Biliary atresia disease in kids: Signs, symptoms and everything else you need to know

Parents often worry about the health and well-being of their children, especially new and first-time parents. And while there are many health issues that are seen in the formative years, biliary atresia — a blockage of bile ducts (tiny channel that carries secretions from liver cells to the gallbladder and small intestine) — is one of the most common causes of serious liver disease in infants.
According to Dr Srikanth KP, MD & DM, consultant – pediatric gastroenterology and hepatology, Manipal Hospital Old Airport Road, Whitefield and Varthur Road, Bengaluru, if not treated appropriately, infants may even develop cirrhosis. “No clear reason for the disease has been identified so far. While some claim it is a sequel of viral infection, others deny it. We know that biliary atresia is not a genetic disease,” he said.

What are the signs and symptoms?
The doctor said that biliary atresia can start around birth to up to 2-3 months of age. The key symptoms children may experience can be pale/light colour/clay stools, jaundice, and dark yellow urine (staining the clothes and diapers) in early infancy.
Explaining further, the doctor said: “The yellowish colouration of the eyes is called jaundice, which can be seen in liver diseases as well. When the red blood cells (RBC) age, the hemoglobin in RBCs is broken at an accelerated phase and is converted to bilirubin which imparts the yellow colour to the eyes. The initial molecule is called ‘unconjugated fraction’ and this is ‘conjugated’ in the liver to make it water-soluble, so that it can be easily excreted through bile into feces.”

He added that when the RBCs are broken down excessively and only the “unconjugated fraction increases”, the stool and urine colour remain unaltered. In the case of liver disease (any), however, the conjugated fraction is elevated in the blood and it spills over to urine, making it yellowish.
“In some situations, bilirubin does not enter the intestine, which makes the stools pale in colour. In biliary atresia, the stool becomes pale and the urine becomes dark yellow (due to conjugated fraction). A combination of blood tests (liver function tests), ultrasound scan of the liver, HIDA scan, and sometimes liver biopsy (tiny piece of liver examined under a microscope) can help us diagnose this condition,” he said.
Can this disease be prevented?
The expert said there is no clear strategy to prevent this condition, as nobody knows why this develops. “We can diagnose it early and prevent liver damage. Once confirmed, the first treatment option is to pass the damaged bile ducts using an intestinal channel (Kasai Portoenterostomy). The earlier it is done (preferably before 60 days, or at least 90 days), the better the result.”
Dr Srikanth further said that in spite of this pass, the success rate is measured at 50-60 per cent. “But, if the diagnosis is delayed beyond 90 days (or in some cases 120 days of life) or the liver damage is already established, the only treatment option is liver transplantation (LT). In fact, this is the most common indication of liver transplantation in children (accounting for 50 per cent of all cases of LT).”
The doctor said children who receive liver transplantation survive, grow, and thrive as normal adults.
📣 For more lifestyle news, follow us on Instagram | Twitter | Facebook and don’t miss out on the latest updates!