A good read: KR Mysore, BL Shneider, S Harpavat. JPGN 2019; 69: 396-403.
This review dissects the evidence that biliary atresia (BA) most often begins in utero.
- Infants with BA have elevated conjugated/direct bilirbuin at birth.
- Infants with BA have biliary abnormalities on fetal ultrasound (eg. gallbladder abnormalities, biliary cysts).
- Infants with BA have abnormal gamma-glutamyl transferase levels in their amniotic fluid with low levels noted at gestational age 18-19 weeks. This finding is not specific for BA as other conditions that affect biliary tree (eg. cystic fibrosis, trisomy 21) can have low levels as well.
- BA is more common in premature infants.
- Early recognition is important. In U.S (from 1976-89), transplant-free survival rates were 63% for Kasai when done in 1st 30 days, 44% for 30-60 days, 40% for 60-90 days, and 29% if >90 days.
- A diagnostic approach is given in Figure 2 but is already out of date due to the availability of MMP-7 testing (article received by JPGN in January 2019).
This review also lists numerous current investigative therapies which include probiotic, steroids, desflurane/sevoflurane (anesthetics), pentoifyline, IVIG, vancomycin, meloxicam, GCSF, Bone marrow stem cells, N-acetylcysteine, and obeticholic acid.
My take: This article shows that the clock on liver injury begins in utero in most cases of BA and this will have implications on pathogenesis and management.
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