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Predicting and managing liver fibrosis in biliary atresia.
Semin Pediatr Surg
January 2025
Section of Pediatric Surgery, Pediatric Liver and Gut Research Group, New Children's Hospital, University of Helsinki and Helsinki University Hospital, Helsinki, Finland; Pediatric Research Center, Children's Hospital, University of Helsinki and Helsinki University Hospital, Helsinki, Finland; Department of Women's and Children's Health, Karolinska Institute, Stockholm, Sweden. Electronic address:
Regardless of the underlying etiology and success of PE, progressive liver fibrosis and eventually cirrhosis represent the dominant pathology and the end-stage of BA. Ascending bile duct injury-induced cholestasis, inflammation and ductular reaction provide profibrogenic cytokine environment leading to myofibroblast activation and rapid progression of fibrosis especially after unsuccessful portoenterostomy. Although liver fibrosis and development of cirrhosis play a crucial role in determining BA outcomes, the exact prognostic significance and dynamics of mild to moderate liver fibrosis remain unclear.
View Article and Find Full Text PDFPredicting and optimising outcome for biliary atresia.
Semin Pediatr Surg
January 2025
Dept of Paediatric Surgery, Kings College Hospital, London SE59RS, England, United Kingdom. Electronic address:
Biliary atresia (BA) remains a disease of significant morbidity and mortality world-wide. Early and accurate diagnosis facilitates early intervention and improves outcomes. The gold standard in diagnosing BA is a liver biopsy followed by cholangiography, usually performed intra-operatively.
View Article and Find Full Text PDFRevision Kasai portoenterostomy: A review of indications and outcomes.
Semin Pediatr Surg
January 2025
Cincinnati Children's Hospital Medical Center, United States. Electronic address:
The Kasai portoenterostomy (KPE) can provide a surgical cure for children with biliary atresia (BA), without the need for a liver transplant (OLTxp). Revision KPE can be attempted following a failed initial KPE where biliary clearance is not achieved. The most common indications for revision KPE are recurrent jaundice or recurrent cholangitis, although it has also been performed for persistent jaundice or bile lakes.
View Article and Find Full Text PDFVitamin D3/VDR alleviates double-stranded RNA virus -induced biliary epithelial cell damage by inhibiting autophagy.
BMC Gastroenterol
January 2025
Department of Pediatrics, First Affiliated Hospital of Xi'an Jiaotong University, Xi'an, Shaanxi Province, 710061, China.
Background: The increased apoptosis of bile duct epithelial cells (BECs) due to some damage factors is considered the initiating factor in the occurrence and progression of biliary atresia (BA). Vitamin D receptor (VDR) is thought to play a crucial role in maintaining the intrinsic immune balance and integrity of bile duct epithelial cells (BECs). To investigate the role of VDRs in the pathogenesis and progression of BA using in vitro and in vivo models.
View Article and Find Full Text PDFIncreased serum GM-CSF at diagnosis of biliary atresia is associated with improved biliary drainage.
Pediatr Res
January 2025
Department of Pediatrics, Medical College of Wisconsin, Children's Wisconsin, Milwaukee, WI, USA.
Background: The immune heterogeneity of biliary atresia (BA) presents a challenge for development of prognostic biomarkers. This study aimed to identify early immune signatures associated with biliary drainage after Kasai Portoenterostomy (KPE).
Methods: Serum samples, liver slides, and clinical data were obtained from patients enrolled in the NIDDK-supported Childhood Liver Disease Research Network.
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