Cronkhite-Canada syndrome with colon cancer, portal thrombosis, high titer of antinuclear antibodies, and membranous glomerulonephritis
Cronkhite-Canada syndrome with colon cancer, portal thrombosis, high titer of antinuclear antibodies, and membranous glomerulonephritis
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DOI：
10.1007/s00535-002-1148-6
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发表时间：
2003-01-01
影响因子：
6.3
通讯作者：
Fukui, H
中科院分区：
文献类型：
--
作者：
Takeuchi, Y;Yoshikawa, M;Fukui, H
一位64岁的男性，因腹泻来找我们，表现为色素沉着过度、脱发和甲萎缩等外胚层变化，并受到结肠直肠和胃息肉病的影响。息肉的组织学上与Cronkhite-Canada综合征（CCS）一致。有趣的是，该患者还患有结肠癌，以及门静脉血栓形成和高浓度的抗核抗体。泼尼松龙治疗改善了症状和胃肠道息肉病，而癌症成功地治疗了半结肠切除术。手术后6个月，患者出现肾病，伴肾病范围蛋白尿，但未复发。肾活检标本显示膜性肾小球肾炎。这是一个罕见的CCS病例，合并有多种并发症，如结肠癌、门静脉血栓形成、高滴度抗核抗体和膜性肾小球肾炎。虽然CCS的发病机制基本上是未知的，这些并发症可能是一个潜在的免疫异常的指示。
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.