About Liver Disease
Liver Diseases of Childhood
Liver disease occurs when liver cells and/or bile ducts are destroyed or impaired or when blood flow to or from the liver are decreased or blocked.
Symptoms of Liver Disease
- Jaundice (yellowing of eyes and skin)
Injured liver cells are unable to process and excrete waste products. When too much bilirubin, a normal waste product of red blood cells, accumulates in the body, a person’s skin turns yellowish-green color.
- Severe itching
The accumulation of bile salts may contribute to severe itching or pruritus.
- Dark urine
- Mental confusion or coma
Ammonia and other toxic substances which build up in the body can cause a brain dysfunction called hepatic encephalopathy
- Vomiting of blood
Portal hypertension also causes varices or enlarged vessels of the esophagus (the food tube that connects your mouth to your stomach). If there is increased pressure, bleeding or rupture of the vessels may occur. Common signs of bleeding or ruptured varices include blood in a person’s bowel movements, weakness or fainting.
- Easy bruising and tendency to bleed
- Gray or clay-colored stools
- Ascites (abnormal accumulation of fluid in the abdomen)
Ascites is due to increased or blocked blood flow through the liver or to decreased albumin, a protein that maintains pressure within the blood vessels. Edema, salt and water imbalance causes fluid to build up in the legs and arms.
- Other complications include infection, kidney failure, anemia and change in hair growth.
- Poor nutrition, another complication, causes fatigue, weakness and apathy.
Types of liver diseases
Biliary Atresia
Biliary atresia is a condition in which the bile ducts that drain bile from the liver to the intestine are blocked or absent. As a result of the blockage, the liver cannot perform its normal functions. The cause of the disease is unknown, but it is believed not to be hereditary. Symptoms of the disease may include poor weight gain and jaundice. Examples of testing performed to diagnose biliary atresia include a liver biopsy, a Tc-HIDA scan of the liver and an intra-operative cholangiogram. If preliminary tests indicate biliary atresia, your child will need exploratory surgery. A Kasai procedure may be performed in which the non-functioning portion of the bile duct is replaced with a piece of small intestine (jejunum). This will provide a route for bile drainage. The Kasai procedure may often be a temporary measure, although it can postpone a transplant for several years. Approximately 1/3 of patients have long-term, good results from the Kasai procedure; 1/3 of the patients develop cirrhosis in early or late childhood and require transplantation; and 1/3 of the patients develop rapid early cirrhosis and require transplant in early infancy.
Alagille’s Syndrome
Alagille’s syndrome is a disease that is inherited from one parent. The bile ducts inside and outside of the liver gradually disappear. Bile builds up in the liver because it is unable to drain through the narrow or damaged ducts. When the liver cells become damaged, the bile builds up and cirrhosis develops. Other symptoms frequently seen in this disease are itching, slow growth, jaundice and pale stools. Children with Alagille’s syndrome have a specific appearance – deep set eyes and a pointed chin.
Alpha-1-Antitrypsin Deficiency
Alpha-1 antitrypsin deficiency is a disease that is inherited from both parents. The children lack an enzyme, alpha-1 antitrypsin that helps break down proteins. The build up of proteins causes damage to liver tissue and leads to scarring. The symptoms are similar to those found with other liver diseases; slow growth, jaundice, fatigue, and an enlarged liver and spleen.
Chronic Active Hepatitis
Chronic hepatitis and eventually cirrhosis may occur when liver cells are damaged by the hepatitis B (HBV) or C virus (HCV). Hepatitis C is one of many viruses that cause inflammation of the liver. Healthy liver cells are replaced with scar tissue, reducing the ability of the liver to perform its many life supporting functions. Patients with chronic viral hepatitis experience fatigue, jaundice, loss of appetite and hepatosplenomegaly (enlargement of the liver and spleen). Additional information about hepatitis (A,B, or C) is available through the Children's Liver Association for Support Services: www.Classkids.org and through Parents for Kids with Infectious Disease: www.pkids.org.
Acute Viral Hepatitis
Hepatitis A, B and C are all examples of acute viral hepatitis. Hepatitis A is transmitted primarily through the oral-fecal route and has been traced to contaminated food and drinking water. Hepatitis B is transmitted by a needle stick or exposure to a blood transfusion or intimate sexual contact. Hepatitis C is also spread through exposure to blood products or a needle stick. Individuals who require multiple transfusions are at the highest risk for acquiring Hepatitis C. Acute viral hepatitis presents with “flu like” symptoms and jaundice. In addition, patients experience fatigue, itching, upset stomach, a low grade fever and dull pain on the right side of the abdomen.
