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Health Care Provider FAQ

This page provides answers to frequently asked questions about biliary atresia screening for health care providers. Click here to download a PDF copy of these frequently asked questions.

What is biliary atresia?

Biliary atresia is a uniquely pediatric liver disease causing a progressive obliteration of the bile ducts, restricting bile flow from the liver to the intestine (cholestasis). It is the most common cause of liver transplant and liver related death in children.

Screening can help identify babies with cholestasis earlier and potentially improve the chance of native liver survival. Babies with early-stage biliary atresia appear healthy and we know that in Ontario most babies with biliary atresia are currently not being diagnosed early enough to give them the best possible outcomes from their treatment.

Families compare their baby’s stool to the pictures on the Infant Stool Colour Card (ISCC) during diaper changes. If the closest match is 1,2,3,4,5 or 6, they are to contact Newborn Screening Ontario using one of the several methods listed on the card so they can be guided through the next steps in the screening process.

  • A team member from Newborn Screening Ontario (NSO) will contact the caregiver within 1 business day to conduct a telephone assessment of the baby which will include questions about the baby’s stool and other questions about the baby’s health.
    • The team member may request that a photo of the stool be submitted as well. 
  • If the team member concludes that the stool is abnormally pale, they will facilitate an urgent remote consultation with a gastroenterologist at one of five pediatric regional treatment centres in Ontario.
  • The GI specialist will then arrange for a total and direct/conjugated bilirubin to be done, will interpret the result, and will arrange next steps with the family.
  • NSO will send a notification to the baby’s primary care provider about the telephone assessment.

The pediatric gastroenterologist who receives the referral from NSO will be ordering the outpatient bloodwork and interpreting the results. We have had many families call us who do not have a primary family doctor.

  • Babies with biliary atresia can appear healthy at first. Initially, they have prolonged jaundice lasting longer than 2 weeks of age and develop acholic (pale) stools, hepatomegaly, and dark urine.
  • Babies with late-stage biliary atresia can go on to develop ascites, splenomegaly, and GI bleeding resulting from portal hypertension, along with failure to thrive, irreversible liver cirrhosis, and eventual death if surgery or liver transplant are not performed.

Bile is a dark green to yellowish pigmented digestive enzyme that is made in the liver and flows into the intestine. It makes stool darker. In biliary atresia, the bile cannot get into the intestine resulting in stool that appears lighter in colour.

Stool screening with the infant stool colour card is not a diagnostic test. It tells us which babies have pale stools, possibly indicating cholestasis. There are several reasons why stool may be pale in colour and several causes of cholestasis, aside from biliary atresia. To make a diagnosis, other diagnostics such as blood tests and imaging of the liver are needed.

There are some syndromes and congenital heart defects that are associated with biliary atresia. In some countries, there appears to be an increased incidence of biliary atresia associated with cytomegalovirus.

Families are advised to screen the stool using the ISCC for one month during diaper changes. Families of babies who are born before 37 weeks are advised to screen for one month past the actual due date.

The treatment for biliary atresia is a surgical procedure called the Kasai portoenterostomy (KPE), where the flow of bile is re-established from the liver to the intestine. Despite receiving a KPE, some babies will still develop liver failure and will need to proceed to liver transplant. The earlier that this procedure can happen, the better the chance of native liver survival. Unless bile flow is established by surgical intervention through a KPE, children with biliary atresia rarely survive beyond 2 years of age without liver transplantation and mortality is inevitable in cases where no surgical repair or liver transplantation is done.

You can ensure that families have an ISCC to screen at home. You can also offer education on BA screening as well as emphasize the importance of screening.

If you are an existing NSO submitter, you can order infant stool colour cards from VWR International along with dried blood spot collection cards.

If you are not an existing NSO submitter, please contact us by email at

No, please do not download the ISCC for clinical use. The original card provided by Newborn Screening Ontario (NSO) must be used as the printing and card stock is controlled to ensure colour accuracy of the specific pale stool colours, which have been validated in previous studies and screening programs. These original cards are provided at no cost to your organization. You may download translated copies of the cards from our main website to use alongside the original card.

Yes, this should be included in prenatal teaching. The more awareness families have about watching for pale stool and screening for biliary atresia using the Infant Stool Colour Card, the more likely they are to screen their infant and identify affected babies early.

Please arrange for a total and direct/conjugated bilirubin level to be done. If there is no pale stool associated with the clinical jaundice, you do not need to call NSO.

Contact Us

Children’s Hospital of Eastern Ontario
415 Smyth Road
Ottawa, Ontario K1H 8M8

Toll-Free: 1-877-627-8330
Local: (613) 738-3222
Fax: (613) 738-0853

Contact NSO

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