In Utero Extrahepatic Bile Duct Damage and Repair: Implications for Biliary Atresia.

Autor:	de Jong IEM; Department of Medicine, University of Pennsylvania, Philadelphia, PA, USA.; Center for Engineering MechanoBiology, University of Pennsylvania, Philadelphia, PA, USA., Wells RG; Department of Medicine, University of Pennsylvania, Philadelphia, PA, USA.; Center for Engineering MechanoBiology, University of Pennsylvania, Philadelphia, PA, USA.; Department of Pathology and Laboratory Medicine, University of Pennsylvania, Philadelphia, PA, USA.; Department of Bioengineering, University of Pennsylvania, Philadelphia, PA, USA.
Jazyk:	angličtina
Zdroj:	Pediatric and developmental pathology : the official journal of the Society for Pediatric Pathology and the Paediatric Pathology Society [Pediatr Dev Pathol] 2024 Jul-Aug; Vol. 27 (4), pp. 291-310. Date of Electronic Publication: 2024 May 19.
DOI:	10.1177/10935266241247479
Abstrakt:	Biliary atresia (BA) is a cholangiopathy affecting the extrahepatic bile duct (EHBD) of newborns. The etiology and pathophysiology of BA are not fully understood; however, multiple causes of damage and obstruction of the neonatal EHBD have been identified. Initial damage to the EHBD likely occurs before birth. We discuss how different developmental stages in utero and birth itself could influence the susceptibility of the fetal EHBD to damage and a damaging wound-healing response. We propose that a damage-repair response of the fetal and neonatal EHBD involving redox stress and a program of fetal wound healing could-regardless of the cause of the initial damage-lead to either obstruction and BA or repair of the duct and recovery. This overarching concept should guide future research targeted toward identification of factors that contribute to recovery as opposed to progression of injury and fibrosis. Viewing BA through the lens of an in utero damage-repair response could open up new avenues for research and suggests exciting new therapeutic targets. Competing Interests: Declaration of Conflicting InterestsThe authors declared no potential conflicts of interest with respect to the research, authorship, and/or publication of this article.
Databáze:	MEDLINE
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