Cronkhite-Canada syndrome: a retrospective analysis of four cases at a single medical center
Cronkhite-Canada syndrome: a retrospective analysis of four cases at a single medical center
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克朗基特-加拿大综合征：单个医疗中心四例病例的回顾性分析
DOI：
10.1080/00365521.2022.2048885
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发表时间：
2022-03-14
影响因子：
1.9
通讯作者：
Chen, Long
中科院分区：
文献类型：
--
作者：
Yu, Xing;Wang, Chengdang;Chen, Long
目的克朗基特-加拿大综合征（CCS）是一种罕见疾病，其特征是多发性胃肠道息肉和外胚层异常。本研究旨在通过展示我们的患者数据来提高对 CCS 的理解。方法对同一医疗中心的四例CCS患者的临床特征、治疗和结果进行回顾性分析。结果 患者年龄32～61岁，平均49.5岁，其中男3例，女1例。所有患者均出现胃肠道症状、外胚层异常和多发性胃肠道息肉。两名患者出现免疫指标异常。 3例患者行磁共振小肠造影，小肠受累的典型表现为弥漫性壁增厚，弥散加权成像信号强度高，强化明显，小肠多发小结节状强化。主要组织学表现为慢性炎症和增生性、腺瘤样、错构瘤样息肉。在两名患者中观察到嗜酸性粒细胞浸润。一名患者在诊断时患有直肠腺癌。 4例患者均接受0.75～1mg/kg/天泼尼松治疗，胃肠道症状逐渐缓解（其中2例外胚层异常，内镜下缓解）。两名患者目前正在接受低剂量泼尼松（2.5-5 mg/天）治疗，分别在 1.5 和 6 年随访期后未出现复发。结论 磁共振小肠造影具有评估CCS小肠病变的潜力。低剂量泼尼松的长期治疗可能有利于维持缓解。
Objective Cronkhite-Canada syndrome (CCS) is a rare disease that is characterized by multiple gastrointestinal polyps and ectodermal abnormalities. This study aimed to improve the understanding of CCS by presenting our patient data. Methods Clinical features, treatment, and outcomes of four CCS patients at a single medical center were retrospectively analyzed. Results The age of the patients ranged from 32 to 61 years (mean: 49.5 years), including three men and one woman. All the patients presented with gastrointestinal symptoms, ectodermal abnormalities, and multiple gastrointestinal polyps. Two patients showed abnormal immune indices. Three patients underwent magnetic resonance enterography, and the typical manifestations of small intestine involvement were diffuse wall thickening, high signal intensity on diffusion-weighted imaging, obvious enhancement, and multiple small nodular enhancements of the small intestine. The main histological manifestations were chronic inflammation and hyperplastic, adenomatoid, and hamartomatoid polyps. Eosinophilic infiltration was observed in two patients. One patient had rectal adenocarcinoma at the time of diagnosis. All the four patients received prednisone at a dose of 0.75-1 mg/kg/day, and had their gastrointestinal symptoms gradually resolved (including two with ectodermal abnormality and endoscopic remission). Two patients are currently receiving low-dose prednisone (2.5-5 mg/day) with no recurrence after a 1.5- and 6-year follow-up periods, respectively. Conclusion Magnetic resonance enterography has the potential to evaluate small-intestinal lesions in CCSs. Long-term therapy with low doses of prednisone may be beneficial in maintaining remission.