Hirschsprung's disease in children.
Several advances in understanding Hirschsprung's disease have developed in the past year. A greater understanding of the etiology of the disease has developed through the association of Hirschsprung's disease with a lack of neural cell adhesion molecule expression and nitric oxide in aganglionic portions of the bowel. It appears that a loss of neural cell adhesion molecule could be the cause of neurocytes failing to migrate to aganglionic regions, and a loss of nitric oxide could well explain the spasticity associated with the aganglionic region. Another advance has come from the analysis of genetic deletions associated with some forms of Hirschsprung's disease. Surgical advances have primarily been related to the increased use of a primary pull-through procedure in the newborn period. Results of this application have led to excellent outcomes without the need for a decompressive colostomy.
Teitelbaum
D H
DH
University of Michigan Hospital, Ann Arbor, USA.
D002648:Child; D002675:Child, Preschool; D002872:Chromosome Deletion; D002879:Chromosomes, Human, Pair 10; D003106:Colon / Q000601:surgery; D003248:Constipation / Q000209:etiology; D004314:Down Syndrome / Q000150:complications; D004760:Enterocolitis / Q000150:complications; D006627:Hirschsprung Disease / Q000209:etiology / Q000235:genetics / Q000601:surgery*; D006801:Humans; D007223:Infant; D007231:Infant, Newborn; D011183:Postoperative Complications; D012007:Rectum / Q000601:surgery