Familial ganglioneuromatous polyposis of the large bowel: Report of a family with associated juvenile polyposis
Familial ganglioneuromatous polyposis of the large bowel: Report of a family with associated juvenile polyposis
复制标题
家族性大肠神经节神经瘤性息肉病：一个伴有幼年性息肉病的家庭报告
DOI：
10.1097/00000478-198407000-00003
复制
发表时间：
1984
期刊：
影响因子：
--
通讯作者：
M. Diamond
中科院分区：
文献类型：
--
作者：
G. Mendelsohn;M. Diamond
本文报告一种独特的大肠息肉样肠神经节细胞瘤病的家族性发病。病变影响了一位38岁的父亲和三个14岁、12岁和10岁的孩子。在这些病例中，结肠粘膜内神经纤维和神经节细胞的广泛弥漫性和息肉样增生与类似青少年息肉病的明显上皮变化相关。所有患者均无任何粘膜神经瘤综合征或Von Recklinghausen神经纤维瘤病的临床证据，肠神经节细胞神经瘤病可能发生。青少年型息肉是否是错构瘤或是否代表一个反应过程的问题得到解决。
The unique familial occurrence of polypoid intestinal ganglioneuromatosis of the large bowel is reported. The lesions affected a 38-year-old father and three children aged 14, 12, and 10 years. In these cases, extensive diffuse and polypoid proliferation of nerve fibers and ganglion cells within the colonic mucosa was associated with pronounced epithelial changes resembling juvenile polyposis. None of the patients had any clinical evidence of the mucosal neuroma syndrome or Von Recklinghausen's neurofibromatosis, conditions in which intestinal ganglioneuromatosis can occur. The question of whether the juvenile-type polyps are hamartomatous or whether they represent a reactive process is addressed.