Colostomy Delays Cell Loss in the Brain and Improves Juvenile Survival in a Neonatal Rat Model of Hirschsprung's Disease.

Hirschsprung's disease is a congenital malformation characterized by the absence of enteric ganglia in the distal intestine and gut obstruction. Our previous study indicates the brain pathology during the disease progression. A subpopulation of Hirschsprung's disease patients is also associated with anomalies of the central nervous system.

Structural heart defects associated with ET mutation, a cause of Hirschsprung disease.

Background: HSCR, a colonic neurocristopathy affecting 1/5000 births, is suggested to associate with cardiac septal defects and conotruncal malformations. However, we question subtle cardiac changes maybe more commonly present due to multi-regulations by HSCR candidate genes, in this instance, ET. To investigate, we compared the cardiac morphology and quantitative measurements of sl/sl rat to those of the control group.

Brain size reductions associated with endothelin B receptor mutation, a cause of Hirschsprung's disease.

Background: ET has been reported to regulate neurogenesis and vasoregulation in foetal development. Its dysfunction was known to cause HSCR, an aganglionic colonic disorder with syndromic forms reported to associate with both small heads and developmental delay. We therefore asked, "is CNS maldevelopment a more general feature of ET mutation?" To investigate, we reviewed the micro-CT scans of an ET model animal, sl/sl rat, and quantitatively evaluated the structural changes of its brain constituents.

Reduced cell proliferation and increased apoptosis in the hippocampal formation in a rat model of Hirschsprung's disease.

Hirschsprung's disease (HSCR) is a congenital malformation characterized by the absence of enteric ganglia in the distal intestine and gut obstruction. Some HSCR patients also have associated neurological symptoms. We studied a rat model of HSCR, also known as spotting lethal (sl/sl) rat, which carries a spontaneous deletion in the gene of endothelin receptor B (EDNRB) and a similar phenotype as humans with HSCR.

Predictors of slow colonic transit in children.

Purpose: Slow transit constipation (STC) and functional fecal retention (FFR) are two forms of severe intractable constipation in childhood diagnosed by nuclear transit studies (NTS). This retrospective study aims to identify the predicting factors for STC and FFR by looking at the association with neuropsychiatric disorders (NPD), obesity, family history of constipation and atopic disease.

Patients And Methods: A retrospective chart review was conducted on children with intractable constipation referred for NTS between 1st April 2003 and 1st April 2014.