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Congenital hepatic fibrosis leading to cirrhosis and hepatocellular carcinoma: a case report

INTRODUCTION: Congenital hepatic fibrosis is an uncommon cause of portal hypertension. Despite the presence of portal hypertension, hepatocellular and renal function are usually well preserved. Congenital hepatic fibrosis is included in the group of congenital diseases of fibropolycystic disorders....

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Detalles Bibliográficos
Autores principales: Ghadir, Mohammad Reza, Bagheri, Mohammad, Ghanooni, Amir Hossein
Formato: Texto
Lenguaje:English
Publicado: BioMed Central 2011
Materias:
Acceso en línea:https://www.ncbi.nlm.nih.gov/pmc/articles/PMC3105948/
https://www.ncbi.nlm.nih.gov/pubmed/21513523
http://dx.doi.org/10.1186/1752-1947-5-160
Descripción
Sumario:INTRODUCTION: Congenital hepatic fibrosis is an uncommon cause of portal hypertension. Despite the presence of portal hypertension, hepatocellular and renal function are usually well preserved. Congenital hepatic fibrosis is included in the group of congenital diseases of fibropolycystic disorders. These include a broad spectrum of clinical diseases which are usually accompanied by hepatic involvement. CASE PRESENTATION: We report the case of a 27-year-old Iranian woman with congenital hepatic fibrosis leading to cirrhosis and subsequently hepatocellular carcinoma. CONCLUSION: Advanced cirrhosis was diagnosed and our patient was scheduled for liver transplantation. During preparation for transplant, a hepatic mass was discovered which was found to be hepatocellular carcinoma. Radiofrequency ablation was performed and our patient was referred for transplantation.