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伴有结肠癌、门静脉血栓形成、高滴度抗核抗体及膜性肾小球肾炎的克朗凯特-加拿大综合征

Cronkhite-Canada syndrome with colon cancer, portal thrombosis, high titer of antinuclear antibodies, and membranous glomerulonephritis.

作者信息

Takeuchi Yoji, Yoshikawa Masahide, Tsukamoto Noboru, Shiroi Akira, Hoshida Yoshihiko, Enomoto Yasuhiro, Kimura Toshiaki, Yamamoto Kazuo, Shiiki Hideo, Kikuchi Eiryo, Fukui Hiroshi

机构信息

Department of Internal Medicine, Division of Gastroenterology, Belland General Hospital, Sakai, Japan.

出版信息

J Gastroenterol. 2003;38(8):791-5. doi: 10.1007/s00535-002-1148-6.

Abstract

A 64-year-old man, who came to us with diarrhea, presented with ectodermal changes such as hyperpigmentation, alopecia, and onychatrophy, and was affected by polyposis in the colorectum and stomach. The polyps were histologically consistent with those in Cronkhite-Canada syndrome (CCS). Interestingly, the patient also had colon cancer, as well as portal thrombosis and a high concentration of antinuclear antibody. Treatment with prednisolone ameliorated the symptoms and the gastrointestinal polyposis, while the cancer was successfully treated with a hemicolectomy. Six months after the surgery, the patient developed nephropathy, with nephrotic-range proteinuria, without recurrence of the cancer. The biopsied renal specimen showed membranous glomerulonephritis. This is a rare case of CCS associated with various complications such as colon cancer, portal vein thrombosis, a high titer of antinuclear antibodies, and membranous glomerulonephritis. Although the pathogenesis of CCS is essentially unknown, these complications might have been indicative of an underlying immunological abnormality.

摘要

一名64岁男性因腹泻前来就诊,伴有色素沉着、脱发和甲萎缩等外胚层改变,且患有结直肠和胃的息肉病。这些息肉在组织学上与克朗凯特-加拿大综合征(CCS)的息肉一致。有趣的是,该患者还患有结肠癌,同时伴有门静脉血栓形成和高浓度抗核抗体。泼尼松龙治疗改善了症状和胃肠道息肉病,而癌症通过半结肠切除术成功治疗。术后6个月,患者出现肾病,伴有肾病范围蛋白尿,癌症未复发。肾活检标本显示为膜性肾小球肾炎。这是一例罕见的与结肠癌、门静脉血栓形成、高滴度抗核抗体和膜性肾小球肾炎等多种并发症相关的CCS病例。尽管CCS的发病机制基本不明,但这些并发症可能提示存在潜在的免疫异常。

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