TY - JOUR
T1 - Hyperammonemic encephalopathy in a patient with fibrolamellar hepatocellular carcinoma
T2 - Case report and literature review
AU - Cho, Janice
AU - Chen, Joy C.Y.
AU - Paludo, Jonas
AU - Conboy, Erin E.
AU - Lanpher, Brendan C.
AU - Alberts, Steven R.
AU - Halfdanarson, Thorvardur R.
N1 - Publisher Copyright:
© Journal of Gastrointestinal Oncology. All rights reserved.
PY - 2019
Y1 - 2019
N2 - Fibrolamellar hepatocellular carcinoma (fHCC) is a rare primary liver cancer that affects young adults with no prior liver disease. fHCC-associated hyperammonemic encephalopathy (HAE) is an uncommon and life-threatening complication. Hyperammonemia has been reported in both typical and fHCC as a result of intrahepatic shunting, side effect from immunotherapy or chemotherapy, or as a paraneoplastic phenomenon. We present a case of a 32-year-old woman with recurrent metastatic fHCC who developed HAE in the setting of steroid administration. Her hyperammonemia was exacerbated by steroid-induced protein catabolism. She was treated with ammonia scavenging medications, a low protein diet, and was placed on chronic ammonia scavenger therapy while undergoing chemotherapy. In this case report, we discuss the proposed mechanisms of HAE, and we review the literature regarding clinical presentation and treatment.
AB - Fibrolamellar hepatocellular carcinoma (fHCC) is a rare primary liver cancer that affects young adults with no prior liver disease. fHCC-associated hyperammonemic encephalopathy (HAE) is an uncommon and life-threatening complication. Hyperammonemia has been reported in both typical and fHCC as a result of intrahepatic shunting, side effect from immunotherapy or chemotherapy, or as a paraneoplastic phenomenon. We present a case of a 32-year-old woman with recurrent metastatic fHCC who developed HAE in the setting of steroid administration. Her hyperammonemia was exacerbated by steroid-induced protein catabolism. She was treated with ammonia scavenging medications, a low protein diet, and was placed on chronic ammonia scavenger therapy while undergoing chemotherapy. In this case report, we discuss the proposed mechanisms of HAE, and we review the literature regarding clinical presentation and treatment.
KW - Fibrolamellar hepatocellular carcinoma (fHCC)
KW - Hyperammonemia
KW - Hyperammonemic encephalopathy (HAE)
KW - Ornithine transcarbamylase (OTC) deficiency
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U2 - 10.21037/jgo.2019.01.28
DO - 10.21037/jgo.2019.01.28
M3 - Review article
AN - SCOPUS:85066936935
SN - 2078-6891
VL - 10
SP - 582
EP - 588
JO - Journal of Gastrointestinal Oncology
JF - Journal of Gastrointestinal Oncology
IS - 3
ER -