Murata Kazumoto, Sato Kiichi, Okada Shinya, Suto Daisuke, Otake Takaaki, Kohgo Yutaka
Division of Virology, Department of Infection and Immunity, Jichi Medical University School of Medicine, Shimotsuke, Japan.
Department of Gastroenterology, International University of Health and Welfare, Nasushiobara, Japan.
Case Rep Gastroenterol. 2020 Oct 30;14(3):561-569. doi: 10.1159/000510920. eCollection 2020 Sep-Dec.
Cronkhite-Canada syndrome (CCS) is a rare disease characterized by diffuse gastrointestinal polyposis with chronic diarrhea and ectodermal change, but its etiology is unknown. We present a case at the age of 26 years complaining of epigastralgia and weight loss. Endoscopic examination revealed extensive diffuse polypoid lesions of the stomach and the terminal ileum, all of which showed hyperplastic polyps pathologically. There were no polypoid lesions in his colon. He has no family history of diffuse gastrointestinal polyposis. Diffuse gastrointestinal hyperplastic polyposis without any hereditary association led us to suspect this case as CCS although he did not show chronic diarrhea and any ectodermal symptoms such as onychodystrophy, alopecia, and hyperpigmentation. After initiation of a corticosteroid therapy, his epigastralgia disappeared and he gained appetite and weight, accompanied by normalization of serum albumin levels. Endoscopic examination 1 year after initiation of corticosteroid therapy revealed a decrease in the number of gastric polyposis and those inflammations. This rare young case may suggest that early therapeutic intervention with corticosteroids could improve the prognosis of CCS, preventing not only malnutrition but also appearance of several ectodermal symptoms.
克朗凯特-加拿大综合征（CCS）是一种罕见疾病，其特征为弥漫性胃肠道息肉病伴慢性腹泻和外胚层改变，但其病因尚不清楚。我们报告一例26岁主诉上腹痛和体重减轻的病例。内镜检查发现胃和回肠末端有广泛的弥漫性息肉样病变，病理检查均显示为增生性息肉。其结肠未见息肉样病变。他没有弥漫性胃肠道息肉病的家族史。尽管该患者未表现出慢性腹泻以及任何外胚层症状，如甲营养不良、脱发和色素沉着，但无任何遗传关联的弥漫性胃肠道增生性息肉病使我们怀疑该病例为CCS。开始使用皮质类固醇治疗后，他的上腹痛消失，食欲和体重增加，血清白蛋白水平恢复正常。皮质类固醇治疗开始1年后的内镜检查显示胃息肉病数量及炎症有所减少。这个罕见的年轻病例可能表明，早期使用皮质类固醇进行治疗干预可以改善CCS的预后，不仅可以预防营养不良，还可以防止出现多种外胚层症状。
