Pediatric Primary Biliary Cholangitis and Hepatic Transplantation
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Questions and Answers

What is the treatment of choice for PBC patients with end-stage liver disease?

  • Kidney dialysis
  • Lung transplant
  • Liver transplantation (correct)
  • Heart bypass surgery

    • Which autoimmune disease is a well-known cause of chronic liver disease in childhood?

  • Rheumatoid arthritis
  • Autoimmune hepatitis (correct)
  • Systemic lupus erythematosus
  • Type 1 diabetes

    • What supported the diagnosis of PBC in the patient according to the AASLD guidelines?

  • Low serum alkaline phosphatase levels
  • Positive AMA, raised serum alkaline phosphatase, and bilirubin levels (correct)
  • Normal bilirubin levels
  • Negative AMA test

    • Who performed the liver transplantation procedure for the patient?

    <p>Hepatobiliary consultant</p> Signup and view all the answers

    What is not known about PBC in childhood according to the text?

    <p>The natural history and exact incidence</p> Signup and view all the answers

    Why is there a need for more awareness regarding PBC in the pediatric age group?

    <p>To report more PBC cases and manage them on time</p> Signup and view all the answers

    Which age group is predominantly affected by Primary Biliary Cirrhosis (PBC)?

    <p>Middle-aged women between 30 and 50 years</p> Signup and view all the answers

    What is the approximate annual incidence of PBC?

    <p>2.7–3.5 cases per 10,0000 people</p> Signup and view all the answers

    What is the role of anti-mitochondrial antibodies (AMA) in diagnosing PBC?

    <p>AMA are present in approximately 90% of PBC cases</p> Signup and view all the answers

    What is the initial autoimmune process in PBC that leads to the disease progression?

    <p>Autoimmune destruction of intrahepatic biliary ducts</p> Signup and view all the answers

    Why is it important to report further cases of PBC in the pediatric age group?

    <p>For better understanding and management of PBC in children</p> Signup and view all the answers

    What is the outcome mentioned for the patient who underwent hepatic transplantation?

    <p>Had successful transplantation and post-operative recovery</p> Signup and view all the answers

    What are the four successive patho-morphologic stages of PBC?

    <p>Portal, peri-portal inflammation, septal scarring, fibrotic cirrhotic stage</p> Signup and view all the answers

    What are the criteria defined by the American Association for the Study of Liver Diseases (AASLD) for patients suspected to have PBC?

    <p>High alkaline phosphatase level, positive AMA serology, liver biopsy showing non-suppurative intrahepatic biliary duct destruction</p> Signup and view all the answers

    What is the hallmark histologic finding in PBC?

    <p>Epithelioid cell granulomas with damaged bile ducts</p> Signup and view all the answers

    What is the FDA-approved medication for PBC patients?

    <p>Ursodeoxycholic acid</p> Signup and view all the answers

    In the advanced stage of PBC, which treatment combinations can be considered along with Ursodeoxycholic acid?

    <p>Prednisolone or azathioprine</p> Signup and view all the answers

    Why is staging with a needle biopsy rarely helpful in PBC?

    <p>Due to different grades of severity seen in various liver areas</p> Signup and view all the answers

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