Parents often worry the health and well-being of their childrenEspecially new and first time parents. And while there are many health problems that are seen in the early years, biliary atresia — a blockage of the bile ducts (the small 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 Srikanth KP, MD and DM, Consultant – Pediatric Gastroenterology and Hepatology, Manipal Hospital Old Airport Road, Whitefield and Varthur Road, Bengaluru, if not treated appropriately, cirrhosis can also occur in infants. “So far no clear cause of the disease has been identified. While some claim that it is a sequel to the viral infection, some 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 up to 2-3 months of age. The major symptoms that children may experience are pale/light colored/clay stools, Jaundiceand dark yellow urine (staining of clothes and diapers) in early infancy.
Elaborating further, the doctor said: “The yellowing of the eyes is called jaundice, which can also be seen in liver diseases. When red blood cells (RBCs) age, the hemoglobin in the RBCs is broken down in an accelerated step and converted into bilirubin which gives the eyes its yellow color. The initial molecule is called the ‘unconjugated fraction’ and is ‘conjugated’ in the liver to make it water-soluble, so that it can be more easily excreted in the stool via bile.
He noted that when RBCs are excessively broken down and only “the unconjugated fraction increases”, then color of stool and urine remain unchanged. In the case of liver disease (any), however, the conjugated fraction becomes elevated in the blood and it diffuses into the urine, making it yellow.
“In some situations, bilirubin does not enter the intestine, causing the stool to turn yellow. In biliary atresia, the stool turns yellow and the urine becomes dark yellow (due to the conjugated fraction). A combination of blood test (liver function test), ultrasound scan of liver, HIDA scan and sometimes liver biopsy (small piece of liver examined under microscope) can help us diagnose this condition,” he said.
Can this disease be prevented?
The expert said that there is no clear strategy to prevent this condition, as no one knows why it develops. “We can diagnose this early and prevent liver damage. Once confirmed, the first treatment option is to bypass the damaged bile ducts using an intestinal channel (Butcher portoenterostomy). The sooner this is done (preferably before 60 days, or at least up to 90 days), the better the outcome.”
Dr. Shrikant further said that despite this bypass, the success rate is estimated at 50-60 percent. “But, if diagnosis is delayed by more than 90 days (or 120 days of life in some cases) or liver damage has already been established, the only treatment option is liver transplantation (LT). In fact, It is the most common indication for liver transplantation in children (responsible for 50 percent of all cases of LT).
The doctor said that children who have a liver transplant live, grow and grow like normal adults.
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