# Biliary Atresia - Clinical Practice Updates — GCMD Library

<p>This clip from the 2020 Pediatric Surgery Update Course features Alexander Bondoc, MD, presenting multiple cases and research conclusions from Atsuyuki Yamataka, MD; Greg Tiao, MD; Jorge Bezerra, MD; and Mark Davenport, MD, to our selected panel for review and debate. Using a recently developed Biliary Atresia Diagnostic Algorithm, we ask the panel, "What would you do?"<br />
<br />
<u>Highlighted Topics Include: </u><br />
- Using Serum Matrix Metalloproteinase-7 levels (MMP-7) to predict Biliary Atresia<br />
- Is Percutaneous Liver Biopsy Obsolete?<br />
- Diagnostic Laparoscopy + Cholangiogram benefits<br />
- Cystic Biliary Atresia<br />
- Open vs Laparoscopic Portoenterostomy<br />
- Indocyanine Green as an Adjunct<br />
- Kasai revision<br />
- Adjuvant Medical Options<br />
- Porto-Dudenostomy vs Roux<br />
- Treating CMV<br />
- Post-Op Steroids</p>


Type: video · posted 2020-09-14
Canonical: https://library.globalcastmd.com/watch/biliary-atresia-clinical-practice-updates-3000

## Chapters
- [0:00](https://library.globalcastmd.com/watch/biliary-atresia-clinical-practice-updates-3000?t=0) Case Presentation and Differential Diagnosis
- [4:44](https://library.globalcastmd.com/watch/biliary-atresia-clinical-practice-updates-3000?t=284) Diagnostic Algorithm and Laboratory Workup
- [11:41](https://library.globalcastmd.com/watch/biliary-atresia-clinical-practice-updates-3000?t=701) Imaging and Laparoscopic Diagnosis Debate
- [20:28](https://library.globalcastmd.com/watch/biliary-atresia-clinical-practice-updates-3000?t=1228) Liver Biopsy Controversy and Centralization
- [24:33](https://library.globalcastmd.com/watch/biliary-atresia-clinical-practice-updates-3000?t=1473) Cystic Biliary Atresia Variant

## Statements
- "In the US and Europe, alpha-1 antitrypsin deficiency is the most common cause of neonatal cholestasis in the differential diagnosis" — Jorge Bezerra (epidemiological) [3:22](https://library.globalcastmd.com/watch/biliary-atresia-clinical-practice-updates-3000?t=202)
- "By two weeks of age, if the baby still has jaundice, the pediatrician needs to fractionate the bilirubin; if the direct or conjugated fraction is elevated, think about pathologic jaundice" — Jorge Bezerra (guideline) [4:16](https://library.globalcastmd.com/watch/biliary-atresia-clinical-practice-updates-3000?t=256)
- "The Taiwanese published in the Journal of Pediatrics in 2006 that they send all their kids home with stool color cards" — Alex Bondoc (clinical) [6:13](https://library.globalcastmd.com/watch/biliary-atresia-clinical-practice-updates-3000?t=373)
- "MMP7 was identified as a serum marker of epithelial injury in biliary atresia through large-scale proteomic analysis of 30 children with biliary atresia in 2017" — Alex Bondoc (clinical) [9:59](https://library.globalcastmd.com/watch/biliary-atresia-clinical-practice-updates-3000?t=599)
- "MMP7 using a cutoff value of 52-53 nanograms per ml demonstrated a sensitivity of diagnosing biliary atresia of about 98% with a specificity of about 95% in 135 consecutive infants in China" — Alex Bondoc (clinical) [10:40](https://library.globalcastmd.com/watch/biliary-atresia-clinical-practice-updates-3000?t=640)
- "The MMP7 assay is run every other day and is a six-hour assay" — Jorge Bezerra (clinical) [12:51](https://library.globalcastmd.com/watch/biliary-atresia-clinical-practice-updates-3000?t=771)
- "MMP7 has the potential to eliminate a lot of other testing and eliminate things like a liver biopsy if confidence grows in its impact" — Alex Bondoc (opinion) [13:10](https://library.globalcastmd.com/watch/biliary-atresia-clinical-practice-updates-3000?t=790)
- "Cutoff values for MMP7 changed depending on reports from Asia, with much lower cutoff levels reported from Taiwan and China" — Jorge Bezerra (clinical) [12:20](https://library.globalcastmd.com/watch/biliary-atresia-clinical-practice-updates-3000?t=740)
- "A combination of blood tests and laparoscopy can diagnose biliary atresia promptly and accurately" — Alex Bondoc (clinical) [14:44](https://library.globalcastmd.com/watch/biliary-atresia-clinical-practice-updates-3000?t=884)
- "Nearly all patients with biliary atresia have a hard liver, whereas hepatitis and Alagille syndrome patients have livers that are not as hard" — Yamataka (clinical) [17:57](https://library.globalcastmd.com/watch/biliary-atresia-clinical-practice-updates-3000?t=1077)
- "Bile duct proliferation and portal edema may not appear on very early liver biopsies and may delay diagnosis by up to 30 days" — Alex Bondoc (clinical) [17:09](https://library.globalcastmd.com/watch/biliary-atresia-clinical-practice-updates-3000?t=1029)
- "In Yamataka's series, 16 cases were not typical biliary atresia on laparoscopy, and all 16 were confirmed not to be biliary atresia, including 8 cases of Alagille syndrome" — Yamataka (clinical) [19:13](https://library.globalcastmd.com/watch/biliary-atresia-clinical-practice-updates-3000?t=1153)
- "If you do a Kasai operation on Alagille patients, you just shorten that patient's natural liver life and convert those patients to needing transplant at a much earlier age" (clinical) [18:24](https://library.globalcastmd.com/watch/biliary-atresia-clinical-practice-updates-3000?t=1104)
- "Infants coming to surgery for other matters who later turned out to have biliary atresia in the first week of life all had normal livers, so you can't diagnose early biliary atresia on liver biopsy" — Mark Davenport (clinical) [22:18](https://library.globalcastmd.com/watch/biliary-atresia-clinical-practice-updates-3000?t=1338)
- "Liver histology looking at inflammation and fibrosis has not shown great discrimination for prognosis in babies coming to surgery before 100 days" — Mark Davenport (clinical) [23:08](https://library.globalcastmd.com/watch/biliary-atresia-clinical-practice-updates-3000?t=1388)
- "Liver biopsy has additional value to quantify inflammation and use that information to personalize treatment" — Jorge Bezerra (opinion) [24:04](https://library.globalcastmd.com/watch/biliary-atresia-clinical-practice-updates-3000?t=1444)
- "The key differential for a prenatally detected cyst is between cystic biliary atresia and a choledochal cyst" — Mark Davenport (clinical) [24:57](https://library.globalcastmd.com/watch/biliary-atresia-clinical-practice-updates-3000?t=1497)
- "If the baby is not jaundiced and not raising conjugated bilirubin, then it's not cystic biliary atresia and you can wait to do the laparotomy" — Mark Davenport (clinical) [25:51](https://library.globalcastmd.com/watch/biliary-atresia-clinical-practice-updates-3000?t=1551)
- "Cystic biliary atresia patients get to surgery earlier, potentially because of prenatal diagnosis and heightened level of suspicion" — Alex Bondoc (clinical) [26:20](https://library.globalcastmd.com/watch/biliary-atresia-clinical-practice-updates-3000?t=1580)

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Not medical advice · citation policy: https://library.globalcastmd.com/ai
