Biliary Atresia


Biliary atresia is a condition where the bile ducts are not formed or does not develop normally. Biliary atresia is a liver disease in children who contributed to 50-60% of liver transplants. The function of the biliary system is to remove metabolic wastes from the liver and transports bile salts needed to digest fats in the small intestine. In biliary atresia there clogging the flow of bile from the liver to the gallbladder. This can cause liver damage and cirrhosis of the liver, which if untreated can be fatal. The incidence of biliary atresia throughout the world 1: 10,000 live births. Biliary atresia is a liver disease in children who contributed to 50-60% of liver transplants.

Causes
The cause is a combination of various things such as viral infections especially reovirus and rotavirus, genetic disorders, toxic material that interferes with the growth of the biliary tract and the presence of perinatal bile duct damage during delivery.

Symptoms
Symptoms usually occur within 2 weeks after birth, which are:
  • Dark-colored urine baby
  • Pale stools
  • Yellow skin
  • Do not gain weight or slow weight gain
  • Enlarged liver.

By the time the baby reaches the age of 2-3 months, will arise the following symptoms:
  • Impaired growth
  • Itching
  • Fussy
  • High blood pressure in the portal vein (blood vessels that carry blood from the stomach, intestines and spleen to the liver).

Type of biliary atresia
There are 3 (three) types of biliary atresia:
  1. Type I, atresia of the common bile duct;
  2. Type II atresia of the duct while hepatikus
  3. Type III obstruction or blockage of the bile duct more upstream in the liver tissue at the porta hepatis and the channel above the porta hepatis.
Most patients with biliary atresia, including the type III, which is as much as 90%.

Pathophysiology

Pathophysiology of biliary atresia is not known with certainty. According to figures histopatologik, it is known that biliary atresia occurs because of prolonged inflammatory processes that cause extrahepatic biliary ducts are progressively damaged. In the advanced state of the inflammatory process spread to the intrahepatic biliary ducts, so it will experience a progressive damage.

Diagnosis
Diagnosis based on symptoms and physical examination.
On abdominal examination, palpable enlarged liver.

Regular checks carried out:
  • Blood tests (there are elevated levels of bilirubin)
  • abdominal ultrasound
  • X-ray abdomen (liver appears enlarged)
  • Cholangiography
  • Liver biopsy
  • laparotomy (usually done before the baby is 2 months old).

Treatment

Kasai operation, according to the name of the Japanese doctor who discovered the technique of the operation, then the technique was performed bypass surgery which makes bile duct from the liver directly into the duodenum or the duodenum. Successful Kasai operation, particularly in type I biliary atresia and a little on the type II. Whereas in type III, the Kasai operation is not very satisfactory results. As a result, the disease will progress to cirrhosis of the liver in which liver texture is soft and supple previously become hard like stone.
In all organic disease that has reached the final stage, there is no other way to treatment than do organ transplants in this case the liver or liver transplant. Special liver transplant in infants is actually relatively easy to find a donor, which is one of the parents, could be the father or mother of the baby. Depending on the results of examination of whether the father or mother of the most close and match blood groups and other test results such as MHC type I, type II MHC, etc..


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