影响整个结肠和直肠的分泌血管活性肠肽的神经节瘤病

Vasoactive intestinal polypeptide-secreting ganglioneuromatosis affecting the entire colon and rectum.

作者信息

Rescorla F J, Vane D W, Fitzgerald J F, West K W, Grosfeld J L

机构信息

Department of Surgery, Indiana University School of Medicine, Indianapolis.

出版信息

J Pediatr Surg. 1988 Jul;23(7):635-7. doi: 10.1016/s0022-3468(88)80634-1.

DOI:10.1016/s0022-3468(88)80634-1

PMID:2849648

Abstract

This report describes a 7-year-old boy presenting with watery diarrhea, hypokalemia, and hypochlohydria associated with vasoactive intestinal polypeptide (VIP)-secreting ganglioneuromatosis involving the entire colon and rectum. The child's symptoms resolved following proctocolectomy, and the VIP levels returned to normal. Although 55 previous children have been reported with VIP-secreting tumors, this case is the first involving the entire colon and rectum.

摘要

本报告描述了一名7岁男孩，他出现水样腹泻、低钾血症和胃酸过少，与累及整个结肠和直肠的分泌血管活性肠肽（VIP）的神经节瘤病有关。患儿的症状在直肠结肠切除术后得到缓解，VIP水平恢复正常。尽管此前已有55例儿童被报道患有分泌VIP的肿瘤，但该病例是首例累及整个结肠和直肠的。