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

ABSTRACT: 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. In the investigation, we studied a rat model of Hirschsprung's disease, known as spotting lethal (sl/sl) ET_B^-/- rats, which carries a spontaneous deletion in endothelin receptor B (human gene name: EDNRB) and manifests a similar phenotype as humans with Hirschsprung's disease. Homozygous mutant sl/sl rats were successfully rescued from premature death by performing colostomy and dramatically surviv

SUBMITTER: Xie D 

PROVIDER: S-EPMC10719028 | biostudies-literature | 2022

REPOSITORIES: biostudies-literature