In United States and Europe by two weeks of age, if the baby still has jaundice, the pediatrician or whoever's seeing the patient needs to fractionate the Billy Rubin. If the direct or conjugated fraction is up, think about the pathologic jaundice.
If they say the baby has normal stool color, we want to see it because there have been many cases in which the parents thought it was okay, but to us it was a colic stool.
epidemiologicalIn the US and Europe, alpha-1 antitrypsin deficiency is the most common cause of neonatal cholestasis in the differential diagnosis↗
▶Ep 8 · 4:16
guidelineBy 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↗
▶Ep 8 · 4:16
quoteIn United States and Europe by two weeks of age, if the baby still has jaundice, the pediatrician or whoever's seeing the patient needs to fractionate the Billy Rubin. If the direct or conjugated fraction is up, think about the pathologic jaundice.↗
▶Ep 8 · 12:20
quoteIt is very uncommon to have a baby with biliary atresia and a low MMP7.↗
▶Ep 8 · 12:20
clinicalCutoff values for MMP7 changed depending on reports from Asia, with much lower cutoff levels reported from Taiwan and China↗
▶Ep 8 · 12:51
clinicalThe MMP7 assay is run every other day and is a six-hour assay↗
▶Ep 8 · 24:04
opinionLiver biopsy has additional value to quantify inflammation and use that information to personalize treatment↗
Biliary Atresia Part II
▶Ep 14 · 1:50
guidelineAmerican 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↗
▶Ep 14 · 1:50
guidelineAmerican 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↗
▶Ep 14 · 2:31
clinicalCincinnati 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↗
▶Ep 14 · 2:31
clinicalCincinnati 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↗
▶Ep 14 · 3:30
clinicalParents reporting normal stool color must show the stool for verification, as many parents misidentify acholic stools as normal↗
▶Ep 14 · 3:30
quoteIf they say the baby has normal stool color, we want to see it because there have been many cases in which the parents thought it was okay, but to us it was a colic stool.↗
▶Ep 14 · 3:30
quoteIf they say the baby has normal stool color, we want to see it because there have been many cases in which the parents thought it was okay, but to us it was a colic stool.↗
▶Ep 14 · 3:30
clinicalParents reporting normal stool color must show the stool for verification, as many parents misidentify acholic stools as normal↗
▶Ep 14 · 4:03
epidemiologicalMMP7 (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)↗
▶Ep 14 · 4:03
quoteMMP7 alone, at a specific curve level, can predict biliary atresia with a positive predictive value of about 96% and negative predictive value of about 98%.↗
▶Ep 14 · 4:03
epidemiologicalMMP7 (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)↗
▶Ep 14 · 4:03
quoteMMP7 alone, at a specific curve level, can predict biliary atresia with a positive predictive value of about 96% and negative predictive value of about 98%.↗
▶Ep 14 · 5:48
epidemiologicalSTART 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)↗
▶Ep 14 · 5:48
epidemiologicalDavenport UK trial showed improvement in jaundice clearance and decrease in bilirubin and liver enzymes with high-dose steroids versus no steroid↗
▶Ep 14 · 14:32
epidemiologicalSTART 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)↗
▶Ep 14 · 14:32
epidemiologicalSTART 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)↗
▶Ep 14 · 16:30
epidemiologicalSTART 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↗
▶Ep 14 · 16:30
epidemiologicalSTART 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↗
▶Ep 14 · 20:12
clinicalAggressive nutrition management is key: maintain adequate weight gain with concentrated formula or NG supplementation as needed, plus fat-soluble vitamin replacement↗
▶Ep 14 · 20:12
clinicalAggressive nutrition management is key: maintain adequate weight gain with concentrated formula or NG supplementation as needed, plus fat-soluble vitamin replacement↗
▶Ep 14 · 30:20
clinicalFor 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↗
▶Ep 14 · 30:20
clinicalFor 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↗
▶Ep 14 · 40:38
clinicalCincinnati has unpublished animal model data showing targeted anti-fibrotic therapy produces remarkable decrease in collagen deposition and hepatic fibrosis↗
▶Ep 14 · 40:38
clinicalCincinnati has unpublished animal model data showing targeted anti-fibrotic therapy produces remarkable decrease in collagen deposition and hepatic fibrosis↗
Summaries Jorge gave as host
· 2 summaries
Recaps of other experts' statements, not Jorge's own clinical position.
Summaries Jorge gave as host · Biliary Atresia2 summaries
host summaryJorge Bezerra summarizing the discussion: 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)↗
▶Ep 14 · 5:48
host summaryJorge Bezerra summarizes what Dr. Mark Davenport said: Davenport UK trial showed improvement in jaundice clearance and decrease in bilirubin and liver enzymes with high-dose steroids versus no steroid↗
