者情况

一名55岁的中国男性于2010年8月首次入住北京友谊医院,有3个月频繁水样腹泻病史(每日10 - 15次)、味觉丧失及体重减轻10千克。该患者左足跟骨骨折发生在腹泻前约2周。

诊断

他被诊断为克-卡综合征。

干预措施

开始口服泼尼松,剂量为每日20毫克。

结果

治疗3个月后,临床表现消失,结肠镜检查显示结肠内息肉分布稀疏。因此,泼尼松剂量减至10毫克。然而,4个月后,再次发现他的指甲萎缩,伴有轻度腹部不适但无腹泻。2011年3月结肠镜检查显示息肉复发。再次使用泼尼松每日20毫克治疗,症状缓解。2011年9月,他接受了结肠镜检查,尽管未观察到明显临床表现。此外,乙状结肠息肉发生癌变。

经验教训

本病例表明身体应激与CCS有关,克-卡综合征息肉会发生恶性转化。弥漫性炎性息肉对类固醇治疗有反应后,其他现存腺瘤需要内镜治疗,这可降低肿瘤转化的可能性。

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Cronkhite-Canada syndrome: an acquired condition of gastrointestinal polyposis and dermatologic abnormalities.克朗凯特-加拿大综合征:一种获得性胃肠道息肉病和皮肤异常疾病。
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Case report of patient with a Cronkhite-Canada syndrome: sustained remission after treatment with corticosteroids and mesalazine.一名患有克朗凯特-加拿大综合征患者的病例报告:经皮质类固醇和美沙拉嗪治疗后持续缓解。
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Recombinant Thrombomodulin Used to Successfully Treat Cronkhite-Canada Syndrome with Disseminated Intravascular Coagulation due to Sepsis in a Compromised Patient.重组血栓调节蛋白成功治疗一名因脓毒症并发弥散性血管内凝血的克朗凯特-加拿大综合征患者。
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