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Biliary atresia, or fusion of the bile ducts, is a congenital defect that is one of the rare diseases. Untreated biliary atresia can lead to cirrhosis of the liver and even death. Research shows that 90 percent of untreated children die before the age of three. What are the causes and symptoms of biliary atresia? How is the treatment going?

Biliary atresia , or fusion of the bile ducts, is a congenital defect, the essence of which is inflammation of the bile ducts, i.e. bile ducts (the liquid secreted by the liver to digest fats) from liver into the small intestine. Inflammation leads to fibrosis of the bile ducts, and further to their obstruction. The consequence of this process is the stoppage of the flow of bile from the liver to the intestines ( cholestasis ). Bile builds up in the liver and bile ducts (over constriction) in excess, and over time it causes an increase in pressure in the bile ducts, which can damage liver cells.

The incidence of biliary atresia is estimated at about 1 in 20,000. kids. Girls get sick more often than boys.

Biliary atresia - causes

The reasons for the fusion of the bile ducts are unknown. It is assumed that the disease has an autoimmune background, i.e. cells of the immune system attack properly formed bile ducts, lead to their inflammation and further fusion.

The disease can also be genetically determined, although this applies to a smaller number of cases. It is also known that biliary atresia is not hereditary.

Biliary atresia - symptoms

Symptoms of biliary atresia appear in the first days or weeks of life, usually between weeks 2 and 6:

  • prolonged jaundice (yellow discoloration of the skin, mucous membranes, whites of the eyes). Jaundice occurs in many newborns and usually resolves within the first two weeks of life. If not, biliary atresia may be suspected
  • gray or light yellow clay stool
  • dark beer-colored urine
  • slightly enlarged liver (located in the upper right square of the abdomen)
  • prolonged bleeding from the navel or elsewhere

Additionally, 10-20 percent children with biliary atresia develop congenital defects, e.g. heart defects, double spleen, polycystic kidney disease.

Biliary atresia - diagnosis

If biliary atresia is suspected, abdominal ultrasound is performed, thanks to which the doctor can assess the function of the liver, bile ducts and gallbladder.

If biliary atresia is suspected, diagnostic tests must be performed very quickly

In addition, it is recommended to perform a bile duct scintigraphy.

Blood tests are also required. In the case of fusion of the bile ducts, increased levels of bilirubin, GGTP, cholesterol, alkaline phosphatase and slightly transaminases are observed.

These tests allow you to confirm the diagnosis and exclude other possible causes of the above-mentioned symptoms such as viral and bacterial liver infections or congenital metabolic diseases.

If the research does not give a clear answer to the question, what is the cause of the above-mentioned ailments, a liver biopsy may be necessary, which involves taking a fragment of the organ with a special needle for examination under a microscope.

Biliary atresia - treatment

The only method of restoring the fused bile ducts, and thus restoring the drainage of bile from the liver to the intestines, is surgery, which should be performed as soon as possible. The later the procedure is performed, the less effective it will be.

The surgical procedure is calledhepato-portal anastomosisorKasai procedure(named after the doctor who introduced it) and consists of complete removal of fibrotic extrahepatic bile ducts and sewing an intestinal loop into the surface of the liver. After the surgery, antibiotic therapy and analgesic treatment are applied.

In about half of the children, especially those who were operated on early (before 50 days of age), the procedure is successful. However, some of the youngest, even after a successful surgery, suffer from various progressive liver damage, which means that it is necessary to transplant it within the first two years of life.

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