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伴有恶病质的幼年性息肉。1例婴儿报告及与成人克朗凯特-加拿大综合征的比较。

Juvenile polyps with cachexia. Report of an infant and comparison with Cronkhite-Canada syndrome in adults.

作者信息

Ruymann F B

出版信息

Gastroenterology. 1969 Oct;57(4):431-8.

PMID:4378049
Abstract

Juvenile polyps occur in adults and infants; where multiple, they may be complicated by progressive cachexia with hypoalbuminemia and electrolyte depletion. We report a fatal case of multiple juvenile polyposis with cachexia, alopecia, and megalocephaly presenting in a 9-month-old infant, and review 2 additional cases in infancy. A similar syndrome in adults had been described by Cronkhite and Canada, with intestinal polyposis, nail dystrophy, hyperpigmentation, and alopecia. Histological examination of a polyp from one case of Cronkhite-Canada syndrome suggests a juvenile rather than adenomatous pathology. Multiple juvenile polyps can cause devastating enteropathy and cachexia.

摘要

幼年性息肉可发生于成人和婴儿;若为多发性,可能会并发进行性恶病质,伴有低白蛋白血症和电解质耗竭。我们报告了一例9个月大婴儿出现多发性幼年性息肉病并伴有恶病质、脱发和巨头畸形的致命病例,并回顾了另外2例婴儿期病例。成人中类似的综合征曾由克朗凯特和加拿大描述过,伴有肠道息肉病、指甲营养不良、色素沉着和脱发。对一例克朗凯特-加拿大综合征病例的息肉进行组织学检查显示为幼年性而非腺瘤性病理改变。多发性幼年性息肉可导致严重的肠病和恶病质。

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