Jaundice is a normal characteristic for many newborns, even those that are not premature. Jaundice is the yellowing of the skin and eyes that is caused by high amounts of bilirubin in the blood. Usually this condition resolves itself in a matter of days. Sometimes babies are put under special lights to treat the jaundice, however many are just sent home from the hospital with no treatment at all. Jaundice can be indicative of nothing, or it can mean that there is a serious underlying medical issue. One of the most serious diseases that affects the liver is a rare but deadly disorder called biliary atresia.
How Biliary Atresia Affects the Liver
Biliary atresia is a disease that affects the bile ducts of the liver. For some unknown reason, this disease causes the bile ducts to fill up with scar tissue, which creates blockages in the ducts. This means that the bile cannot get through the liver and into the intestines so that it can be processed out of the body. These blockages can occur inside the liver, or just outside the liver in what is called the "biliary tree", where the liver and the intestines meet. As the bile becomes trapped, it inflames the liver and can damage it irrevocably. As liver damage worsens, liver failure becomes more imminent.
Diagnosis of Biliary Atresia
Diagnosis of biliary atresia is notoriously difficult because there are many diseases that can cause jaundice. Blood tests are done to show how much bilirubin is in the bloodstream. A biopsy of the liver can be done to see what type of damage the cells are undergoing. But the most definitive way to ascertain whether or not an infant has biliary atresia is a procedure called a cholangiogram. During a cholangiogram dye is injected into the liver and its path is watched closely by the doctors. If there are narrowings or blockages of the bile ducts, the dye is unable to flow correctly through the ducts. In certain circumstances, an operation called the "Kasai" procedure is done. The doctors will remove the biliary tree that is inbetween the liver and the intestine. Using special surgical techniques, they will then attach the intestine directly to the liver. This is not always an option, nor does it always work if it is performed.
Options and Treatment For This Rare Disorder
For many patients faced with biliary atresia, a liver transplant is the only option. This does not mean that a patient with biliary atresia will need a transplant right away. Liver failure can happen quickly, but it can also span over time. In the meantime, certain medications that can help facilitate the bile flow through the liver along with medications that can help support the liver can be prescribed.
Although this disease is very rare, there are resources available that can help answer questions. One of those resources is the Biliary Atresia Research Consortium. This was formed by a group of leading pediatric hospitals to do research on biliary atresia. The hope is to find the cause of the disease, which can lead to understanding biliary atresia better and maybe even preventing it.
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