Autoimmune Hepatitis
Autoimmune hepatitis is a form of hepatitis that is associated with circulating autoantibodies which are present for at least six months. It is a form of hepatitis that occurs in people who have no known exposure to a virus or metabolic abnormality. This form of hepatitis is probably related to an abnormal immunologic mechanism. Many of the presenting symptoms are similar to those found in chronic hepatitis, including arthritis-type pain, exhaustion and jaundice. However, it is different from viral hepatitis because it occurs more frequently in women. As the disease advances, the patient develops symptoms of end stage liver disease – exophageal varices, ascites and hepatosplenomegaly (enlargement of the liver and spleen).
Fulminant Hepatitis and Toxic Liver Failure
A small group of patients (less than 1 percent) may develop fulminant hepatitis in which the patient will fall into a coma. The cause of this type of hepatitis is unknown, and there is no specific treatment available. Possible complications include severe breathing problems, unstable heart rate, GI bleeding and kidney failure. Swelling of the brain may occur in young children and adolescents. Emergency liver transplantation may be necessary. Even with liver transplantation, the survival rate is approximately 50 percent.
The most common cause of toxic liver failure is excessive ingestion (overdose) of acetaminophen (Tylenol). It is important to recognize the diagnosis promptly because there is a specific medical treatment (N-acetylcysteine) which, if given promptly, usually prevents the worst complications and results in a good outcome. The usual clinical findings of acetaminophen overdose include neurologic symptoms such as disorientation, irritability and vomiting; in more severe cases,, coma and increased intracranial pressure, episodes of bleeding, unstable blood pressure, severe breathing problems, low sugar levels in the blood, kidney failure and infection may occur. In those cases, supportive care is provided., and the liver should recover completely. Occasionally patients may require liver transplantation if the liver is severely damaged.
Tyrosinemia
Tyrosinemia is inherited from both parents as an inborn error of metabolism. The features of the disease are similar to other end stage liver diseases: slow growth, enlarged liver and spleen, an increased tendency to bleed and a distended abdomen. Early liver transplantation is indicated for these patients because of a high incidence of secondary liver cancer.
Glycogen Storage Diseases
Glycogen storage diseases are due to a deficiencies of one of the enzymes that break down glycogen (the starch in which sugars are stored in the liver.) As a result of the enzyme deficiency,y of alpha-1,4 glycan-6-glucosyl transferase which causes toxic glycogen to accumulates in the liver and skeletal muscles. Frequent findings include an enlarged liver and spleen and poor weight gain and further down the road cirrhosis of the liver and portal hypertension. Children with this disease may experience low blood sugar without any symptoms.
Congenital Hepatic Fibrosis
Congenital hepatic fibrosis usually presents in late childhood with an enlarged liver, portal hypertension and esophageal varices. In addition to the cystic disease of the liver, 50 percent of these children will experience cystic disease of the kidney.
Prolonged Familial Intrahepatic Cholestasis (PFIC)
These diseases, which include Byler’s syndrome, are progressive diseases in which the child presents with neonatal cholestasis or the accumulation of bile in the liver. They are diseases that are inherited from one parent. The usual symptoms are jaundice, pruritus (itching) and an enlarged liver. Vitamin deficiencies are frequently found in children with this disease.
Wilson’s Disease
Wilson’s disease is a genetic disease in which there is an inadequate formation of ceruloplasmin, a copper transporting protein. Excessive quantities of copper are absorbed from the intestine, excreted in the bile, and stored in the liver. Water and food are sources of copper. Damage to liver tissue along with neurologic and emotional changes are frequent findings and diagnosis is made by low serum ceruloplasmin (a copper carrying protein), high urine copper and high liver copper. Genetic testing is now available for this disease.
Hepatoblastoma
Hepatoblastoma is a malignant tumor which occurs in infants and children. It is associated with polyposis coli (a precancerous growth in the large bowel or colon). It is usually a solid mass in the right lobe of the liver and may vary in size from 5 to 25 cm. Ninety-five percent of patients can be treated with a combination of surgery and chemotherapy. Rarely a tumor involving the entire liver can be treated with liver transplantation and chemotherapy.
