The liver synthesizes components of digestion and blood clotting factors and serves to store energy and detoxify the blood. Its significance often under recognized the liver and biliary tract cannot be artificially synthesized, bypassed, or sustained outside of the body in times of failure. Often critically ill, many children born with liver and biliary tract abnormalities ultimately require liver transplantation for survival.
Congenital Anatomic Abnormalities
Choledochal cysts form outside and inside of the liver, resulting from abnormal development and growth of the bile ducts and gallbladder. If severe enough, they may block the flow of bile from the gallbladder into the intestine, resulting in liver toxicity. When symptomatic at birth, they cause jaundice in the newborn, requiring differentiation from the more serious disease biliary atresia. Biliary atresia, the fibrous obliteration of the biliary system beginning outside of the liver in the gallbladder and common bile duct, progresses and eventually involves the biliary tract throughout the liver. Although of unknown etiology, biliary atresia remains the most common indication for pediatric liver transplantation in the United States. Children born with biliary atresia develop liver failure, ultimately requiring liver transplant for survival. Liver biopsies most reliably aid in diagnosing biliary atresia.
Metabolic and Genetic Disease
Inherited liver diseases variably affect the liver, sometimes causing liver failure and death, unless patients receive a liver transplant. Common genetic conditions include tyrosinemia, alpha-1 antitrypsin disease, Wilson disease and glycogen storage disease. All of these inherited metabolic storage disorders often obstruct the liver's normal function. Diseases such as Alagille syndrome involve destruction of the bile ducts resulting in liver failure, but are associated with other abnormalities of the heart, eyes, vertebral column, and facial structure. Blood tests detecting abnormal levels of proteins or their specific genetic mutations aid in diagnosing these disorders.
Inflammatory and Immune Disease
Inflammatory cells surround, infiltrate, and destroy bile ducts in primary sclerosing cholangitis which has a strong association with ulcerative colitis in children. Radiographic studies revealing areas of narrowing along bile passages reliably aid in this diagnosis. Liver transplant is often required; unfortunately, some transplant recipients develop recurrence of disease in the new liver, says the Transplant Living website.
Infectious Liver Disease
Perhaps surprisingly, the most common form of infectious hepatitis in children has no identifiable etiologic agent. According to major transplant centers such as Cincinnati Children's Medical Center, these non-typable forms of hepatitis progress to liver failure requiring transplant in nearly in nearly 80 percent of patients. Hepatitis A rarely results in liver failure in children in the United States. Hepatitis B and C diagnosed in children usually result from direct infection from mothers before birth. Unusual in pediatric patients, liver transplantation for hepatitis B and C remains an option later in life because of progressive fibrosis and cirrhosis resulting in liver failure. Recurrence of disease after transplant is not uncommon. Other viral infections acquired before birth such as Herpesvirus and Cytomegalovirus may result in neonatal pediatric liver disease of varying severity.
References
- Cincinnati Children's Medical Center: Liver Transplantations
- "Robbins and Cotran Pathologic Basis of Disease, 7th Edition"; Kumar V, Abbas A, and Fausto N.; 2005.
- Transplant Living: Organ Donation and Transplantation Information for Patients
