Ullah Kaleem, Uddin Shams, Dogar Abdul Wahab, Jan Zaka Ullah, Abbas Syed Hasnain
Pir Abdul Qadir Shah Jeelani Institute of Medical Sciences, Gambat, Sindh, Pakistan.
Pir Abdul Qadir Shah Jeelani Institute of Medical Sciences, Gambat, Sindh, Pakistan.
Int J Surg Case Rep. 2021 Aug;85:106215. doi: 10.1016/j.ijscr.2021.106215. Epub 2021 Jul 18.
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.
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.
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.
原发性胆汁性肝硬化（PBC）是一种病因不明的慢性进行性自身免疫性肝病。该疾病主要特征为肝内胆管的肉芽肿性破坏、严重的门周炎症，并最终进展为肝纤维化和肝硬化。在此，我们报告一名5岁被诊断为PBC的女童，因终末期肝病前来我们这里进行肝移植。我们的患者成功接受了肝移植，恢复顺利。该病例凸显了在儿童年龄组中提高对PBC病例报告意识的必要性。
一名5岁女童，有6个月进行性黄疸病史。她因肝性脑病多次入院，此次因肝移植入院。检查时，她有黄疸且肝肿大。经过全面检查，她成功接受了肝移植，术后情况良好。在6个月的随访中，她恢复良好。
PBC在儿童期罕见。儿童期PBC的自然病程和确切发病率尚不清楚。因此，有必要提高意识，在儿童年龄组中报告更多PBC病例。
