Training in Hepatobiliary Disorders

The overall goal of training in pediatric hepatology is to prepare the trainee to evaluate, diagnose, and treat routine and unusual causes of acute and chronic liver and biliary tract disorders and to understand the impact of such illnesses on the child and family. Trainees should demonstrate a thorough understanding of the age-related differences in physiology, drug metabolism, and nutritional requirement in order to understand and manage these disorders effectively. Regularly scheduled conferences will include didactic lectures, case discussions, literature reviews, and research topics in the area of hepatobiliary diseases. In addition, there will be conferences in which radiologic and histologic findings are presented and discussed, as part of the training. A rotation in liver transplant medicine will also afford trainees with a rich learning experience in hepatobiliary disorders.

At the end of training the trainee will be able to:

• Understand the basic embryology, biology, and pathobiology of the liver and biliary system in the developing infant and child
• Develop differential diagnostic skill for common initial symptoms of pediatric liver disease (neonatal cholestatic disorders, jaundice, cholestasis, hepatomegaly, hepatic mass, splenomegaly and portal hypertension, steatorrhea, failure to thrive, pruritus, fat-soluble vitamin deficiencies, gastrointestinal hemorrhage, and fulminant liver failure)
• Diagnose intra- and extrahepatic biliary atresia, choledochal cyst, neonatal hepatitis, metabolic liver diseases, paucity of interlobular bile ducts, cystic liver diseases and other cholangiopathies, parenteral nutrition-related cholestasis, perinatal infections (viral, bacterial, protozoan, spriochetal), and hypoxic and ischemic liver injury
• Learn the diagnosis and possible treatment of metabolic liver diseases including alpha-1-antitrypsin deficiency, cystic fibrosis, galactosemia, hereditary fructose intolerance, tyrosinemia, Wilson disease, hemochromatosis, peroxisomal disorders, bile acid synthesis defects, neonatal iron storage disease, fatty acid oxidation defects, glycogen storage diseases, lipid storage diseases, urea cycle defects, progressive intrahepatic cholestasis, and other hereditary forms of cholestasis and Reye syndrome
• Establish the diagnosis of the disorders of bilirubin metabolism including physiologic indirect hyperbilirubinemia, Gilbert syndrome, breast milk-associated jaundice, Crigler-Najjar syndrome (types I and II), and Dubin-Johnson and Rotor syndromes
• Diagnose acute and chronic hepatitis caused by viruses (e.g., hepatitis virus types A, B, C, D, and E, and herpes viruses), drugs, and toxins
• Acquire knowledge of the biology, consequences, treatment and prevention (where such knowledge exists) of both vertically and horizontally transmitted viral
• Diagnose autoimmune and immune hepatobiliary disorders, including autoimmune hepatitis, primary sclerosing cholangitis, graft-versus-host disease, liver allograft rejection, and liver abnormalities associated with autoimmune disorders
• Recognize and treat drug- and toxin-related acute and chronic liver disease, including alcohol-related disease in the adolescent
• Able to recognize and treat fulminant hepatic failure and hepatic coma and its associated complications, such as cerebral edema, hepatorenal syndrome, and coagulopathy
• To diagnose cirrhosis and treat its complications, including portal hypertension, esophageal varices, ascites, portosystemic encephalopathy, hepatorenal syndrome, and spontaneous bacterial peritonitis
• Learn the reasons of gallstone formation and gallbladder diseases in infancy and childhood
• Be familar with hepatobiliary disorders and infections encountered in the immune-deficient or immune-suppressed pediatric patient
• Learn the recognition, diagnosis and prognosis of hepatic tumors and masses occurring in childhood
• Evaluate and manage the nutritional complications of pediatric hepatobiliary diseases
• Learn the role of radiologic, nuclear, and other imaging procedures in a cost-effective and age-appropriate evaluation and management
• Be familiar with the pharmacology, indications, age-appropriate dosages, toxicity, and side effects of commonly used medications for treatment of hepatic disorders, including antiviral and immunosuppressive agents
• Know the recommended schedules and programs for active and passive immunization of pediatric patients against hepatitis viruses and the impact of underlying liver disease or liver transplantation on these and other immunization schedules
• Acquire appropriate knowledge of the histologic features of childhood liver disorders at both light and electron microscopic levels
• Acquire technical skill and cognitive competence in the performance of diagnostic and therapeutic procedures essential to the evaluation and management of pediatric hepatobiliary disorders
• Be familiar with the indications, contraindications, and complications of percutaneous liver biopsy, transjugular intrahepatic biopsy, ERCP, paracentesis, sclerosis or band ligation of esophageal varices
• Be familiar with indications, contraindications, complications, of portosystemic shunting procedures (TIPP, surgical)
• Have a basic understanding of the indications for liver transplantation
• Participate in the appropriate evaluation and preparation of a child for transplantation
• Be familiar with the types of transplantation options available for pediatric patients
• Have expertise in the long-term management of the child who has undergone transplantation

During the fellowship program, a trainee is expected to have performed the requisite number of percutaneous liver biopsies under supervision to satisfy the requirements of the Academy of Pediatrics’ sub-board of Pediatric Gastroenterology and Hepatology.