期刊
SEMINARS IN PEDIATRIC SURGERY
卷 19, 期 3, 页码 194-200出版社
ELSEVIER SCIENCE INC
DOI: 10.1053/j.sempedsurg.2010.03.004
关键词
Hirschprungs disease; Aganglionosis; Etiology; Genetics
资金
- Hong Kong Research Grants Council [HKU 765407M, HKU 775907M]
- Action Medical Research
- Medical Research Council
- Children's Research Fund
Hirschsprung's disease (HSCR) is characterized by absence of the enteric nervous system in a variable portion of the distal gut Affected infants usually present in the days after birth with bowel obstruction Despite surgical advances. long-term outcomes remain variable In the last 2 decades, great advances have been made in understanding the genes and molecular biological mechanisms that underlie the disease In addition, our understanding of normal enteric nervous system development and how motility develops in the developing fetus and infant has also increased This review aims to draw these strands together to explain the developmental and biological basis of HSCR, and how this knowledge may be used in the future to aid children with HSCR Crown Copyright (C) 2010 Published by Elsevier Inc All rights reserved
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