Pediatric Primary Biliary Cholangitis and Hepatic Transplantation

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

Liver transplantation

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

Autoimmune hepatitis

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

Positive AMA, raised serum alkaline phosphatase, and bilirubin levels

Who performed the liver transplantation procedure for the patient?

Hepatobiliary consultant Signup and view all the answers

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

The natural history and exact incidence Signup and view all the answers

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

To report more PBC cases and manage them on time Signup and view all the answers

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

Middle-aged women between 30 and 50 years Signup and view all the answers

What is the approximate annual incidence of PBC?

2.7–3.5 cases per 10,0000 people Signup and view all the answers

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

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

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

Autoimmune destruction of intrahepatic biliary ducts Signup and view all the answers

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

For better understanding and management of PBC in children Signup and view all the answers

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

Had successful transplantation and post-operative recovery Signup and view all the answers

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

Portal, peri-portal inflammation, septal scarring, fibrotic cirrhotic stage 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?

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

What is the hallmark histologic finding in PBC?

Epithelioid cell granulomas with damaged bile ducts Signup and view all the answers

What is the FDA-approved medication for PBC patients?

Ursodeoxycholic acid Signup and view all the answers

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

Prednisolone or azathioprine Signup and view all the answers

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

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