scholarly journals Hyperammonemic encephalopathy in patients with fibrolamellar hepatocellular carcinoma: a new physiopathological pathway

HPB ◽  
2019 ◽  
Vol 21 ◽  
pp. S858-S859
Author(s):  
R. Cañada Surjan ◽  
E. Santana dos Santos ◽  
F. Ferrari Makdissi ◽  
T. Basseres ◽  
M. Autran Cesar Machado
Keyword(s):  
Hepatocellular Carcinoma ◽  
Fibrolamellar Hepatocellular Carcinoma ◽  
Hyperammonemic Encephalopathy

scholarly journals Fibrolamellar Hepatocellular Carcinoma and Noncirrhotic Hyperammonemic Encephalopathy

2018 ◽  
Vol 2018 ◽  
pp. 1-6 ◽  
Author(s):  
Oscar Suarez ◽  
María Perez ◽  
Martin Garzon ◽  
Rodrigo Daza ◽  
Geovanny Hernandez ◽  
...  
Keyword(s):  
Hepatocellular Carcinoma ◽  
Weight Loss ◽  
Clinical Presentation ◽  
Young Patients ◽  
Fibrolamellar Hepatocellular Carcinoma ◽  
Underlying Liver Disease ◽  
Hyperammonemic Encephalopathy ◽  
Cadaver Donor ◽  
Atypical Manifestation ◽  
Donor Transplant

Fibrolamellar hepatocarcinoma is an infrequent liver tumor, currently considered to be a variant different from hepatocarcinoma. The differences lie in genomic alterations, a greater prevalence of fibrolamellar hepatocarcinoma in young patients, and its lack of association with underlying liver disease. The clinical presentation is unspecific, with symptoms ranging from abdominal pain, malaise, and weight loss to atypical manifestation which include hyperammonemic encephalopathy. We present the case of a 33-year-old woman with no prior medical history who presented with a coma and a diagnosis of inoperable fibrolamellar hepatocarcinoma requiring a cadaver donor transplant. While she was on the waiting list, she received hemofiltration and ammonium benzoate treatment, with progressive improvement in her state of consciousness.

Hyperammonemic Encephalopathy: A Rare Presentation of Fibrolamellar Hepatocellular Carcinoma

2009 ◽  
Vol 338 (6) ◽  
pp. 522-524 ◽  
Author(s):  
Saurabh Sethi ◽  
Nishant Tageja ◽  
Maneesh Dave ◽  
Apurva Badheka ◽  
Sanjay Revankar ◽  
...  
Keyword(s):  
Hepatocellular Carcinoma ◽  
Fibrolamellar Hepatocellular Carcinoma ◽  
Rare Presentation ◽  
Hyperammonemic Encephalopathy

scholarly journals Hyperammonemic encephalopathy in patients with fibrolamellar hepatocellular carcinoma: a new physiopathological pathway

HPB ◽  
2019 ◽  
Vol 21 ◽  
pp. S673
Author(s):  
R. Cañada Surjan ◽  
E. Santana dos Santos ◽  
F. Ferrari Makdissi ◽  
T. Basseres ◽  
M. Autran Cesar Machado
Keyword(s):  
Hepatocellular Carcinoma ◽  
Fibrolamellar Hepatocellular Carcinoma ◽  
Hyperammonemic Encephalopathy

scholarly journals Hyperammonemic encephalopathy in a patient with fibrolamellar hepatocellular carcinoma: case report and literature review

2019 ◽  
Vol 10 (3) ◽  
pp. 582-588 ◽  
Author(s):  
Janice Cho ◽  
Joy C. Y. Chen ◽  
Jonas Paludo ◽  
Erin E. Conboy ◽  
Brendan C. Lanpher ◽  
...  
Keyword(s):  
Hepatocellular Carcinoma ◽  
Case Report ◽  
Literature Review ◽  
Fibrolamellar Hepatocellular Carcinoma ◽  
Hyperammonemic Encephalopathy

Hyperammonemic Encephalopathy Exacerbated by Parenteral Nutrition in Fibrolamellar Hepatocellular Carcinoma

2020 ◽  
Vol 133 (12) ◽  
pp. e745-e746
Author(s):  
Pir Ahmad Shah ◽  
Janice Cho ◽  
Hasan Ahmad Hasan Albitar ◽  
Gregory Pajot ◽  
Mengfei Liu ◽  
...  
Keyword(s):  
Hepatocellular Carcinoma ◽  
Parenteral Nutrition ◽  
Fibrolamellar Hepatocellular Carcinoma ◽  
Hyperammonemic Encephalopathy

568: Treatment of Hyperammonemic Encephalopathy in Fibrolamellar Hepatocellular Carcinoma

2020 ◽  
Vol 49 (1) ◽  
pp. 277-277
Author(s):  
Brian Dee ◽  
Justine Wang ◽  
Lauren Goldberg ◽  
Brandon Sterling ◽  
S. Egbert Pravinkumar
Keyword(s):  
Hepatocellular Carcinoma ◽  
Fibrolamellar Hepatocellular Carcinoma ◽  
Hyperammonemic Encephalopathy

scholarly journals Molecular Basis of Hyperammonemic Encephalopathy in Fibrolamellar Hepatocellular Carcinoma

2021 ◽  
Author(s):  
Rodrigo Cañada Trofo Surjan ◽  
Thais Martins de Lima ◽  
Elizabeth Santana dos Santos ◽  
Sergio do Prado Silveira ◽  
Marcel Cerqueira Cesar Machado ◽  
...  
Keyword(s):  
Hepatocellular Carcinoma ◽  
Ornithine Decarboxylase ◽  
Aurora Kinase ◽  
Chromosome 19 ◽  
Fibrolamellar Hepatocellular Carcinoma ◽  
Adequate Treatment ◽  
Aurora Kinase A ◽  
Hyperammonemic Encephalopathy ◽  
Fibrolamellar Carcinoma ◽  
Kinase A

Abstract Purpose hyperammonemic encephalopathy is a potentially fatal condition associated with fibrolamellar hepatocellular carcinoma. The mechanism involved in hyperammonemia in patients with fibrolamellar carcinoma was unclear until a possible physiopathological pathway was recently proposed. An ornithine transcarboxylase dysfunction was suggested as a result of increased ornithine decarboxylase activity induced by c-Myc overexpression. This c-Myc overexpression resulted from Aurora Kinase A overexpression derived from the activity of a chimeric kinase that is the final transcript of a deletion in chromosome 19, common to all fibrolamellar carcinomas. Methods we performed the analysis of the expression of all enzymes involved and tested for the mutation in chromosome 19 in fresh frozen samples of fibrolamellar hepatocellular carcinoma, non-tumor liver and hepatic adenomatosis. Results specific DNAJB-PRKACA fusion protein that results from the recurrent mutation on chromosome 19 common to all fibrolamellar carcinoma was detected only in the fibrolamellar carcinoma sample. Fibrolamellar carcinoma and adenomiomatosis samples presented increased expression of Aurora Kinase A, c-MYC and ornithine decarboxylase when compared to normal liver, while ornithine transcarbamylase was decreased. Conclusion The proposed physiopathological pathway is correct and that overexpression of c-Myc may also be responsible of hyperammonemia in patients with other types of rapidly growing hepatomas. This gives further evidence to apply new and adequate treatment to this severe complication.

Sign in / Sign up

Export Citation Format

Share Document