Screening for biliary atresia

Abstract

Early diagnosis followed by proper KP is essential for the improvement of long-term prognosis for patients with BA. It is increasingly accepted that KP at ≤ 30 days of age significantly improves native liver survival rate. Published analyses in English and Japanese indicate that screening by SCC and DB/CB is potentially feasible. Screening with SCC has been implemented in Tochigi Prefecture, Japan, since 1994. The concept of SCC was introduced from Japan to Taiwan and resulted in nationwide screening with SCC for the first time in Taiwan in 2004, followed by Japan in 2012. Home-based screening using SCC is easy and cost-effective; however, it may cause some difficulties for families in case of stools with intermediate colors. Laboratory-based screening using DB/CB may detect the suspected cases earlier, resulting in an increase in the number of patients with BA who undergo KP at ≤ 30 days of age; however, the recall rate is 1% and may be beyond an acceptable range. Further studies are needed to assess the feasibility and cost-effectiveness of both home-based (SCC) and laboratory-based (DB/CB) screening for BA.

Keywords: Bile acid; Biliary atresia; Conjugated bilirubin; Direct bilirubin; Native liver survival; Positive predictive value; Screening; Sensitivity; Specificity; Stool color card.