Summary about Disease
Biliary atresia is a rare condition in infants in which the bile ducts outside and inside the liver are blocked or absent. Bile, which helps digest fats, cannot flow out of the liver, leading to liver damage and cirrhosis. Without treatment, it is fatal.
Symptoms
Jaundice (yellowing of the skin and eyes) typically appearing in the first few weeks of life.
Dark urine
Pale, clay-colored stools
Enlarged liver and spleen
Poor weight gain
Irritability
Causes
The exact cause of biliary atresia is unknown. It is believed to be multifactorial, potentially involving:
Viral infections
Immune system problems
Genetic mutations
Problems during liver and bile duct development in pregnancy
Medicine Used
There's no medicine to cure biliary atresia.
Ursodeoxycholic acid (UDCA) may be used to improve bile flow and liver function after surgery
Antibiotics are used to treat any infections
Vitamins and supplements like fat-soluble vitamins (A, D, E, and K) and medium-chain triglycerides (MCT oil) are given to address malabsorption.
Is Communicable
No, biliary atresia is not communicable. It is not contagious and cannot be spread from person to person.
Precautions
There are no specific precautions to prevent biliary atresia as the exact cause is unknown.
How long does an outbreak last?
Biliary atresia is not an outbreak-related disease. It is a congenital condition, meaning it is present at birth or develops shortly thereafter.
How is it diagnosed?
Diagnosis typically involves:
Physical examination: Assessing for jaundice, enlarged liver, and other signs.
Blood tests: Liver function tests to check bilirubin levels and other indicators of liver health.
Ultrasound: Imaging of the liver and bile ducts to look for abnormalities.
Liver biopsy: Removing a small sample of liver tissue for examination under a microscope.
Hepatobiliary scan (HIDA scan): A nuclear medicine scan to assess bile flow.
Intraoperative cholangiogram: An X-ray of the bile ducts performed during surgery.
Timeline of Symptoms
First few weeks of life: Jaundice typically appears.
Within 2-8 weeks: Dark urine and pale stools become noticeable.
Within 2-3 months: Liver enlargement, poor weight gain, and irritability may develop.
Without treatment: Liver damage progresses to cirrhosis within the first year.
Important Considerations
Early diagnosis and treatment (Kasai procedure) are crucial to improve the infant's prognosis.
Liver transplantation may be necessary if the Kasai procedure is not successful or if liver damage progresses.
Long-term monitoring and management are essential for children with biliary atresia, even after successful treatment.
Nutritional support is vital to address malabsorption issues.