What is Biliary Atresia (BA)?
Biliary atresia is a rare pediatric liver disease with symptoms typically developing about two to eight weeks after
birth and no approved pharmacological therapies. Damaged or absent bile ducts outside the liver result in bile and bile
acids being trapped inside the liver, quickly resulting in cirrhosis, and even liver failure.
There are currently no approved pharmacological treatments for biliary atresia, which impacts an estimated 40,000 to
50,000 people worldwide.
What Happens in the Body?
A healthy liver produces bile, a liquid that helps with digesting fats and absorbing vitamins. Bile is transported
through the bile ducts.
In people with BA, bile ducts do not develop normally and can become blocked by build-ups of bile. These blockages can
lead to liver damage and scarring, which can be life threatening if not treated, as well as jaundice. Bile contains
“bilirubin,” a yellowish pigment. When bile ducts become blocked, bilirubin builds up in blood, causing the yellowing of
the skin and whites of the eyes.
What are the Symptoms Associated with BA?
BA doesn’t affect everyone the same, but the most common symptoms include:
- Dark urine
- Portal hypertension (increase in blood pressure in the veins that carry blood from the intestine to the liver)
- Yellowing of skin (jaundice)
- Abnormally pale stool
- Swollen stomach
Path to Diagnosis
It is important to diagnose BA early to begin a treatment plan. A pediatrician or general practitioner may recommend
that a child see a liver specialist, called a hepatologist, to diagnose and manage BA. Diagnosing BA may include:
- Blood tests
- Abdominal or bile duct X-ray
- Abdominal ultrasound
- Liver biopsy
- Hepatobiliary iminodiacetic acid scan (diagnostic test for the gallbladder and bile ducts)
Treatment Options
If a baby has BA, they will likely require a type of surgery called a Kasai procedure, which involves fixing the bile
ducts to help restore bile flow. There are no medical treatments yet for BA, but researchers are studying
investigational medicines in clinical trials. Approximately 1/3 of pediatric liver transplants are caused by BA, making
it the most common cause.
Resources
For more details on BA, including what causes the disease, the common signs and symptoms and how it is diagnosed,
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