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Plain English summary of "A Proposed Physiopathological Pathway to Hyperammonemic Encephalopathy in a Non-Cirrhotic Patient with Fibrolamellar Hepatocellular Carcinoma without Ornithine Transcarbamylase (OTC) Mutation" 

Published March 2017 in The American Journal of Case Reports

High levels of ammonia have been a serious complication of advanced fibrolamellar.  Clinicians have found that treatments such as lactulose, which work well for other liver cancers, do not work well for fibrolamellar. In this paper, the authors propose and test a novel treatment on one patient which proves successful in treating his severe hyperammonia.

The authors noted two earlier findings: that a protein called Aurora Kinase A is increased in fibrolamellar; and that Aurora Kinase A is expected to increase the expression of a protein called ornithine decarboxylase, which can produce an imbalance in the urea cycle and thus, high levels of ammonia.  The authors then propose a novel treatment of sodium benzoate (3 g) and arginine (3 g) administered every four hours via nasogastric tube.  The authors demonstrate the successful use of this treatment on one 31-year-old fibrolamellar patient. Sodium benzoate is a food preservative found in salad dressings, carbonated drinks, jams and fruit juices, pickles, and condiments. It has been successfully used before to treat urea disorders but never, apparently, fibrolamellar.  

This research offers the hope of alleviating the effects of hyperammonemia in fibrolamellar.  

--Dr. Sandy Simon, Fibrolamellar Registry