Alagille Syndrome is an inherited disorder that closely resembles other forms of liver disease seen in infants and young children. However, a group of unusual features affecting other organs distinguishes Alagille syndrome from the other liver and biliary diseases of infants.
Alcohol – Related Liver Disease
Alcohol-related liver disease, as the name implies, is caused by excessive consumption of alcohol and is a common, but preventable, disease. For most people, moderate drinking will not lead to the disease.
Alpha-1 Antitrypsin Deficiency
Alpha-1 antitrypsin deficiency (Alpha-1) is a hereditary genetic disorder which may lead to the development of lung and/or liver disease. It is the most common genetic cause of liver disease in children. Adults can also be affected by Alpha-1 and may develop lung conditions such as emphysema as well as liver problems. Fortunately, many persons diagnosed with Alpha-1 never develop any of the associated diseases.
Autoimmune hepatitis is a disease in which the body’s own immune system attacks the liver and causes it to become inflamed. The disease is chronic, meaning it lasts many years. If untreated, it can lead to cirrhosis and liver failure.
There are two forms of this disease. Type 1, or classic, autoimmune hepatitis is the more common form. This is the form that mostly affects young women and is often associated with other autoimmune diseases. Type 2 autoimmune hepatitis is less common and generally affects girls between the ages of 2 and 14.
Benign Liver Tumours
Noncancerous, or benign, liver tumors are common. They do not spread to other areas of the body, and they usually do not pose a serious health risk.
A tumor is an abnormal growth of cells or tissues. Some tumors are malignant, or cancerous. Others are benign, or noncancerous.
Cancerous liver tumors can be fatal.
Biliary atresia is a disease of the bile ducts that affects only infants. Bile is a digestive liquid that is made in the liver. It travels through the bile ducts to the small intestine, where it helps digest fats. In biliary atresia, the bile ducts become inflamed and blocked soon after birth. This causes bile to remain in the liver, where it starts to destroy liver cells rapidly and cause cirrhosis, or scarring of the liver.
Cirrhosis of the Liver
Cirrhosis refers to the replacement of normal liver tissue with non-living scar tissue. It is always related to other liver diseases.
Galactosemia is an inherited disorder that prevents a person from processing the sugar galactose, which is found in many foods. Galactose also exists as part of another sugar, lactose, found in all dairy products. Normally when a person consumes a product that contains lactose, the body breaks the lactose down into galactose and glucose. Galactosemia means too much galactose builds up in the blood. This accumulation of galactose can cause serious complications such as an enlarged liver, kidney failure, cataracts in the eyes or brain damage. If untreated, as many as 75% of infants with galactosemia will die.
Gilbert Syndrome is a mild genetic disorder in which the liver does not properly process a substance called bilirubin. Bilirubin is made by the break down of red blood cells. Gilbert Syndrome affects three to seven percent of people in the United States. Gilbert Syndrome is more common in men than women.
Hemochromatosis is one of the most common genetic disorders in the United States. It is an inherited condition in which the body absorbs and stores too much iron. The extra iron builds up in several organs, especially the liver, and can cause serious damage. Without treatment, the disease can cause these organs to fail.
Iron is an essential nutrient found in many foods. Healthy people usually absorb about 10 percent of the iron contained in the food they eat to meet the body’s needs.
People with hemochromatosis absorb more than the body needs. The body has no natural way to rid itself of the excess iron, causing the excess to build up in the organs.
Hepatic Encephalopathy, sometimes referred to as portosystemic encephalopathy or PSE, is a condition that causes temporary worsening of brain function in people with advanced liver disease. When your liver is damaged it can no longer remove toxic substances from your blood. These toxins build up and can travel through your body until they reach your brain, causing mental and physical symptoms of HE.
Hepatitis A is a liver disease caused by the hepatitis A virus (HAV). HAV causes the liver to swell and prevents it from working well. HAV usually goes away on its own in almost all cases with no serious complications. However, HAV may cause some patients to suffer liver failure. Those at risk of serious long term effects from HAV include people with other liver diseases and people over 60.
Hepatitis B is a high preventable liver disease caused by the hepatitis B virus (HBV). HBV causes the liver to swell and prevents it from working well.
