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Primary biliary cirrhosis in early childhood - A rare case report
Kaleem Ullah1, Shams Uddin1, Abdul Wahab Dogar1
1Pir Abdul Qadir Shah Jeelani Institute of Medical Sciences, Gambat, Sindh, Pakistan.
Insights
Primary biliary cirrhosis (PBC), a rare autoimmune liver disease, was diagnosed in a five-year-old girl. She successfully underwent liver transplantation, highlighting the need for increased awareness of PBC in children.
Area of Science:
- Hepatology
- Autoimmune Diseases
- Pediatric Gastroenterology
Background:
- Primary biliary cirrhosis (PBC) is a chronic, progressive autoimmune liver disease of unknown cause.
- It is characterized by inflammation and destruction of intrahepatic bile ducts, leading to fibrosis and cirrhosis.
- PBC is exceptionally rare in the pediatric population.
Purpose of the Study:
- To report a case of primary biliary cirrhosis in a five-year-old child.
- To emphasize the importance of recognizing PBC in pediatric patients.
- To highlight the successful management of end-stage liver disease due to PBC in a child.
Main Methods:
- A five-year-old female child presented with a six-month history of progressive jaundice and hepatic encephalopathy.
- Comprehensive diagnostic workup was performed.
- The patient underwent successful liver transplantation.
Main Results:
- The patient presented with end-stage liver disease requiring liver transplantation.
- The liver transplantation procedure was successful with an uneventful recovery.
- At six months post-transplantation follow-up, the patient is reported to be doing well.
Conclusions:
- Primary biliary cirrhosis is a rare condition in childhood with an unknown natural history and incidence.
- Increased awareness and reporting of pediatric PBC cases are crucial for better understanding and management.
- Liver transplantation is a viable treatment option for end-stage liver disease caused by PBC in children.
Introduction And Importance:
Primary biliary cirrhosis (PBC) is a chronic and progressive autoimmune liver disease with no known etiology. This disease is mainly characterized by granulomatous destruction of intrahepatic biliary ducts, severe peri-portal inflammation, and ultimate progress to liver fibrosis and cirrhosis. Here, we report a five-year-old girl diagnosed with PBC, presented to us with end-stage liver disease for liver transplantation. Our patient successfully underwent liver transplantation with an uneventful recovery. This case highlights the need for awareness to report further PBC cases in the pediatric age group.
Case Presentation:
A five-year old female child presented with a 6 months history of progressive jaundice. She had multiple admissions for hepatic encephalopathy and this time she was admitted for hepatic transplantation. On examination, she was icteric and had hepatomegaly. After thorough workup, she underwent successful hepatic transplantation and was alright post-operatively. At 6 months follow up, she is doing well.
Conclusion:
PBC is rare in childhood. The natural history and exact incidence of PBC in childhood are not known. Hence, there is a need for awareness to report further PBC cases in the pediatric age group.
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