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一例加拿大克朗凯特综合征:采用抗幽门螺杆菌疗法治疗后病情缓解。

A case of Cronkhite-Canada syndrome: remission after treatment with anti-Helicobacter pylori regimen.

作者信息

Okamoto Kenta, Isomoto Hajime, Shikuwa Saburo, Nishiyama Hitoshi, Ito Masahiro, Kohno Shigeru

机构信息

Department of Internal Medicine, Nagasaki National Medical Center, Omura, Japan.

出版信息

Digestion. 2008;78(2-3):82-7. doi: 10.1159/000165354. Epub 2008 Oct 24.

DOI:10.1159/000165354
PMID:18948692
Abstract

A 67-year-old man with nausea, appetite loss, frequent diarrhea and severe weight loss presented with alopecia, skin hyperpigmentation and onychodystrophy. Laboratory investigations showed mild anemia, hypoproteinemia and hypoalbuminemia. Colonoscopy identified the numerous, hyperemic and sessile polyps with mucous exudation of various sizes throughout the colorectum. The ileocecal valve was substantially swollen. Magnified chromoendoscopy revealed sparsely distributed crypt openings with widening of the preicryptal space without destruction in the affected lesions. Upper gastrointestinal endoscopy revealed multiple small, reddish, and sessile polyps in the duodenum and Helicobacter pylori-associated gastritis. Histopathological examination of the colonic polyps revealed cystic dilatation and elongation of scattered glands with epithelial hyperplasia and stromal edema and inflammatory cell infiltrates. Thus, a diagnosis of Cronkhite-Canada syndrome was made. The patient was given clarithromycin, amoxicillin and lansoprazole, resulting in negative (13)C-urea breath tests. Three months later, his clinical symptoms and edema of the legs resolved with normalization of serum total protein and albumin levels and return to his previous body. The ectodermal abnormalities were resolved 8 months later. On repeat colonoscopic examinations, there was progressive remission of the duodenal and colorectal polyposis, leaving scattered pedunculated polyps in the transverse and ascending colon and on the almost normal-appearing ileocecal valve. At the follow-up magnifying endoscopic examination 8 months later, small round or round-oval pits were densely and regularly distributed.

摘要

一名67岁男性,有恶心、食欲减退、频繁腹泻及严重体重减轻症状,伴有脱发、皮肤色素沉着和甲营养不良。实验室检查显示轻度贫血、低蛋白血症和低白蛋白血症。结肠镜检查发现整个结直肠有大量大小不一、充血的无蒂息肉并有黏液渗出。回盲瓣明显肿胀。放大色素内镜检查显示在受累病变处隐窝开口稀疏分布,隐窝前间隙增宽但无破坏。上消化道内镜检查发现十二指肠有多个小的、红色的无蒂息肉以及幽门螺杆菌相关性胃炎。结肠息肉的组织病理学检查显示散在腺体呈囊性扩张和延长,伴有上皮增生、间质水肿和炎性细胞浸润。因此,诊断为克朗凯特 - 加拿大综合征。给予患者克拉霉素、阿莫西林和兰索拉唑治疗,(13)C - 尿素呼气试验结果转为阴性。三个月后,他的临床症状和腿部水肿消退,血清总蛋白和白蛋白水平恢复正常,体重恢复至先前水平。8个月后外胚层异常症状消失。在重复结肠镜检查时,十二指肠和结直肠息肉病逐渐缓解,仅在横结肠和升结肠以及外观几乎正常的回盲瓣上留下散在的带蒂息肉。8个月后的随访放大内镜检查显示小圆形或椭圆形凹坑密集且规则分布。

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