The liver is the largest solid organ in the human body. It lies within the tummy on the right side of under the ribs, just below the lungs. The liver has two lobes and further divided into 8 segments.
The liver and Bile Duct System
Unlike other organs in the human body. The liver’s blood supply is double by two vessels that supply blood, oxygen, and nutrients. The portal vein brings blood from the digestive system and accounts for 70-80% of the liver’s blood supply. The hepatic artery carries 20-30% of blood to the liver. Liver tumors get to take blood supply from branches of the hepatic artery. The liver is your body’s metabolic factory and helps to control more than 500 chemical reactions, including.
- Storing carbohydrates.
- Breaking down proteins.
- Converting ammonia to urea; a substance found in urine.
- Using fats to provide energy.
- Producing cholesterol which needed for the production of bile (which in turn helps to digest fats in the food) and hormones.
- Storing minerals and vitamins
- Processing blood (breaking down old red blood cells and producing clotting factors).
Biliary atresia is a liver disease affecting small children. It causes damage, scarring, and blockage of the bile ducts. In healthy babies, bile ducts carry bile from the liver to the gallbladder and eventually to the small intestine. Bile includes chemicals that the body trying to get rid of. When the bile ducts are blocked, bile flow stops. This causes these bad chemicals to collect in the liver. This is called cholestasis. Biliary atresia only occurs in young infants (less than 3-4 months of age). Biliary atresia a rare disease affecting 1 in 8,000 to 1 in 18,000 live births worldwide. About 10-20% of infants with biliary atresia abnormalities in other organs. Such as heart defects or issues with their spleen.
Causes of Biliary Atresia:
The cause of biliary atresia is unknown. A number of ideas being tested. One theory suggests viral infection as the possible reason others believe. it may cause by an overactive immune system, a problem in the development of the bile duct, or a chemical or something toxic that damages the bile duct. Genetic reason or a combination of these things also been a postulated cause of biliary atresia.
Regardless of the cause and biliary atresia leads to inflammation and scarring of bile ducts outside and inside the liver. The scarring can lead to cirrhosis (severe scarring) by 6 to 12 months of age. When a liver is badly scarred it cannot do its job correctly.
Symptoms of Biliary Atresia:
Infants with biliary atresia have jaundice (yellowing of eyes and skin). Jaundice is normal in many newborns, but clear off by 2 weeks of age and particularly in breastfed babies. Jaundice part of biliary atresia caused by a substance called “bilirubin” building up in the blood. Any infant with jaundice that lasts past 2 weeks of age should be seen by a doctor. The doctor can check the baby’s blood to see if the liver working correctly. Infants with biliary atresia may also have a stool color that is lighter than normal. This is called “acholic stools” and the result of bile (which gives stool color) not getting to the intestines.
Infants with biliary atresia may also dark urine, a large liver, or a large spleen. If infants have problems in other parts of the body such as heart defects. they can a heart murmur, low oxygen levels, or poor growth. Later on, infants develop fluid in the belly (ascites) and itchiness. In healthy babies and children, bile helps with digestion and absorption of food and vitamins. Infants and children who don’t have good bile flow. because biliary atresia may a hard time gaining weight and absorbing nutrients and typically has “failure to thrive”.
Biliary Atresia diagnosed:
Infants who have jaundice that lasts beyond 2-3 weeks of age or who have light-colored stools should be seen by a doctor. The doctor can check the baby’s blood to see if the liver working correctly. very important to diagnose biliary atresia as soon as possible. because scarring and cirrhosis in the liver can occur rapidly if the disease not treated. To help make the diagnosis, the medical team may order a number of tests, including.
- Blood and urine tests looking for other common or treatable causes of jaundice.
- Ultrasound to look at the bile ducts, gall bladder, and liver.
- Liver biopsy
- HIDA scan (hepatic scintigraphy) to see if bile getting into the intestine.
If these tests point to a possible diagnosis of biliary atresia, your doctor may order the intraoperative cholangiogram. a surgical operation where the bile ducts searched for and then dye injected directly into the gallbladder to get the best look possible at the bile ducts. If bile ducts cannot be seen by this testing, then the surgical operation is continued to treat the biliary atresia by the Kasai procedure.
Biliary Atresia treated:
If the testing and cholangiogram show biliary atresia, infants need a surgical operation for treatment, known as the Kasai procedure or hepatoportoenterostomy. In this procedure, the blocked bile ducts are removed and a piece of the small intestine directly connected to the liver in place of the bile duct. This allows bile to leave the liver and enter the intestine. Other supportive medical treatments recommended before and after the Kasai procedure. These include making sure nutrition is ideal by using infant formulas with higher calories or certain types of fat. Doctors might also use additional vitamins, ursodeoxycholic acid, and low-dose antibiotics to prevent bacterial infection of the bile ducts (cholangitis). When the Kasai procedure is successful, the stools become yellow or brown. The bilirubin normalizes in the blood and jaundice disappears, and nutrition and wellbeing improve.
Outlook for infants diagnosed with Biliary Atresia:
The chance of a successful Kasai procedure is best if the procedure is done before 30-45 days of age. Occasionally, infants who not diagnosed until after 3-4 months of age and already have cirrhosis at the time of diagnosis, do not a Kasai procedure. These infants and then listed for liver transplantation without undergoing a Kasai.
If the Kasai procedure is successful, jaundice disappears and bilirubin levels return to normal. This happens in 40-60% of infants having a Kasai procedure. Even with the Kasai procedure and many children with biliary atresia still develop more scarring in the liver and eventually need a liver transplant.
Biliary atresia is the most common reason for liver transplantation in children. About half of infants who undergo a Kasai procedure will still need liver transplantation by three years of age. In about one-quarter of infants, jaundice will go away at first, but those children will need liver transplantation by the teenage years. because of slowly progressive liver scarring. With our current therapies, only about one-quarter of babies who have the Kasai procedure will survive to their 20’s without needing liver transplantation.
Although liver transplantation a major surgical procedure requiring long term immunosuppression medicines. this life-saving procedure markedly improved survival rates of infants and children with biliary atresia. Long-term survival after liver transplantation for biliary atresia over 90%.
Mr. Touseef Ahmed
Transplant Coordinator: (for post-transplant follow up patients):
Hepatobiliary & Pancreatic Surgery Services:
Muhammad Asif 0341-0543883
Qaiser Rafiq John
24 Hours Patients emergency Helpline:
042 111 117 554