Hirschsprung's associated enterocolitis.

ABSTRACT:

Purpose of review

Hirschsprung's disease (HSCR) is characterized by an absence of ganglion cells in the distal hindgut, extending from the rectum to a variable distance proximally, and results from a failure of cranial-caudal neural crest cell migration. Hirschsprung's-associated enterocolitis (HAEC) is a condition with classic manifestations that include abdominal distention, fever and foul-smelling stools, and is a significant and life-threatening complication of HSCR. The purpose of this review was to critically evaluate recent findings regarding the pathophysiology of HAEC.

Recent findings

Several recent studies have investigated the cause of HAEC in humans and mouse models. These studies suggest that alterations in the intestinal barrier, including goblet cell number an

SUBMITTER: Gosain A 

PROVIDER: S-EPMC4451822 | biostudies-literature | 2015 Jun

REPOSITORIES: biostudies-literature