All You Need to Know About Biliary Atresia

All You Need to Know About Biliary Atresia

Biliary atresia is the narrowing of the biliary tract, which occurs during development in the womb. The causes of this are largely unknown, but studies have found connections to certain viral diseases. A disease manifests itself in persistent jaundice after childbirth, colorless stools, brown colored urine, an enlarged liver and later an enlarged spleen, water retention and a tendency to bleed.

What is biliary atresia?

Bile duct atresia is a rare disease. It only occurs in newborns, the so-called neonatal period. This is the time from birth to the fourth week of life. It is an atresia of the biliary tract inside or outside the liver.

An atresia generally denotes the closure or non-placement of a body opening or a hollow organ. Regardless of whether the atresia is inside or outside the liver, biliary atresia affects the entire liver and bile ducts. Therefore, in the meantime, it has been decided not to speak of an extra- or intrahepatic form.

The incidence of an illness varies between every 3,000 and every 20,000 births, depending on the area. The greatest susceptibility is in the Pacific, while the rarest cases occur in Western Europe. In general, girls are affected somewhat more often than boys.

In around every tenth case, the occurrence is associated with other malformations such as heart defects or polysplenia. In this case, the diseases are grouped together as a syndromic form. If the bile duct atresia occurs alone, it is referred to as a nonsyndromic form.

Causes

Narrowing of the biliary tract, which ultimately leads to its atresia, begins early in the child’s development in the mother’s love. This can be concluded from studies of the liver with ultrasound technology. The reasons for this are largely unknown. However, a connection with certain viral diseases can be recognized from medical examinations.

These include the Ebstein-Barr viruses, which cause Pfeiffer’s glandular fever, and respiratory syncitial viruses, a typical cold virus. However, no connection to the hepatitis A viruses, B or C has been established. Genetic causes also follow from the results of several studies.

Studies show familial or ethnic clusters of occurrence. Certain HLA types are increasingly found in affected children. HLA is a group of genes that are central to the immune system. A cause can also be deduced from this.

Symptoms, ailments & signs

About half of all healthy newborns develop a weakly developed in the first days after jaundice. This is harmless and subsides after a week at the latest. In contrast to this typical neonatal jaundice, jaundice lasts longer in children with biliary atresia. This is due to the bilirubin.

It is a breakdown product of the red blood pigment hemoglobin and a bile pigment. Affected newborns pass acholic, i.e. discolored, stool and the urine takes on a brownish color. Another key symptom is hepatomegaly, an enlargement of the liver. In addition, a disease can be derived from accompanying symptoms such as malformations of the eyes, dysmorphism in the face and heart disease.

Course

The child’s development is not impaired in the first few months. It also shows weight loss and over-excitability. The increase in pressure in the portal vein of the liver causes the spleen to enlarge and water to accumulate in the abdomen. Furthermore, too little bile acid reaches the intestine. This needs the connection for the digestion of fat and for the absorption of vitamins. Vitamin K intake is particularly affected. A lack of this leads to a tendency to bleed.

Complications

The symptoms and complications of biliary atresia usually occur immediately after the child is born. The infant suffers from a relatively mild jaundice immediately after birth. There will also be discolored urine and bowel movements.

The spleen and liver can enlarge, and in most cases there is severe pain. As the disease progresses, other symptoms develop, such as heart defects or malformations of the eyes. In the worst case, the patient can become completely blind or suffer from further malformations in the area of the face.

If the biliary atresia is not treated, death will result. As a rule, the treatment is carried out by surgery. If this fails, a liver transplant can also be carried out. Often the parents of the child are also severely affected by biliary atresia and suffer from depression and other mental illnesses.

The support of a psychologist is definitely necessary. The further development of the child is largely normal and does not lead to further complications if the treatment of the biliary atresia has been carried out successfully. Life expectancy is also not minimized as a result.

When should you go to the doctor?

In many cases, facial dysmorphisms and eye misalignments are noticed by obstetricians and doctors in the newborn immediately after birth. This is followed by inpatient examinations, so that a diagnosis of biliary atresia is usually already available at this point in time. If the disease is not diagnosed immediately, the parents should consult a doctor as soon as they notice visual abnormalities on the face within the natural growth process of their child in the first few weeks of life.

Consultation with a doctor is also necessary once the newborn shows yellow skin. In addition, discoloration of the faeces or urine in infants is considered unusual. A doctor should be consulted as soon as the excretions are discolored or the urine has a brownish color. If there are malformations of the skeletal system, a doctor should also be consulted. If the child loses weight despite the recommended diet, there is cause for concern.

