What is the most common reason for liver transplant in children? The answer is biliary atresia, according to data from the United Network for Organ Sharing.
Biliary atresia accounts for 60% of liver transplants in infants younger than 1 year and 30% of all liver transplants in pediatric patients.
Primary care providers (PCPs) play an essential role in improving outcomes in this devastating disease by identifying infants before 1 month of age and referring them for treatment with Kasai portoenterostomy. Earlier surgery is the only proven way to delay or prevent the need for liver transplant.
The new AAP clinical report Identifying Infants with Biliary Atresia by 2-4 Weeks of Life: Guidance for the Primary Care Provider outlines steps to quickly identify infants who have biliary atresia.
The clinical report, from the Section on Gastroenterology, Hepatology and Nutrition, the Committee on Fetus and Newborn, and the Section on Surgery, is available at https://doi.org/10.1542/peds.2024-070077 and will be published in the March issue of Pediatrics.
Why is biliary atresia so difficult to identify?
Infants with biliary atresia often go unnoticed initially because they usually appear healthy. The first sign is jaundice, which is visually indistinguishable from the physiological jaundice seen in healthy newborns.
As a result, infants with biliary atresia typically are referred for surgery after the 2 month well-child visit. This delay is problematic because infants treated with Kasai portoenterostomy before 30-45 days of life have the best outcomes. During the operation, the obstructed bile ducts are removed, and the liver is connected to the intestines in an attempt to stop bile from backing up into and damaging the liver.
PCPs can use a common blood test — direct or conjugated bilirubin level — to identify infants who may have biliary atresia.
Identifying infants at the 2-4 week well-child visit
Every child should see the PCP in the first 2-4 weeks of life, according to the Bright Futures Periodicity Schedule. This is the ideal period for the PCP to ask three questions.
- Is the infant jaundiced? Sometimes jaundice is easier to notice by looking in the eyes instead of at the skin. If an infant has jaundice beyond 2 weeks of life, there may be a liver problem.
- Does the infant have pale stools? If so, this is a sign that bile is trapped in the liver and not reaching the intestine.
- Did the infant have a direct or conjugated bilirubin that was high while in the newborn nursery? To answer this question, records from the nursery may need to be reviewed. A high initial level could be a sign of liver disease.
If the answer to any of the questions is yes, PCPs can check a direct or conjugated bilirubin level. If the level is 1 milligram/deciliter (mg/dL) or higher, a liver disease such as biliary atresia may be present. PCPs then can contact a pediatric specialist to discuss next steps and plan for possible urgent referral.
Key actions for pediatricians
- At the 2-4 week well-child visit, ask three questions: Is jaundice present? Are the stools pale? Was an initial direct or conjugated bilirubin level high?
- If the answer to any of these questions is yes, consider checking a direct or conjugated bilirubin level.
- If a direct or conjugated bilirubin level is 1 mg/dL or higher at 2-4 weeks of life, a liver disease such as biliary atresia may be present. PCPs can contact pediatric gastroenterology to discuss next steps.
Dr. Harpavat is a lead author of the clinical report and chair of the AAP Section on Gastroenterology, Hepatology and Nutrition.
