Bile Duct Atresia: Symptoms And Treatment
Bile duct atresia is a chronic and progressive liver disease that becomes visible shortly after birth.
The liver is an organ that contains well-known passages known as bile ducts. The function of these structures is to drain a substance that the liver produces, called bile, directly into the intestines and kidneys. Bile is essential for proper digestion and transports waste products from the liver to the intestines and kidneys to excrete them from the body.
These bile ducts are blocked in people suffering from bile duct atresia, which means that bile cannot reach the liver. This organ is thus damaged, which affects many vital functions in the body.
Bile duct atresia is the most common cause of liver transplants in children living in the United States. It occurs in one in 18,000 births. It is also a bit more common in women than in men. People of Asian or African descent or premature babies also suffer from this disease more often compared to Caucasian babies.
Causes of biliary atresia
So far, the causes of the development of this disease are unknown. Some researchers believe that babies are born with it, which means that it manifests itself during pregnancy while the liver is being developed.
Other experts believe that this disease manifests itself after birth, possibly due to exposure to toxic or infectious substances.
In principle, it is believed that bile duct atresia is not associated with medication that the mother took, illnesses she had or anything that she did during pregnancy.
In addition, it is currently unknown to what extent a genetic component is behind the development of this disease. However, the disease is unlikely to occur more than once in the same family.
The symptoms of biliary atresia
Although newborns suffering from this condition may appear normal at birth, jaundice (yellowish skin and mucous membranes) occurs in the second or third week after birth.
The baby can usually gain weight during the first month. After that time, the baby will lose weight, become more irritable and the jaundice will get worse. They may also develop other symptoms, such as:
- Slow growth
- Pale or clay-colored stools
- Dark urine
- Limited or no weight gain
- Splenomegaly (enlarged spleen)
Treatment of biliary atresia
The therapeutic management of bile duct atresia is very controversial, as 80-85% of those suffering from this disease will end up undergoing a transplant. As we already mentioned above, it is also an irreversible disease. No medicine can open the bile ducts or lead to the development of new bile ducts.
Today, a kasai postoenterostomy is considered the first choice for treatment. However, as we have mentioned above, it is controversial because many experts discuss the benefits of this intervention as the first choice of treatment, while others defend transplantation as the primary choice.
Kasai portoenterostomi
This surgery is aimed at draining bile.
Kasai postoenterostomy is more effective if performed before the baby is three months old. It also allows the baby to grow normally and have a relatively good health for many years. However, it is important to note that cholestasis can occur, which is an accumulation of bile in the liver that can cause liver damage.
Liver transplantation
As the name suggests, this surgery involves replacing a diseased liver with a healthy one. This new organ may come from a deceased donor, part of a liver from a deceased donor, or part of a liver from a living family member or other person whose tissue type is compatible with the patient’s tissue type.
After surgery, the baby’s health often improves rapidly. However, they will need medication to prevent the body from rejecting the new organ. This rejection is a normal protective mechanism that tries to defend the body against viruses, tumors and other foreign bodies.
