Biliary Atresia: A Clinical and Translational Science Research Workshop

Event Details

Summary

Biliary Atresia: Clinical and Research Challenges for the Twenty-First Century.

Background

Biliary atresia is a rare, but one of the most common severe pediatric liver diseases affecting approximately 1:5000 to 1:18,000 newborns. Recent intervention studies on immune modulation for biliary atresia have not yielded any substantial improvement in altering the liver disease trajectory and associated liver disease related complications. As such, biliary atresia continues to account for approximately 40-50% of pediatric liver transplantations in the United States.

Meeting Objectives

The purpose of this meeting will be to bring together clinical and translational experts in biliary atresia to examine the current state of clinical and translational science that will allow for the identification and prioritization of achievable and feasible research priorities that will further advance the understanding of the clinical pathophysiology of this condition.

Further details regarding the agenda, date and location of this meeting will be forthcoming on this website.

Organizing Committee

Ronald Sokol, M.D., University of Colorado School of Medicine
Rebecca Wells, M.D., University of Pennsylvania School of Medicine
Jorge Bezera, M.D., Cincinnati Children's Hospital Medical Center
Edward Doo, M.D., NIDDK/NIH
Jay Hoofnagle, M.D., NIDDK/NIH

Registration Deadline

June 1, 2017 or until full.

RSVP to Lauren Meskill via email with your name, affiliation and phone number.

Agenda

June 28, 2017

8:00 a.m. – 8:10 a.m.

Welcome and Introduction
Edward Doo and Ronald Sokol

Session I: Clinical Aspects and Genetics of Biliary Atresia (BA)

Moderator: Ronald Sokol

8:10 a.m. – 8:30 a.m.

Biliary atresia: A clinical challenge in the 21st Century
Ronald Sokol

8:30 a.m. – 8:50 a.m.

Clinical Variants of biliary atresia
Mark Davenport

8:50 a.m. – 9:10 a.m.

Genetic Susceptibility and Causation of BA
Nancy Spinner

9:10 a.m. – 9:30 a.m.

Prenatal versus Postnatal onset of BA: Implications for pathogenesis
Benjamin Shneider

9:30 a.m. – 9:45 a.m.

Discussion

9:45 a.m. – 10:00 a.m.

Break and Poster Viewing

Session II: Cholangiocyte Biology, Injury and Fibrosis

Moderator: Rebecca Wells

10:00 a.m. – 10:20 a.m.

Embryology and Regulation of Bile Duct Development and BA
Frederick Lemaigre

10:20 a.m. – 10:40 a.m.

Genetic Variation in the Proteostatic Stress Response - Potential BA Risk Factor
Michael Pack

10:40 a.m. – 11:00 a.m.

Biliatresone: Novel toxic injury to the cholangiocyte
Rebecca Wells

11:00 a.m. – 11:20 a.m.

Mechanisms of Fibrosis in Cholangiopathies
Mario Strazzabosco

11:20 a.m. – 11:40 a.m.

Discussion

11:40 a.m. – 12:40 p.m.

Lunch (on your own) and Poster Viewing

Session III: Inflammation and Fibrosis in Biliary Atresia

Moderator: Jorge Bezerra

12:40 p.m. – 1:00 p.m.

Role of Viruses in Biliary Atresia: From mouse models to human data
Claus Petersen

1:00 p.m – 1:20 p.m.

Immunologic Effectors of Biliary Atresia: Injury and survival signals
Jorge Bezerra

1:20 p.m. – 1:40 p.m.

Autoimmunity in Biliary Atresia
Cara Mack

1:40 p.m. – 2:00 p.m.

Liver Fibrosis in Biliary Atresia
Mikko Pakarinen

2:00 p.m.. – 2:20 p.m.

Discussion

2:20 p.m. – 2:35 p.m.

Break and Poster Viewing and Removal

Session IV: Translating Science into Action

Moderator: Saul Karpen

2:35 p.m.. – 2:55 p.m.

Novel Therapeutic Targets and Strategies
Saul Karpen

2:55 p.m – 3:15 p.m.

Epigenetic Targets in Obstructive Cholestasis
Frederick Suchy

3:15 p.m. – 3:35 p.m.

Biliary Organoids to Model Biliary Diseases
Ludovic Vallier

3:35 p.m. – 3:55 p.m.

Cell based therapeutics: Is there a role in biliary atresia?
Holger Willenbring

3:55 p.m. – 4:20 p.m.

Discussion

Session V

Moderators: Jay Hoofnagle, Ronald Sokol, Rebecca Wells, Jorge Bezerra

4:20 p.m. – 5:00 p.m.

Defining research challenges, priorities, and opportunities for biliary atresia
Audience and Moderators