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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.

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

a 59 × 39 mm mass in the right liver lobe.
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Figure 2: a 59 × 39 mm mass in the right liver lobe.

Mentions: During preparation for transplantation, a 59 × 39 mm mass was observed in her right liver lobe, which was unnoted in previous evaluations (Figure 2). A percutaneous liver biopsy was reported to demonstrate HCC. Her serum alpha fetoprotein (AFP) level was 1900 IU/ml (normal < 6 IU/ml), serum glutamic oxaloacetic transaminase 72 IU/L, serum glutamic pyruvic transaminase 39 IU/L, alkaline phosphatise 120 IU/L, total bilirubin 3.2 mg/dL, direct bilirubin 2.3 mg/dL, albumin 2.7 g/dL, prothrombin time 16.1 seconds and her international normalized ratio 1.44.


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

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

a 59 × 39 mm mass in the right liver lobe.
© Copyright Policy - open-access
Related In: Results  -  Collection

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

Figure 2: a 59 × 39 mm mass in the right liver lobe.
Mentions: During preparation for transplantation, a 59 × 39 mm mass was observed in her right liver lobe, which was unnoted in previous evaluations (Figure 2). A percutaneous liver biopsy was reported to demonstrate HCC. Her serum alpha fetoprotein (AFP) level was 1900 IU/ml (normal < 6 IU/ml), serum glutamic oxaloacetic transaminase 72 IU/L, serum glutamic pyruvic transaminase 39 IU/L, alkaline phosphatise 120 IU/L, total bilirubin 3.2 mg/dL, direct bilirubin 2.3 mg/dL, albumin 2.7 g/dL, prothrombin time 16.1 seconds and her international normalized ratio 1.44.

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