About Biliary atresia with splenic malformation syndrome
What is Biliary atresia with splenic malformation syndrome?
Biliary atresia with splenic malformation syndrome is a rare congenital disorder characterized by the absence or underdevelopment of the bile ducts, which are responsible for carrying bile from the liver to the small intestine. It is also associated with malformations of the spleen, including hypoplasia (underdevelopment) or aplasia (absence). Symptoms of this condition include jaundice, dark urine, pale stools, and abdominal swelling. Treatment typically involves surgery to remove the affected bile ducts and to create a new connection between the liver and small intestine.
What are the symptoms of Biliary atresia with splenic malformation syndrome?
The symptoms of Biliary Atresia with Splenic Malformation Syndrome (BASM) vary depending on the severity of the condition. Common symptoms include:
-Jaundice (yellowing of the skin and eyes)
-Poor weight gain
-Abnormal blood tests (elevated liver enzymes, low albumin, and high bilirubin)
What are the causes of Biliary atresia with splenic malformation syndrome?
The exact cause of biliary atresia with splenic malformation syndrome is unknown. It is believed to be caused by a combination of genetic and environmental factors. Some research suggests that it may be caused by a viral infection, such as cytomegalovirus, during pregnancy. Other possible causes include exposure to certain medications or toxins, or a genetic mutation.
What are the treatments for Biliary atresia with splenic malformation syndrome?
1. Kasai Procedure: This is the most common treatment for biliary atresia with splenic malformation syndrome. It involves surgically connecting the bile ducts to the small intestine, allowing bile to flow from the liver to the intestine.
2. Liver Transplant: If the Kasai procedure is not successful, a liver transplant may be necessary. This is a major surgery and requires lifelong immunosuppressant medications.
3. Antibiotics: Antibiotics may be prescribed to help prevent or treat infections.
4. Vitamin K Supplementation: Vitamin K supplementation may be necessary to help prevent bleeding problems.
5. Dietary Changes: A special diet may be recommended to help reduce the risk of liver damage.
What are the risk factors for Biliary atresia with splenic malformation syndrome?
1. Premature birth
2. Low birth weight
3. Maternal diabetes
4. Maternal cholestasis
5. Maternal drug use
6. Maternal viral infection
7. Family history of biliary atresia
8. Genetic factors
9. Exposure to certain environmental toxins
Is there a cure/medications for Biliary atresia with splenic malformation syndrome?
There is no cure for biliary atresia with splenic malformation syndrome. Treatment focuses on managing the symptoms and complications of the condition. Medications may be prescribed to help reduce inflammation and pain, and to prevent infection. Surgery may be recommended to help improve the flow of bile from the liver. In some cases, a liver transplant may be necessary.