About 95% of adults who are exposed to HBV fully recover within 6 months (acute HBV) without medication. About 5% have HBV all their lives (chronic HBV) unless they are successfully treated with medications. Infants born to mothers infected with HBV are at high risk of developing chronic HBV. Chronic HBV can lead to cirrhosis (scarring) of the liver, liver cancer, and liver failure.
Hepatitis C is a disease caused by a virus that infects the liver. The virus, called the Hepatitis C virus or HCV for short, is just one of the hepatitis viruses. The other common hepatitis viruses are A and B, which differ somewhat from HCV in the way they are spread and treated.
Intrahepatic Cholestasis of Pregnancy (ICP)
Intrahepatic Cholestasis of Pregnancy (ICP) is a liver disorder which occurs during pregnancy. This condition affects the normal flow of bile. Bile acids are chemicals in the bile of the liver that help with digestion. With ICP the bile flow begins to slow down in tern the bile acids build up in the blood. This results in the woman itching that can vary in severity and type. The itching can be bothersome to severe itching and is often worse at night. There is rarely jaundice when experiencing this condition. Although it has been reported as early as a few 8 weeks pregnant, it is more common for it to begin in the third trimester, when hormone concentrations are at their highest levels. The figure for the percentage of women for whom Intrahepatic Cholestasis of Pregnancy will recur in future pregnancies is 60% or as high as 90% for severe ICP.
Jaundice is the yellowing of the skin and eyes. It results from having too much bilirubin in the blood. Bilirubin is a yellow substance made from the breakdown of red blood cells. Most newborns develop jaundice. Jaundice in newborns is usually mild and goes away within one to two weeks. However, babies with jaundice need to be regularly seen by a doctor because severe jaundice can cause brain damage.
The American Academy of Pediatrics (AAP) recommends that every newborn be checked for jaundice before leaving the hospital and three to five days after birth.
Lysosomal Acid Lipase Deficiency
Lysosomal acid lipase deficiency (LAL-D) is a rare, chronic, progressive inherited disorder. It affects the body’s ability to produce an enzyme called lysosomal acid lipase (LAL). This enzyme is needed for the breakdown of fats (lipids) and cholesterol in your cells. When the LAL enzyme is missing or deficient, fats accumulate in organs and tissues throughout the body, primarily leading to liver disease and high “bad cholesterol,” which is linked to cardiovascular disease.
Liver cancer is the growth and spread of unhealthy cells in the liver. Cancer that starts in the liver is called primary liver cancer. Cancer that spreads to the liver from another organ is called metastatic liver cancer. Hepatocellular carcinoma (HCC) is the most common type of primary liver cancer.
Liver cysts are abnormal sacs filled with fluid in the liver.
Non-Alcoholic Fatty Liver Disease
Non-alcoholic fatty liver disease (NAFLD) is the build up of extra fat in liver cells that is not caused by alcohol. It is normal for the liver to contain some fat. However, if more than 5% – 10% percent of the liver’s weight is fat, then it is called a fatty liver (steatosis).
Primary Sclerosing Cholangitis
Primary sclerosing cholangitis (PSC) is a chronic, or long-term, disease that slowly damages the bile ducts. Bile is a digestive liquid that is made in the liver. It travels through the bile ducts to the gallbladder and the small intestine, where it helps digest fats and fatty vitamins.
Reye syndrome is a rare illness that affects all bodily organs but is most harmful to the brain and the liver. It occurs primarily among children who are recovering from a viral infection, such as chicken pox or the flu. It usually develops a week after the onset of the viral illness but can also occur a few days after onset. Liver-related complications of Reye syndrome include fatty deposits, abnormal liver function tests, and poor blood clotting and bleeding caused by liver failure.
Wilson disease is an inherited condition that causes the body to retain excess copper. The liver of a person who has Wilson’s disease does not release copper into bile as it should. As the copper builds up in the liver, it begins to damage the organ. After enough damage, the liver releases the copper directly into the bloodstream, which carries the copper throughout the body. The copper buildup leads to damage in the kidneys, brain, and eyes. If not treated, Wilson’s disease can cause severe brain damage, liver failure, and death.