A doctor should be consulted as soon as the newborn continues to experience weight loss over several days. Consultation with a doctor is necessary if behavioral problems occur. If the child shows an overreaction, hyperactivity or aggressive behavior, a doctor must be consulted to determine the cause. If the abdominal girth increases abnormally, water can build up. These are to be examined and treated as soon as possible.

Treatment & Therapy

If left untreated, biliary atresia leads to death within the first few years of life. For treatment, an operation according to Kasai is initially performed. In this case, the connective tissue between the two pford vein branches of the liver and the changed bile tissue are removed.

In order to ensure that the intestine is supplied with bile, a loop of the intestine is then sewn onto the portal of the liver. There are also various medicinal products for therapy. These include drugs that slow down the remodeling of the liver, anti-inflammatory preparations and active ingredients that improve the flow of bile. However, their use is controversial.

The success of the Kasai operation is reflected in the increasing brown color of the stool and lighter color of the urine. Nevertheless, even in this case, many patients develop cirrhosis of the liver, which leads to death. It can be traced back to the increasing bile congestion.

At the latest now, or if the operation according to Kasai has failed, a liver transplant is necessary. This usually takes place at the age of two, but some cases require it earlier at six months. The availability of the necessary transplant organs has increased recently.

This is thanks to new processes. Since the liver is able to regenerate itself, donations of life are also possible. For this reason, it is also possible to split one donor organ between two recipients. This so-called split procedure is particularly suitable for children, as there are not many suitable organs for child liver transplants.

Outlook & forecast

If left untreated, biliary atresia has a very poor prognosis. The changes arise in the natural development and growth process of the fetus in the womb. If there is insufficient medical care immediately after the birth, the child will die within the first year of life. Surgical intervention is required, which is associated with the usual risks and complications.

If the operation is successful, there is an improvement in health. Nevertheless, there is a risk of life shortening even in patients whose corrective intervention leads to an adequate supply of bile to the intestine.

There is a risk of secondary diseases such as cirrhosis of the liver. A donor organ is required for this because life expectancy is also reduced without a transplant. The process up to the transplant is lengthy and the procedure is a challenge. The donated organ is not always accepted by the organism.

Despite various therapeutic approaches for biliary atresia and the possibility of alleviating the symptoms, patients with this disease can expect lifelong therapy. Physical resilience is limited in direct comparison to fellow human beings and various complications can be expected. The general lifestyle must be adapted to the health conditions so that survival is assured.

Prevention

Since the causes of biliary atresia disease are largely unknown, there are no known ways of preventing it. In addition, the fact that it is a congenital disease that occurs early in development poses a problem in the development of preventive measures. To counteract persistent jaundice, a low-fat, high-fiber diet is recommended.

Aftercare

In the case of biliary atresia, the options for follow-up care are severely limited. With this disease, the person affected is primarily dependent on direct medical treatment by a specialist in order not to reduce life expectancy and to avoid further complications. Self-healing is not possible, so medical treatment cannot be avoided.

If the bile duct atresia is not treated, the affected person will die after a few years. With this disease, those affected are mostly dependent on taking medication to alleviate the symptoms. Care must be taken to ensure that these medications are taken regularly and correctly, with the parents in particular having to ensure that they are taken correctly in the case of children.

Often possible interactions with other drugs should also be considered in order not to impair healing. Since the bile duct atresia also affects the internal organs, those affected should undergo regular examinations by a doctor in order to detect damage to the internal organs at an early stage. Contact with other biliary atresia patients can also be useful, as this leads to an exchange of information that can make everyday life easier.

You can do that yourself

Parents whose child has been diagnosed with biliary atresia should refer to the doctor’s suggestions for next steps. In general, several operations are performed. The healing process can be supported by rest and bed rest after an operation.

The child should not be exposed to stress for the first few weeks and must also be closely monitored by parents. Should further complaints or complications arise, a doctor must be consulted immediately. Accommodation in a specialist clinic may be necessary.

After a liver transplant, it should be ensured that the child is drinking enough fluids. In addition, the diet has to be changed. The child should eat as little fat as possible and instead eat a lot of fruit and vegetables. Adequate personal hygiene should also be observed in order to avoid infection and other complications.

Even shortly after the transplant, regular breathing exercises and physiotherapy exercises are useful to prevent infection of the respiratory tract. Regular check-ups by a doctor support these measures and ensure a symptom-free healing process. If unusual symptoms occur, it is best to contact the emergency medical service.

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