The Biliary Atresia Research Consortium (BARC) is a group of doctors, nurses, research coordinators and medical facilities in the U.S. working together to study infants with cholestasis (blockage of bile flow from the liver). Poor bile flow and buildup of substances in the liver can cause serious illness and lead to liver injury.
Liver disease in newborns is rare. In addition, there are not enough babies with cholestasis seen each year at a single pediatric liver center to adequately study the disease. Therefore the National Institutes of Health (NIH) is funding a network of ten pediatric liver centers across the United States to study causes and improve the diagnosis and treatment of infants and children with cholestatic liver disease.
Infants and children with cholestatic liver disease who receive their medical care at one of the participating BARC centers may be eligible to enroll in studies conducted by the BARC.
The Liver - An Introduction
The liver is the largest organ in the body. It is essential in keeping the body functioning properly. It removes poisons from the blood, produces agents to control infection, and removes germs and bacteria from the blood. It makes proteins that regulate blood clotting and produces bile to help absorb fats and fat-soluble vitamins.
Signs of Infant Liver Disease
In a baby there can be one or more signs that the liver is not working properly. The skin and eyes may be jaundiced (appear yellow). Jaundice is caused by a buildup of bilirubin in the blood. The abdomen may look swollen or stick out. The urine may be dark yellow or brown. The stools are often grey or white instead of green or yellow. There may also be bleeding or easy bruising. The blood might contain higher than normal levels of liver enzymes. The liver may feel large or look large on an x-ray. Jaundice, if it occurs in the first few days after birth and then goes away by about a week of age, is not harmful. This is called 'physiologic jaundice.'
Jaundice that remains or increases after a week from birth or 14 days for preterm infants, may be due to a build-up of bile in the liver (cholestasis) and requires further testing.
The Biliary Tract
Bile is a fluid made in and released by the liver. It flows from the liver, through the bile ducts and into the small intestine where it is needed for the digestion of fats. The gallbladder is an organ which stores bile produced by the liver. Eating signals the gallbladder to send bile down the bile ducts to the small intestine.
The Biliary Atresia Research Consortium (BARC) Network studies infants that are diagnosed before the age of six months with Cholestasis (blockage of bile flow). Cholestasis can happen for many different reasons. It can result from a brief illness or from injury to the liver; when these are the causes it will frequently go away without any special treatment. It can be caused by a number of inherited conditions (metabolic disease) that may need treatment. It can be caused by drugs and often goes away when the drug is stopped. Cholestasis can also be the result of more serious liver diseases such as idiopathic neonatal hepatitis or biliary atresia.
What Is Idiopathic Neonatal Hepatitis?
Idiopathic neonatal hepatitis is an irritation of the liver with no known cause. It occurs in newborn babies. Symptoms include jaundice and liver cell changes. It can resemble biliary atresia but is usually a short illness from which children can recover completely. Over 80% of infants with this disease fully recover. The cause of idiopathic neonatal hepatitis does not appear to be related to an infection.
What Is Biliary Atresia?
Biliary atresia is a condition in which the ducts that carry bile from the liver to the gallbladder and small intestine are not normal. These ducts can be injured, blocked, or missing. When the ducts that carry bile out of the liver are closed, bile backs up and damages liver tissue. In time the liver can develop scarring or cirrhosis. The gallbladder may also be abnormal or missing. The cause of Biliary Atresia is unknown. It occurs in one infant in every 15,000 live births. It is a serious disease which requires immediate surgical and medical treatment.
Other Cholestatic Liver Diseases of Infancy
Babies with jaundice and liver disease who are 0-180 days of age may be eligible for enrollment in BARC studies. Jaundiced babies who are enrolled in BARC and are found to have a diagnosis other than biliary atresia or neonatal hepatitis may also be eligible for enrollment in CLiC (Cholestatic Liver Diseases of Childhood) studies.
The five diseases being studied in CLiC are:
• Alagille Syndrome
• Alpha-1 Antitrypsin Deficiency (Alpha-1)
• Bile Acid Synthesis Defect
• Progressive Familial Intrahepatic Cholestasis (PFIC)
• Mitochondrial Hepatopathy