Biliary Atresia - Clinical Practice Updates

In the US and Europe, alpha-1 antitrypsin deficiency is the most common cause of neonatal cholestasis in the differential diagnosis

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

The Taiwanese published in the Journal of Pediatrics in 2006 that they send all their kids home with stool color cards

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

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

Cutoff values for MMP7 changed depending on reports from Asia, with much lower cutoff levels reported from Taiwan and China

The MMP7 assay is run every other day and is a six-hour assay

MMP7 has the potential to eliminate a lot of other testing and eliminate things like a liver biopsy if confidence grows in its impact

A combination of blood tests and laparoscopy can diagnose biliary atresia promptly and accurately

Bile duct proliferation and portal edema may not appear on very early liver biopsies and may delay diagnosis by up to 30 days

Nearly all patients with biliary atresia have a hard liver, whereas hepatitis and Alagille syndrome patients have livers that are not as hard

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

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

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

Liver histology looking at inflammation and fibrosis has not shown great discrimination for prognosis in babies coming to surgery before 100 days

Liver biopsy has additional value to quantify inflammation and use that information to personalize treatment

The key differential for a prenatally detected cyst is between cystic biliary atresia and a choledochal cyst

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

Cystic biliary atresia patients get to surgery earlier, potentially because of prenatal diagnosis and heightened level of suspicion