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克朗凯特-加拿大综合征必然是一种晚发性疾病吗?

Is Cronkhite-Canada Syndrome necessarily a late-onset disease?

作者信息

Vernia Piero, Marcheggiano Adriana, Marinaro Vanessa, Morabito Santo, Guzzo Isabella, Pierucci Alessandro

机构信息

Gastroenterology Unit, Department of Clinical Science, University of Rome La Sapienza, Rome, Italy.

出版信息

Eur J Gastroenterol Hepatol. 2005 Oct;17(10):1139-41. doi: 10.1097/00042737-200510000-00022.

Abstract

Cronkhite-Canada Syndrome is a non-inherited, non-congenital disease characterized by juvenile hamartomatous gastrointestinal polyps with a typically late onset. In the case described herein the disease was diagnosed in a 17-year-old male with type I diabetes and thalassaemia minor, in coincidence with severe symptomatic intestinal candidiasis. Following the disappearance of the mycosis and correction of the protein and electrolyte imbalance, the ectodermal abnormalities returned to normal and the patient remained asymptomatic during a 7-year follow-up period, despite proteinuria resulting from membranous glomerulopathy. The concept that Cronkhite-Canada Syndrome is a late-onset disease should probably be reconsidered as it may remain asymptomatic, and thus not diagnosed, for a long a time.

摘要

克朗凯特-加拿大综合征是一种非遗传性、非先天性疾病,其特征为青少年错构瘤性胃肠道息肉,通常起病较晚。在本文所述病例中,一名患有I型糖尿病和轻度地中海贫血的17岁男性被诊断出患有该疾病,同时伴有严重的症状性肠道念珠菌病。随着真菌病消失以及蛋白质和电解质失衡得到纠正,外胚层异常恢复正常,并且在7年的随访期内患者一直无症状,尽管存在膜性肾小球病导致的蛋白尿。克朗凯特-加拿大综合征是一种起病较晚的疾病这一概念可能需要重新考虑,因为它可能在很长一段时间内无症状,因而未被诊断出来。

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