Biliary Atresia: Where are we now? Advanced Practice Providers Pediatric...
Pathologic jaundice occurs in 1 in 2500 live births
epidemiological2:24 ↗
Biliary atresia is a progressive, obliterative, idiopathic cholangiopathy with two forms: perinatal/postnatal (80-90%) and fetal/embryonic (10-20%)
clinical5:01 ↗
Biliary atresia incidence ranges from 1 in 5000 in Taiwan, 1 in 8-10,000 in Japan, and 1 in 18,000 in the United States and Europe
epidemiological5:42 ↗
More females than males are affected by biliary atresia
epidemiological6:05 ↗
Seasonal clustering of biliary atresia cases suggests viruses or infectious processes may be triggers
clinical6:09 ↗
Biliary atresia is lethal if untreated
clinical6:10 ↗
If conjugated or direct bilirubin is greater than or equal to 2, the child needs closer evaluation
guideline7:59 ↗
Some babies with biliary atresia have normal ultrasounds
clinical8:16 ↗
The gold standard for biliary atresia diagnosis is the intraoperative cholangiogram
guideline8:26 ↗
At Children's Hospital Los Angeles, babies are pre-treated with 5 days of phenobarbital before HIDA scan
clinical9:31 ↗
Timing of diagnosis and Kasai procedure is extremely important, with the sweet spot being 6 to 8 weeks of age
clinical15:14 ↗
A French study showed 23% of Kasai patients had their native liver 20 years post-Kasai
epidemiological15:50 ↗
Factors affecting survival with native liver include age at Kasai, type of biliary atresia, liver histology (bridging fibrosis), and center caseload
clinical16:24 ↗
In England, consolidating Kasai procedures to 3 centers increased success rates
epidemiological16:48 ↗
At Children's Hospital Los Angeles, 6 to 12 biliary atresia cases are seen per year, considered a high rate
epidemiological17:20 ↗
If cirrhosis is seen on liver biopsy or frozen section during intraoperative cholangiogram, the procedure is stopped and the patient is listed for transplant
clinical19:57 ↗
Kasai procedures in older babies (3.5-4 months) can bridge patients to transplant at one year of age, though with complications including ascending cholangitis and frequent hospitalizations
clinical20:20 ↗
Bigger babies at time of transplant have easier transplant procedures
opinion21:24 ↗
Children's Hospital Los Angeles has a very large, possibly the largest, living donor liver transplant center in the country, offering an alternative to cadaveric transplant
clinical22:27 ↗
Most centers put babies on antibiotic prophylaxis postoperatively until they are eating
clinical23:23 ↗
Post-operative steroids for biliary atresia are unproven
clinical23:48 ↗
At Children's Hospital Los Angeles, peripheral IV lines are used postoperatively rather than Broviacs, and babies are not sent home on IV antibiotics
clinical24:06 ↗
Babies are switched from IV antibiotics to Bactrim once taking orals; babies under 6 weeks receive amoxicillin until 6 weeks of age, then switch to Bactrim at 4-5 mg/kg/day
clinical25:08 ↗
Babies are placed on ursodeoxycholic acid twice daily post-Kasai
clinical25:35 ↗
Biliary atresia babies are often deficient in fat-soluble vitamins D, E, and K, which are supplemented using aqueous solutions (Aquadek or ADeck) for better absorption
clinical25:41 ↗
Probiotics, herbal remedies, and omega-3 supplements have not been proven beneficial for biliary atresia
clinical26:29 ↗
The BARC (Biliary Atresia Research Consortium) protocol enrolled 140 babies in a randomized double-blind placebo-controlled study with steroids
clinical27:01 ↗
When a post-Kasai baby has unexplained fever, ascending cholangitis is suspected
clinical28:15 ↗
Multiple episodes of ascending cholangitis cause further liver damage and may lead to earlier need for transplant
clinical29:36 ↗
Biliary atresia is the most common indication for pediatric liver transplant
epidemiological31:09 ↗