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Updated Biliary Atresia Epidemiology

September 6, 2017 7:00 am

A recent retrospective study (PC Hopkins, N Yazigi, CM Nylund. J Pediatr 2017; 187: 253-7) provides an update on the recent incidence of biliary atresia in the US from 1997-2012. This study relied on coding for biliary atresia or Kasai hepatoportoenterostomy to identify cases using HCUP-KID database. This database provides a nationally representative sample of pediatric hospitalizations and captures ~96% of pediatric hospitalizations in the US.

Key findings:

Incidence of biliary atresia (BA) was 4.47 per 100,000 (1 in 22,371 infants)
BA was more common in females (RR 1.43), Asian/Pacific Islanders (RR 1.89), and blacks (RR 1.30)
Median age at the time of the Kasai procedure was 63 days with no improvement over the course of the study period. More than 50% of all children underwent the Kasai procedure after the optimal window of 60 days of life

My take: In my view, at this time, obtaining a blood test for direct bilirubin in the first two weeks of life will need to be adopted broadly if we are going to diagnose biliary atresia at an earlier age.

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