Biliary Atresia Part II
With Dr. George Bezerra & Dr. Mark Davenport & Dr. Greg Tiao & Dr. Atsuyuki Yamataka · hosted by Dr. Ray Henke · StayCurrentMD
Part of
Biliary Atresia 26 items
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Educational content from recorded physician discussions — not medical advice. Talk to your (or your child's) care team about your situation.
Video
Error Traps and Culture of Safety in Biliary Atresia
CCHMC Pediatric Surgery · 4 min · Published Nov 2019
Video
Prof. Shilpa Sharma - Best of the Best in Pediatric Surgery 2024
Dr. Todd Ponsky · 5 min · Published Feb 2024
Video
Biliary Atresia with Dr. Greg Tiao
CCHMC Pediatric Surgery · 11 min · Published Jun 2022
Video
Biliary Atresia - Clinical Practice Updates
Published Dec 2020
Video
Biliary Atresia: Where are we now? Advanced Practice Providers Pediatric...
Dr. Todd Ponsky · 48 min · Published Jul 2017
Podcast
Biliary Atresia Part I
31 min · Published Jan 2022
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Pediatric Surgical Oncology Research Collaborative (PSORC): Studying Rare Pediatric Tumors
56 s · Published May 2026
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Update Course Rewind 2025: Hirschsprung + ARM: Rare but Real
1 min · Published May 2026
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Update Course Rewind 2025: Hirschsprung + ARM: Rare but Real
1 min · Published May 2026
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Pooling Patients to Study Rare Pediatric Tumors: An Introduction to PSORC
56 s · Published May 2026
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The fetal frontier: A review of current and emerging fetal therapies for genetic diseases
44 s · Published May 2026
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Indocyanine green assists with sentinel lymph node mapping in pediatric and adolescent patients
1 min · Published May 2026
What the experts said
American Academy of Pediatrics recommends fractionation of bilirubin for any baby with persistent jaundice beyond two weeks of age, with referral to pediatric gastroenterologist if direct bilirubin is elevated
Cincinnati protocol: ultrasound and blood work same day, alpha-1 antitrypsin testing, liver biopsy if alpha-1 normal and acholic stool present, decision on Kasai within 5-7 days
Parents reporting normal stool color must show the stool for verification, as many parents misidentify acholic stools as normal
MMP7 (matrix metalloprotein 7) ELISA on serum predicts biliary atresia with 96% positive predictive value and 98% negative predictive value in Wuhan cohort study (in press)
START trial subset analysis for patients <70 days old: placebo arm 57% jaundice clearance, steroid arm 72% clearance—difference not statistically significant due to insufficient numbers (type 2 error)
King's College initial low-dose steroid trial (2mg/kg/day prednisone): no difference in jaundice clearance between groups, but statistically significant lower bilirubin at one month in steroid group
King's College high-dose steroid protocol (5mg/kg/day): 67% jaundice clearance vs 52% in controls, difference reached statistical significance with larger numbers
Tokyo protocol: start prednisone 4mg/kg/day after CRP normalizes post-op, taper over 15 days (4→3→2→1→0.5mg/kg, 3 days each), return to higher dose if stool becomes acholic
King's College current protocol: 5mg/kg/day prednisone starting day 4 post-op when oral feeds begin
Cincinnati molecular profiling identified two biliary atresia phenotypes: inflammatory (potentially steroid-responsive) and fibrotic (less likely to respond)
START trial design was adequately powered as designed; randomized phase had N=70 per arm, larger than Davenport's randomized phase (N=18 steroid, N=19 placebo)
START trial documented that serious adverse events occurred earlier in steroid group compared to placebo during the period of steroid administration, but no difference in total SAE frequency
GI bleeding is a potential steroid complication; King's College always gives ranitidine with steroids, which may not have been standard in START trial
START trial started steroids on post-op day 1, whereas Tokyo and King's protocols wait 4-7 days until CRP normalizes—this timing difference may affect safety profile
Early post-operative cholangitis is a bad prognostic sign for long-term native liver survival
King's College antibiotic protocol: IV tazocin-gentamicin for 5 days, then oral cephalosporin for 1 month
