Liver Diseases We Treat at CHOC
At CHOC Children’s we work with patients and their families to diagnose and treat a variety liver diseases including:
Alpha-1 Antitrypsin Deficiency
Alpha-1 antitrypsin deficiency is an inherited condition that can cause liver and lung disease. The liver makes a protein called alpha-1 antitrypsin that goes into the bloodstream. Alpha-1 antitrypsin deficiency is one of the most common genetic causes of liver disease in children.
Alagille syndrome – also known as Alagille-Watson syndrome, syndromic bile duct paucity and ateriohepatic dysplasia – is an inherited disorder associated with liver, heart, eye and skeletal abnormalities. Liver problems can include cholestasis (stoppage of bile flow out of the liver), jaundice (yellow skin color) and too few bile ducts.
Biliary atresia is a chronic, progressive liver problem that becomes evident shortly after birth. 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. Learn more about biliary atresia.
A choledochal cyst is a congenital anomaly of the duct that transports bile from the liver to the gall bladder and small intestine. The liver produces bile to help digest food. When a child has a choledochal cyst, a swelling of that duct, bile may back up in the liver. This can cause liver problems or inflammation of the pancreas (pancreatitis) because it blocks the main duct from the pancreas gland to the intestine.
Cirrhosis is damage to the liver that decreases blood flow through it over time. As the normal liver tissue is lost, nutrients, hormones, drugs and poisons are not processed by the liver as they should. In addition, protein production and other substances produced by the liver are decreased or stopped all together. Learn more about cirrhosis.
Fatty Liver Disease
Someone who has fat deposits in their liver has fatty liver disease. These deposits may keep the liver from doing a good job of removing toxins from your blood. Learn more about fatty liver disease.
Hepatitis is an inflammation of the liver and can result in liver cell damage and destruction. Hepatitis is a concern because it often originates from a virus and can be spread to others. Learn more about hepatitis.
Metabolic Liver Disease
Two main metabolic disorders affect the liver:
- Hemochromatosis (also called iron overload disease). This disease is characterized by the absorption of too much iron from food. Instead of secreting the excess iron, the iron is stored throughout the body, including the liver and pancreas. The excess iron can damage these organs. Hemochromatosis is a hereditary disease that can lead to liver disease, liver failure, liver cancer, heart disease and diabetes.
- Wilson disease. This disease is characterized by the retention of too much copper in the liver. Instead of releasing the copper into the bile, the liver retains the copper. Eventually, the damaged liver releases copper into the bloodstream. This hereditary disease can cause damage to the kidneys, brain and eyes, and can lead to severe brain damage, liver failure and death.
Primary Sclerosing Cholangitis
Bile ducts serve as passageways to drain bile from the liver. In primary sclerosing cholangitis (PSC), inflammation causes the bile ducts to become too narrow and eventually they are blocked completely.