Liver Diseases
Some liver diseases can be treated. But when a child reaches or will soon reach end-stage liver disease, or liver failure, liver transplant is the treatment of choice.
Conditions that can lead to liver failure include short-term (acute) diseases, long-term (chronic) damage to the liver, liver tumors and metabolic disorders.
Acute Liver Failure
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Acute hepatitis, including infectious A, B and C viruses
Short-term inflammation of the liver is called acute hepatitis. It can damage liver cells and keep the liver from working properly. In children, the cause is usually infection with a virus. Other conditions that affect the liver can also lead to hepatitis. These include autoimmune liver disease. Read more about viral hepatitis.
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Fulminant hepatic failure (FHF)
Doctors use this term when the liver suddenly stops working in a person who had no known liver disease. At first, the symptoms may seem like hepatitis, but they get worse quickly. FHF leads to severe health issues, like the blood not being able to clot properly. Children may show signs (like confusion or slurred speech) that mean the brain is not working normally because the liver is not clearing toxins from the blood. This brain problem is called hepatic encephalopathy.
Chronic Liver Failure
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Alagille syndrome
Alagille syndrome is an inherited (genetic) disorder. It can affect multiple organs, including the liver. Children with Alagille syndrome may have too few bile ducts in the liver. This causes problems with the way bile moves and makes it hard for the body to remove toxins.
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Alpha-1-antitrypsin (AT) deficiency
Alpha-1-AT deficiency is an inherited (genetic) disease that causes the body to make too much of a protein called alpha-1-AT. This protein gets stuck in the liver instead of moving into the bloodstream to help other body parts, like the lungs. If the liver isn't able to get rid of it, the extra protein can scar the liver and damage it.
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Autoimmune hepatitis
Autoimmune hepatitis is a chronic disease of the liver that happens if your child's own immune system attacks their liver cells. The trigger for the immune attack is not well understood, but there is a higher risk in families with other autoimmune diseases. A child with this condition may have sudden, severe liver failure. Or they may have no clear symptoms until the immune system slowly damages their liver over a long time, causing cirrhosis.
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Biliary atresia
Biliary atresia happens when a baby's bile ducts do not form normally. Instead of being open tubes to drain bile from the liver, the bile ducts are missing, or they are blocked because of scarring. This keeps bile from flowing out of the liver. Instead, bile backs up in the liver. Backed-up bile (congestion) in the liver is called cholestasis. It causes scarring (cirrhosis) and a yellowish tint to the skin and eyes (jaundice). Read more.
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Chronic hepatitis B and C
If the virus that causes hepatitis B or C stays in the body for longer than 6 months, doctors consider the infection chronic. Most children with chronic hepatitis B or C do not have symptoms. But they might need treatment to prevent health problems later, like cirrhosis and liver cancer. Read more.
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Cirrhosis
Cirrhosis is severe scarring of the liver. In people with cirrhosis, scar tissue replaces healthy tissue over time. This causes less blood to flow through the liver and makes the liver unable to work as well. While many people think cirrhosis is caused by alcohol use, it can have other causes, such as hepatitis, fatty liver disease, diseases that damage or destroy the bile ducts and metabolic liver disease.
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Cystic fibrosis liver disease
Cystic fibrosis is a condition that causes thick, sticky mucus to build up and clog some parts of the body. Most often, the mucus clogs the lungs and pancreas, but it can also affect the liver. When mucus clogs the liver, fluids are not able to flow as they should. This can cause scarring or swelling and affect the way the liver works. Read more.
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Primary sclerosing cholangitis (PSC)
PSC is a chronic liver disease caused by inflammation and scarring of the bile ducts of the liver. The scarring happens slowly over time. It may lead to jaundice (a yellowish tint to the skin and eyes), itching and cirrhosis. Doctors think the inflammation might happen because the child’s own immune system attacks cells in the bile ducts (autoimmunity). Many people with PSC also have inflammatory bowel disease.
Tumors
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Hepatoblastoma
This is a type of liver cancer. It is most common in babies and children younger than 5 years old. Usually, children with liver cancer have surgery to remove only part of their liver, along with other treatments. In very rare cases, doctors need to remove the whole liver and transplant a healthy liver from a donor. Some other liver conditions can increase the risk for hepatoblastoma. They include glycogen storage disease. Read more.
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Hepatocellular carcinoma
This type of liver cancer usually affects older children and teens, as well as adults. People are at greater risk if they have another liver condition, such as Alagille syndrome, glycogen storage disease, hepatitis B that spread from parent to child at birth or progressive familial intrahepatic cholestasis. Read more.
Metabolic disorders
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Crigler-Najjar syndrome
Bilirubin is a yellow substance formed as a normal part of red blood cells breaking down. Usually, the liver filters bilirubin from the blood. Then, it becomes part of the bile and leaves the body in the stool (feces). In Crigler-Najjar syndrome, bilirubin cannot dissolve in water and be passed out of the body. Instead, it builds up in the blood, liver, spleen and other parts of the body. Over time, this can damage the brain and nervous system.
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Progressive familial intrahepatic cholestasis (PFIC)
Cholestasis means the flow of bile is reduced or blocked. Several liver problems can scar the bile ducts or make them swollen, which can block bile. These problems include PFIC, a group of inherited (genetic) diseases of the liver that tend to worsen over time. Children with this condition are born with a liver that cannot clear toxins properly. Depending on which genes are affected, the disease may be mild or severe.
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Glycogen storage disease (GSD)
Glycogen is the form of sugar stored in the liver and muscles to be used later for energy. In GSD, the body cannot make, store or use glycogen properly. This happens because the body is missing an enzyme (or doesn't have enough of the enzyme). As a result, glycogen may build up in the liver, form in an abnormal way that the body cannot use or trigger an immune reaction that scars the liver or other organs.
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Maple syrup urine disease (MSUD)
MSUD is a rare genetic disorder. It happens when the body cannot break down certain parts of proteins due to too little of an enzyme. The disease can lead to a build-up of toxic substances that damage organs, including the brain. Learn how a family with four children with MSUD have managed this disease.
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Propionic acidemia (PA)
This is an organic acid disorder. Children with these disorders cannot properly use protein for energy. This can cause toxic acids to build up in their bodies. They may then have problems with growth, learning and overall health. PA happens due to lack of an enzyme.
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Urea cycle disorders
Children with these disorders cannot use protein properly for energy. This can cause toxic by-products to build up in their bodies. The build-up can lead to problems with growth, learning and overall health. Examples of urea cycle disorders are ornithine transcarbamylase (OTC) deficiency, argininosuccinic aciduria and citrullinemia.
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Wilson disease
Children with Wilson disease cannot get rid of extra copper they receive from their diet but do not need. Most children's bodies filter out this extra copper. But for children with Wilson disease, the copper builds up and can damage their liver, brain and other organs.