Cincinnati antibiotic protocol: cephalosporin until taking PO, then Bactrim or amoxicillin for minimum 3 months post-op
Aggressive nutrition management is key: maintain adequate weight gain with concentrated formula or NG supplementation as needed, plus fat-soluble vitamin replacement
For patients presenting at 100+ days with cirrhosis on ultrasound (heterogeneous surface, ascites), consider primary transplant rather than Kasai; primary transplant rate in England/Wales is approximately 5%
King's College data from 2001: 100+ day Kasai patients achieved approximately 40% five-year native liver survival
Tokyo approach to late presenters: decision based on timing of acholic stool onset, not chronological age—if acholic from birth, consider primary transplant; if yellow stool for first month of life, proceed to Kasai even at 100 days
For cystic biliary atresia with visible intrahepatic ducts on cholangiogram, prognosis is better even in late presenters
Cincinnati approach: clinical assessment with consideration of biopsy fibrosis; will explore surgically unless significant cirrhosis on pre-op biopsy; some 120-day patients have done well while some 60-day patients had advanced disease
King's College data with high-dose steroids: 100% of patients who underwent Kasai before 30 days of age cleared their jaundice
Tokyo data shows age at Kasai is not significantly related to outcome; duration of acholic stool (disease duration) is the most important prognostic factor
Cholangitis management: early recognition critical, families instructed to seek immediate medical attention for fever, not wait at home
Cincinnati cholangitis protocol: IV antibiotics, consider short course of steroids if no response after several days
King's College does not use steroids for cholangitis treatment and does not perform early revision Kasai
Tokyo cholangitis protocol: intensive IV antibiotics until CRP and white count normalize, then steroids; revision Kasai not performed due to poor outcomes
Cincinnati performs revision Kasai only in highly selected patients: those who previously cleared jaundice and normalized bilirubin, then became acholic after cholangitis; 25-patient series showed native liver salvage possible, some patients 5-10 years out with native liver
For patients who never clear jaundice post-Kasai: focus on aggressive nutrition and proceed to liver transplant evaluation; nutritional support is key driver in determining post-transplant complications
Transplant referral timing: if bilirubin has not dropped below 2 at 3 months post-Kasai, refer to transplant center for evaluation even if not immediately listing
CMV IgM-positive biliary atresia represents approximately 10% of cases in Western Europe and North America; prevalence higher in China and Japan
Cincinnati mouse model identified peptide sequence common to rotavirus, CMV, and EBV that may govern host immune response in biliary atresia
King's College retrospective data (2010-11): CMV IgM-positive patients were older at Kasai, had different histology, and cleared jaundice in less than 10% of cases without antiviral treatment
King's College prospective antiviral treatment (valganciclovir): 7 of 9 (approximately 78%) CMV-positive patients cleared jaundice, dramatic improvement from historical <10%
CMV PCR levels in biliary atresia show inverse relationship with age and AST levels; highest viral loads in younger babies, suggesting body clears virus over time
Increasing proportion of biliary atresia patients require transplant despite modest bilirubin elevation due to progressive fibrosis and portal hypertension complications
King's College colchicine trial (1970s-80s) for liver fibrosis showed no difference in long-term fibrosis levels or clinical outcomes between treatment and placebo groups
Cincinnati has unpublished animal model data showing targeted anti-fibrotic therapy produces remarkable decrease in collagen deposition and hepatic fibrosis
START trial (US multicenter, double-blind, placebo-controlled): no significant difference in jaundice clearance at 6 months between steroid and placebo groups in all comers (58% vs 48%, p>0.05)
Davenport UK trial showed improvement in jaundice clearance and decrease in bilirubin and liver enzymes with high-dose steroids versus no steroid
START trial antibiotic protocol: treatment dose for 2 weeks, then prophylaxis for 6 months