Biliary atresia

PKH Tam, RG Wells, CSM Tang, VCH Lui… - Nature Reviews …, 2024 - nature.com
Biliary atresia (BA) is a progressive inflammatory fibrosclerosing disease of the biliary
system and a major cause of neonatal cholestasis. It affects 1: 5,000–20,000 live births, with …

Targeted analysis of the size distribution of heavy chain-modified hyaluronan with solid-state nanopores

DA Erxleben, RJ Dodd, AJ Day, DE Green… - Analytical …, 2024 - ACS Publications
The glycosaminoglycan hyaluronan (HA) plays important roles in diverse physiological
functions where the distribution of its molecular weight (MW) can influence its behavior and …

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

IEM de Jong, RG Wells - Pediatric and Developmental …, 2024 - journals.sagepub.com
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 …

[HTML][HTML] Biliary atresia susceptibility gene EFEMP1 regulates extrahepatic bile duct elastic fiber formation and mechanics

K Gupta, J Llewellyn, E Roberts, C Liu, A Naji… - JHEP Reports, 2025 - Elsevier
Background & Aims EGF-containing fibulin extracellular matrix protein 1 (EFEMP1, also
called fibulin-3) is an extracellular matrix protein linked in a genome-wide association study …

[HTML][HTML] Microcystin-RR is a biliary toxin selective for neonatal cholangiocytes

K Gupta, D Chen, RG Wells - bioRxiv, 2023 - ncbi.nlm.nih.gov
Microcystin-RR is a biliary toxin selective for neonatal cholangiocytes - PMC Back to Top Skip
to main content NIH NLM Logo Access keys NCBI Homepage MyNCBI Homepage Main …

Transcriptional analysis of murine biliary atresia identifies macrophage heterogeneity and subset-specific macrophage functions

KD Gromer, SY Chen, G Gadhvi, L Feng… - Frontiers in …, 2025 - frontiersin.org
Introduction Macrophages play an important role in disease progression of pediatric
cholestatic liver disease, particularly biliary atresia (BA); however, the restorative versus …

Silencing of maternally expressed RNAs in Dlk1-Dio3 domain causes fatal vascular injury in the fetal liver

H Yu, Y Zhao, R Cheng, M Wang, X Hu… - Cellular and Molecular …, 2024 - Springer
Abstract The mammalian imprinted Dlk1-Dio3 domain contains multiple lncRNAs, mRNAs,
the largest miRNA cluster in the genome and four differentially methylated regions (DMRs) …

Low-dose biliatresone treatment of pregnant mice causes subclinical biliary disease in their offspring: Evidence for a spectrum of neonatal injury

K Gupta, JP Xu, T Diamond, IEM de Jong, A Glass… - Plos one, 2024 - journals.plos.org
Biliary atresia is a neonatal disease characterized by damage, inflammation, and fibrosis of
the liver and bile ducts and by abnormal bile metabolism. It likely results from a prenatal …

[HTML][HTML] Microcystin-RR is a biliary toxin selective for neonatal extrahepatic cholangiocytes

K Gupta, D Chen, RG Wells - JHEP Reports, 2025 - Elsevier
Background & Aims Biliary atresia is a fibrosing cholangiopathy affecting neonates that is
thought to result from a prenatal environmental insult to the bile duct. Biliatresone, a plant …

Biliatresone treatment of pregnant mice causes changes in bile metabolism and liver inflammation in their offspring

K Gupta, JP Xu, T Diamond, I De Jong, A Glass… - BioRxiv, 2023 - biorxiv.org
Abstract Background & Aims Biliary atresia is a neonatal disease characterized by bile duct
and liver damage, fibrosis, inflammation and abnormal bile metabolism. It appears to result …