What is Biliary Atresia?
Biliary atresia is a life-threatening condition that occurs in infants where the bile ducts inside or outside the liver are damaged and do not have normal openings. The bile ducts may have formed abnormally whilst the foetus was still developing (foetal atresia) or else have become inflamed or obstructed shortly after birth (perinatal atresia).
Bile is a fluid made by the liver that helps the body digest fats and absorb fat-soluble vitamins, and also carries toxins and waste products out of the body. Bile ducts drain bile from the liver to the gall bladder for storage and to the small intestine for digestion. When bile ducts are damaged, bile builds up in the liver and causes damage which can lead to liver scarring, loss of liver tissue and cirrhosis – a chronic condition that prevents the liver from removing toxins from the blood and can lead to liver malfunction and failure.
Biliary atresia is a rare condition affecting approximately 1 out of 18,000 children worldwide, but you are not alone. The CLDF has a blog section where you can read stories from those affected by biliary atresia.
Cause
The cause(s) of biliary atresia are currently unknown, but are likely to be numerous and complex. It is not contagious and cannot be passed on from one child to another. There is no evidence to suggest that it is a heredity disease passed on to the child by their parents, or that it is caused by anything the parents may have done or taken.
Symptoms
Perinatal atresia is more common than foetal atresia and babies may appear well during the first few weeks of life.
The first main sign of biliary atresia is prolonged jaundice, a yellow discolouration of the skin and the whites of the eyes lasting longer than 2-3 weeks. Another sign is pale, white or grey stools. The Children's Liver Disease Foundation (CLDF) have a stool chart that can be used to compare your babies stools with healthy and potentially unhealthy stools. Normally the urine of a newborn baby is colourless, and a persistently dark or yellow coloured urine could also indicate liver disease.
These symptoms present because the liver is unable to remove bilirubin, a reddish-yellow product formed from the breakdown of the protein haemoglobin found in blood. Normally bilirubin is absorbed and processed in the liver and released in bile. When the bile ducts are blocked bilirubin builds up in the blood causing jaundice, enters the urine making it darker in colour, and cannot reach the intestines contributing to the pale stools.
In addition to these symptoms a low weight gain and growth of your baby may be an indication of liver disease.
Diagnosis
Several other liver diseases can cause similar symptoms as seen in biliary atresia so a series of tests are required to make a diagnosis. These may include:
During the Kasai procedure the damaged bile ducts are removed and a loop of intestine is brought up to replace them. Connecting the liver and small intestine in this way allows the bile to flow directly into the small intestine.
A Kasai procedure usually needs to be performed within the first 2-3 months of life before the liver becomes too damaged in order to be successful. If biliary atresia is untreated or the treatment was unsuccessful the build up of bile causes liver damage and eventually results in scarring and cirrhosis and if left untreated can be fatal. In these situations a liver transplantation is the definitive treatment and is usually required within the first 2 years of life. Biliary atresia is responsible for approximately 60% of liver transplants in children under the age of 2 years. Even if the Kasai procedure has been successful it is not a cure for biliary atresia and it is likely that the patient may need a liver transplantation in adulthood.
Nowadays liver transplantation is much more efficient and available and the majority of infants with biliary atresia now survive. New treatments can now use portions of an adult liver to be transplanted into the infant, and as healthy liver tissue grows quickly the infant can grow a complete liver over time.
Following a liver transplantation medication is required to prevent the immune system from rejecting the new liver along with other medications such as antibiotics or blood pressure medicines. A specialised diet and vitamin supplements might also be necessary.
Biliary atresia is a life-threatening condition that occurs in infants where the bile ducts inside or outside the liver are damaged and do not have normal openings. The bile ducts may have formed abnormally whilst the foetus was still developing (foetal atresia) or else have become inflamed or obstructed shortly after birth (perinatal atresia).
Bile is a fluid made by the liver that helps the body digest fats and absorb fat-soluble vitamins, and also carries toxins and waste products out of the body. Bile ducts drain bile from the liver to the gall bladder for storage and to the small intestine for digestion. When bile ducts are damaged, bile builds up in the liver and causes damage which can lead to liver scarring, loss of liver tissue and cirrhosis – a chronic condition that prevents the liver from removing toxins from the blood and can lead to liver malfunction and failure.
Biliary atresia is a rare condition affecting approximately 1 out of 18,000 children worldwide, but you are not alone. The CLDF has a blog section where you can read stories from those affected by biliary atresia.
Cause
The cause(s) of biliary atresia are currently unknown, but are likely to be numerous and complex. It is not contagious and cannot be passed on from one child to another. There is no evidence to suggest that it is a heredity disease passed on to the child by their parents, or that it is caused by anything the parents may have done or taken.
Symptoms
Perinatal atresia is more common than foetal atresia and babies may appear well during the first few weeks of life.
The first main sign of biliary atresia is prolonged jaundice, a yellow discolouration of the skin and the whites of the eyes lasting longer than 2-3 weeks. Another sign is pale, white or grey stools. The Children's Liver Disease Foundation (CLDF) have a stool chart that can be used to compare your babies stools with healthy and potentially unhealthy stools. Normally the urine of a newborn baby is colourless, and a persistently dark or yellow coloured urine could also indicate liver disease.
These symptoms present because the liver is unable to remove bilirubin, a reddish-yellow product formed from the breakdown of the protein haemoglobin found in blood. Normally bilirubin is absorbed and processed in the liver and released in bile. When the bile ducts are blocked bilirubin builds up in the blood causing jaundice, enters the urine making it darker in colour, and cannot reach the intestines contributing to the pale stools.
In addition to these symptoms a low weight gain and growth of your baby may be an indication of liver disease.
Diagnosis
Several other liver diseases can cause similar symptoms as seen in biliary atresia so a series of tests are required to make a diagnosis. These may include:
- Blood tests – In particular a test for bilirubin levels which are abnormally high in biliary atresia
- Urine tests
- An ultrasound of the liver
- Abdominal x-ray to check for enlarged liver and spleen
- A liver scan where dye is used to trace the flow of bile in the body and show if and where bile flow is blocked
- A liver biopsy to study the liver tissue
- Diagnostic surgery – if biliary atresia is suspected from the other diagnostic tests the final step is a surgical procedure where a paediatric surgeon directly examines the liver and bile ducts. If the surgeon confirms biliary atresia the primary treatment procedure, a Kasai procedure, will usually be performed immediately
During the Kasai procedure the damaged bile ducts are removed and a loop of intestine is brought up to replace them. Connecting the liver and small intestine in this way allows the bile to flow directly into the small intestine.
A Kasai procedure usually needs to be performed within the first 2-3 months of life before the liver becomes too damaged in order to be successful. If biliary atresia is untreated or the treatment was unsuccessful the build up of bile causes liver damage and eventually results in scarring and cirrhosis and if left untreated can be fatal. In these situations a liver transplantation is the definitive treatment and is usually required within the first 2 years of life. Biliary atresia is responsible for approximately 60% of liver transplants in children under the age of 2 years. Even if the Kasai procedure has been successful it is not a cure for biliary atresia and it is likely that the patient may need a liver transplantation in adulthood.
Nowadays liver transplantation is much more efficient and available and the majority of infants with biliary atresia now survive. New treatments can now use portions of an adult liver to be transplanted into the infant, and as healthy liver tissue grows quickly the infant can grow a complete liver over time.
Following a liver transplantation medication is required to prevent the immune system from rejecting the new liver along with other medications such as antibiotics or blood pressure medicines. A specialised diet and vitamin supplements might also be necessary.
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