Jaundice (yellow- mellow) is a frequent entity in first few days of life. Most of the babies with this condition can get better with some or no intervention.
But, jaundice > 2 weeks duration in newborns or jaundice for any duration beyond 28 days period is a matter of concerns and warrants referral to Pediatric gastroenterologist and Hepatologist.
1. What is biliary atresia?
Biliary atresia is a chronic, progressive liver problem that becomes evident shortly after birth. Tubes in the liver, called bile ducts, normally allow a liquid produced by the liver called bile to drain into the intestines and kidneys. Bile aids in digestion and carries waste products from the liver to the intestine and kidneys for excretion. In biliary atresia, bile ducts in the liver are blocked. When the bile is unable to leave the liver through the bile ducts, the liver becomes damaged and many vital body functions are affected.
2. What causes biliary atresia?
The cause of biliary atresia is unknown. Some researchers and doctors believe that babies are born with biliary atresia, implying the problem with the bile ducts occurred during pregnancy while the liver was developing. Others believe that the disease begins after birth, and may be caused by exposure to infections or toxic substances.
Biliary atresia does not seem to be linked to medications the mother took, illnesses the mother had, or anything else the mother did during her pregnancy. Currently, there is not a genetic link known for biliary atresia. The disease is unlikely to occur more than once in a family.
3. How often does biliary atresia occur and who is at risk?
4. Why is biliary atresia a concern?
Biliary atresia causes liver damage and affects numerous processes that allow the body to function normally. Biliary atresia is a life-threatening disease and is fatal without treatment.
5. What are the symptoms of biliary atresia?
Infants with biliary atresia usually appear healthy at birth. Most often, symptoms develop between two weeks and two months of life, and may include:
Jaundice is a yellow discoloration of the skin and whites of the eyes due to an abnormally high level of bilirubin (bile pigment) in the bloodstream, which is then excreted through the kidneys. High levels of bilirubin may be attributed to inflammation or other abnormalities of the liver cells, or blockage of the bile ducts. Jaundice is usually the first sign, and sometimes the only sign, of liver disease.
Symptoms of biliary atresia may resemble other liver conditions or medical problems. Please consult your child's doctor for a diagnosis.
6. How is biliary atresia diagnosed?
A doctor or other health care provider will examine your child and obtain a medical history. Several diagnostic procedures are done to help evaluate the problem and may include the following:
The test that gives the most definitive diagnosis is a liver biopsy. A tissue sample is taken from your child's liver and examined for abnormalities, allowing biliary atresia to be distinguished from other liver problems.
7. What is the treatment for biliary atresia?
Biliary atresia is an irreversible problem. There are no medications that can be given to unblock the bile ducts or to encourage new bile ducts to grow where there were none before. Until that happens, biliary atresia will not be curable. However, two different operations can be done that will allow the child with biliary atresia to live longer and have a better quality of life. Your child's doctor can help determine whether either of these operations are an option:
After surgery, the new liver begins functioning and the child's health often improves quickly. After a liver transplant, children will need to take medications to prevent the body from rejecting the new organ. Rejection occurs due to one of the body's normal protective mechanisms that helps fight against invasion of viruses, tumors, and other foreign substances. Antirejection medications are taken in order to prevent this normal response of the body from fighting against the transplanted organ. Frequent contact with the hepatologist and transplant surgeon is crucial after a liver transplant.
The following is a list of some of the diseases that may cause chronic (long-standing jaundice usually > 2 months) hepatitis in children:
8. Metabolic liver disease :
Conditions that affect liver in 1st year of life and are life threatening:
PFIC 1,2 3 (progressive familial intrahepatic cholestasis)
Glycogen storage disease
Fatty acid oxidation defects
Amino acid metabolism defect .
Urea cycle defect
Maple syrup disease
Mitochondrial liver disease