Outcome after Portoenterostomy in Biliary Atresia: Pivotal Role of Degree of Liver Fibrosis and Intensity of Stellate Cell Activation

Abstract

Intense liver fibrogenesis is already established in many cases of BA at the time of KP. Fibrosis scores and intensity of alpha-SMA expression may be predictors of outcome after KP and may indicate those patients who might benefit from trials of potential antifibrotic agents early in the course of BA.

Genome-wide association study identifies a susceptibility locus for biliary atresia on 10q24.2

“…Increased actin and myosin deposition around bile canaliculi has been observed in BA patients who did not exhibit bile flow after surgery (27). Also, the expression intensity of a-smooth-muscle actin is correlated with the degree of fibrosis in patients with BA (28).…”

Screening and outcomes in biliary atresia

“…The sequential use of HPE followed by liver transplantation is the standard surgical paradigm followed around the world. 37,51,52 Future trends that will bear watching include the growing laparoscopic experience with the HPE procedure.…”

“…Fibrosis is variable, and its severity may be predictive of the long-term success of HPE. 37 The differential diagnosis (Table 2) of a biliary obstructive pattern in a liver biopsy of a cholestatic infant includes choledochal cysts, bile duct strictures, alpha-1 antitrypsin deficiency, total parenteral nutrition-associated cholestasis, cystic fibrosis, multiple drug resistance 3 deficiency (progressive familial intrahepatic cholestasis type III), North American Indian childhood cirrhosis (cirhin deficiency), and Alagille syndrome. Thus, differential diagnosis relies on clinical and laboratory test results in addition to the histology.…”

Analysis of Biliary Epithelial-Mesenchymal Transition in Portal Tract Fibrogenesis in Biliary Atresia

“…Among available phenotypic markers, a-SMA remains the most reliable marker of myofibroblastic cells. The source of a-SMA in biliary atresia as evidenced by immunohistochemistry and its synthesis is generally detectable in selected subpopulations of fibroblasts located around the portal area [15,16]. Sicklick et al [17], studying a single human patient with biliary atresia, noted a-SMA reactivity in structures that appeared to be bile ducts.…”