Ductal plate malformation in patients with biliary atresia

Abstract

Early onset of jaundice, regardless of DPM histology, was the most ominous sign of poor outcome in infants with BA after HPE.

Impact of ductal plate malformation on survival with native liver in children with biliary atresia

“…The finding of DPM-like arrays in BA suggests an early developmental onset for the disease [3,5]. In our series, DPM was seen in all children with BASM and 49 % without.…”

“…The overall incidence of DPM reported in most series is 20-50 % [3,5,7,8], but these studies were done with single wedge biopsies of the liver taken at the time of KPE in which the large portal tracts are underrepresented leading to lower incidence. In our series, which looked at explant liver sections, it was high at 52 % because multiple sections with large portal tracts were available for study and, definitive and possible DPM-like arrays as defined by Pacheco were included.…”

“…These cells are arranged around the portal vein in a circular fashion and differentiate to form the primitive ductal structure finally maturing into tubular ducts [5]. Arrest in the differentiation process leads to persistence of an embryonic structure known as DPM [2].…”

“…Bipotential hepatic progenitor cells differentiate into periportal hepatocytes and cholangiocytes when they come into contact with the mesenchyme of the portal vein [2,5].…”

“…DPM ductal plate malformation, LT liver transplantation, PELD pediatric-end stage liver disease Pediatr Surg Int in children who underwent KPE[5]. This information was not available in our series since all children had KPE in different centers.…”