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Technical report examines strategies to identify biliary atresia :

November 30, 2015

A new AAP technical report describes potential screening strategies for early identification of biliary atresia, the most common cause of pediatric end-stage liver disease and the leading indicator for pediatric liver transplantation.

The technical report Newborn Screening for Biliary Atresia is available at http://pediatrics.aappublications.org/cgi/doi/10.1542/peds.2015-3570 and is published in the December issue of Pediatrics.

Infants with biliary atresia develop jaundice and pale, acholic stools within the first few weeks after birth. Early diagnosis and early performance of the Kasai hepatic portoenterostomy with successful surgical drainage of bile are associated with better outcomes for infants with biliary atresia.

However, because noncholestatic jaundice is extremely common in early infancy, it is difficult to identify the rare infant with cholestasis who has biliary atresia. The need for timely diagnosis of this disease warrants investigation into the feasibility, effectiveness and costs of screening for biliary atresia to improve outcomes, according to the report.

Newborn screening for biliary atresia is assessed using a number of different methods, including serologic testing, ultrasonography and biopsy. The report discusses two screening tests: serum conjugated or direct bilirubin concentrations and stool color cards. Both tests have exhibited reasonable clinical validity, and neither poses significant risk to the infant undergoing screening. In addition, published analyses indicate that these screening methods are potentially life-saving and cost-effective.

The reportrecommends that educational programs for pediatricians about the importance of early identification of biliary atresia may help improve outcomes for infants with this rare but highly morbid disease. Offering stool color cards to parents to use as a reference for comparison also could aid in early detection.

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