1. Clinical Course of Untreated BA Phoebe part

2. Clinical Course of Untreated BA Most present within four to six weeks of conjugated jaundice and acholic stools, and, although still a challenging diagnosis to make, therein lies the opportunity of changing the course of this otherwise inexorable disease Uniformly fatal after 2 years Median survival of 8 months

3. Clinical Course of Untreated Biliary Atresia Untreated biliary atresia leads to: – biliary cirrhosis – portal hypertension – esophageal varices – failure to thrive – liver failure – death

4. Clinical Course of Untreated Biliary Atresia Portal Hypertension – As the liver becomes scarred, it presses against the walls of the veins. This constricts the veins and blood cannot pass through them properly. High blood pressure in the portal vein results. Ascites – Another complication of portal hypertension, ascites occur when there is an abnormal build up of fluid in the space between the lining of the abdominal wall and the lining of the organs. Treatment is a low-sodium diet and medication.

5. Varices – A complication of portal hypertension, varices occur when veins encounter increased blood pressure which causes them to weaken and expand. There is no treatment for varices until they bleed. Stools will appear black and vomit will be blood-stained. Immediate medical attention is necessary to determine severity

6. Failure to Thrive – Lack of bile salts in the intestine, needed for fat digestion, result in poor growth and fat-soluble vitamin deficiency in the infant. Vitamins A, D, E and K (water-soluble) can be given orally to aid in fat digestion. Cirrhosis – Occurs when there is severe damage to the liver. When cirrhosis develops, liver transplantation is considered.

7. Clinical Course of Untreated BA Untreated, patients typically die before 2 years of age from hepatic failure