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

Ghadir MR, Bagheri M, Ghanooni AH - J Med Case Rep (2011)

Bottom Line: 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.

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Affiliation: Qom University of Medical Sciences, Qom, Iran. ghadir@ddrc.ac.ir.

ABSTRACT

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.

No MeSH data available.


Related in: MedlinePlus

Left hemispheric atrophy with severe dilation of the left ventricle.
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Figure 3: Left hemispheric atrophy with severe dilation of the left ventricle.

Mentions: She was removed from the transplantation waiting list and was admitted for staging and planning treatment strategies. During admission, she developed abdominal pain, worsening abdominal distention and hepatic encephalopathy. Because of a decreased level of consciousness and seizure, a brain CT scan was done which revealed unexpected left hemispheric atrophy with severe dilation of her left ventricle (Figure 3). Proper management accompanied by frequent abdominal paracentesis led to partial improvement.


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

Ghadir MR, Bagheri M, Ghanooni AH - J Med Case Rep (2011)

Left hemispheric atrophy with severe dilation of the left ventricle.
© Copyright Policy - open-access
Related In: Results  -  Collection

License
Show All Figures
getmorefigures.php?uid=PMC3105948&req=5

Figure 3: Left hemispheric atrophy with severe dilation of the left ventricle.
Mentions: She was removed from the transplantation waiting list and was admitted for staging and planning treatment strategies. During admission, she developed abdominal pain, worsening abdominal distention and hepatic encephalopathy. Because of a decreased level of consciousness and seizure, a brain CT scan was done which revealed unexpected left hemispheric atrophy with severe dilation of her left ventricle (Figure 3). Proper management accompanied by frequent abdominal paracentesis led to partial improvement.

Bottom Line: 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.

View Article: PubMed Central - HTML - PubMed

Affiliation: Qom University of Medical Sciences, Qom, Iran. ghadir@ddrc.ac.ir.

ABSTRACT

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.

No MeSH data available.


Related in: MedlinePlus