Near-total intestinal aganglionosis in the Waardenburg-Shah syndrome

被引：14
作者：
Shim, WKT
Derieg, M
Powell, BR
Hsia, YE
机构：
[1] Univ Hawaii, Kapiolani Med Ctr Women & Children, Honolulu, HI 96822 USA
[2] Univ Hawaii, John A Burns Sch Med, Dept Surg, Honolulu, HI 96822 USA
[3] Univ Hawaii, John A Burns Sch Med, Dept Pediat, Honolulu, HI 96822 USA
[4] Univ Hawaii, John A Burns Sch Med, Dept Genet, Honolulu, HI 96822 USA
来源：
JOURNAL OF PEDIATRIC SURGERY | 1999年 / 34卷 / 12期
关键词：
total aganglionosis; Hirschprung's disease; Waardenburg-Shah syndrome;
D O I：
10.1016/S0022-3468(99)90330-5
中图分类号：
R72 [儿科学];
学科分类号：
100202 ;
摘要：
Both pigmentation and otic defects of Waardenburg Syndrome and Hirschsprung's disease have a common origin in neural crest cells and were described in 1951 and 1887, respectively. The clinical manifestations of both in the same patient were described in 1981 in 12 infants so afflicted. The authors present such a case of long segment aganglionosis in a 15-day-old Marshallese girl with Waardenburg-Shah syndrome and discuss diagnosis, treatment, and prognosis. J Pediatr Surg 34:1853-1855. Copyright (C) 1999 by W.B. Saunders Company.
引用
收藏
页码：1853 / 1855
页数：3
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