Biliary atresia, or absence of bile ducts, is a condition that results in progressive and serious liver disease that begins with obstruction of the bile ducts before or immediately after birth, and is more common in girls.
Bile ducts are the pathways that carry bile produced in the liver to the intestine. Bile is necessary for digestion and to transport certain waste products from the liver. When the bile ducts do not develop, bile cannot be transported from the liver and liver damage begins. The cause is unknown; despite many studies and theories, no factor causing biliary atresia has been found. There are two main distinct types.
The liver has many vital functions. Bile production and the passage of bile into the intestines are among the most important functions. The inability to produce and excrete bile in the liver impairs the functions of the liver over time.
Diagnosis:
Babies with biliary atresia at birth generally appear healthy and have a normal weight. . Their first stools are colored and normal. Jaundice begins in the first 2-4 weeks after birth, and the color of the stool lightens and turns into a yellow-white paste. The baby develops abdominal swelling and weight gain is insufficient. With blood tests, liver functions, bilirubin levels, protein levels produced by the liver (albumin, total protein and globulin), levels of clotting factors and immune factors produced in the liver are checked. Efforts are made to eliminate these defects. With Ultrasonography, the presence of the gallbladder, the size and condition of the liver are visualized. The most important indicators are the changes in the liver biopsy and the attempts to show whether the bile ducts are present or not. Imaging can be performed with various interventions in the operating room. The bile ducts are imaged by inserting a needle through the skin and injecting radiologically visible dye into the gallbladder. One can try to cheat. The same procedure can also be performed laparoscopically (by entering the abdomen with a light imaging device or by accessing the gallbladder through open surgery. If the bile ducts are not developed, a section of the intestine is brought to the lower part of the liver to ensure the flow of bile from the liver, and bile production and release, if present, is ensured to pass into the intestines. (Kasai surgery). Timing is very important in the success of the surgery. If the diagnosis is made before 60 days and the operation is performed successfully, approximately 80% bile flow can be achieved. However, the success rate after operations performed after 90 days is around 20%. If the bile flow normalizes after the surgery, the baby's jaundice decreases and the baby starts to have colored stools. Thus, time is gained for the baby to grow and develop. Since biliary atresia is a progressive disease, changes and damage to the liver continue. The definitive treatment is liver transplantation. Therefore, a successful Kasai surgery takes this period. It saves time for the patient.
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