Hirschsprung-Associated Enterocolitis: Transformative Research from Bench to Bedside.
Zhen ZhangBo LiQian JiangQi LiAgostino PierroLong LiPublished in: European journal of pediatric surgery : official journal of Austrian Association of Pediatric Surgery ... [et al] = Zeitschrift fur Kinderchirurgie (2022)
Hirschsprung disease (HSCR) is a congenital disease that is characterized by the absence of intrinsic ganglion cells in the submucosal and myenteric plexuses of the distal colon and is the most common cause of congenital intestinal obstruction. Hirschsprung-associated enterocolitis (HAEC) is a life-threatening complication of HSCR, which can occur either before or after surgical resection of the aganglionic bowel. Even though HAEC is a leading cause of death in HSCR patients, its etiology and pathophysiology remain poorly understood. Various factors have been associated with HAEC, including the mucus barrier, microbiota, immune function, obstruction of the colon, and genetic variations. In this review, we examine our current mouse model of HAEC and how it informs our understanding of the disease. We also describe current emerging research that highlights the potential future of HAEC treatment.
Keyphrases
- mouse model
- end stage renal disease
- chronic kidney disease
- induced apoptosis
- ejection fraction
- prognostic factors
- low birth weight
- cell cycle arrest
- gene expression
- genome wide
- minimally invasive
- risk assessment
- cell proliferation
- copy number
- endoplasmic reticulum stress
- replacement therapy
- combination therapy
- pi k akt