https://www.ncbi.nlm.nih.gov/pubmed/29570556

J Pediatr Gastroenterol Nutr. 2018 Mar 22. doi: 10.1097/MPG.0000000000001950. [Epub ahead of print]

Province-Wide Biliary Atresia Home Screening Program in British Columbia: Evaluation of First Two Years. Woolfson JP, Schreiber RA, Butler AE, MacFarlane J, Kaczorowski J, Masucci L, Bryan S, Collet JP.

Abstract

BACKGROUND:
Biliary atresia (BA), a rare newborn liver disease, is the leading cause of liver-related death in children. Early disease recognition and timely surgical Kasai hepatoportoenterostomy (KP) offers long-term survival without liver transplant. Universal BA screening in Taiwan using infant stool color cards (ISCCs) has proven effectiveness. We report our experience with ISCC BA screening in a province-wide program in British Columbia (BC).

OBJECTIVES:
To assess program performance and cost from launch April 1, 2014-March 31, 2016.

METHODS:
ISCCs distributed to families upon maternity ward discharge. Parents were instructed to monitor their infant's stool color for one month and contacted the screening center with concerns. The number of live births, ISCC distribution, BA cases, and costs were recorded. Cases with Program screen success had both acholic stool recognition (ISCC screen success) and timely referral for BA.

RESULTS:
All 126 maternity units received ISCCs. Of 87,583 live births there were 6 BA cases. Of the five cases with ISCC Screen Success three had Program Screen Success. The median KP age in the program screen success and failure groups was 49 (42-52) and 116 (49-184) days respectively. Program sensitivity was 50%, specificity 99%, PPV 4% and NPV 99%. A random sample of 1054 charts at BC Children's Hospital found an ISCC distribution rate of 94%. After a phase-in period, the annual program cost was $30,033.82, and the ISCC cost per birth was $0.68.

CONCLUSIONS:
The screening program has high specificity and distribution with low cost. Successful program case identification had earlier age at KP. Program modifications aim to improve sensitivity. Longer-term studies will determine program impact on health outcomes.