Fulminant Hepatic Failure Caused by Amanita phalloides Toxicity

Abstract

C 64-year-old Korean woman presented 3 days after ingesting 2 bowls of cooked wild mushrooms from her backyard n Delaware. She reported having nausea, vomiting, diarrhea, nd cramping abdominal pain starting 12 hours after ingestion. -acetylcysteine (NAC) was instituted and she was transferred o our institution. Her mental status and physical examination ere within normal limits with the exception of moderate bdominal tenderness. Pertinent laboratory results included: spartate aminotransferase level of 1,111 U/L, alanine aminoransferase level of 899 U/L, alkaline phosphatase level of 103 /L, bilirubin level of 1.3 mg/dL, lactic acid level of 6.8 mg/dL, nd an international normalized ratio of 3.42. Abdominal comuted tomography showed liver enhancement with attenuation nd periportal edema (arrow) consistent with fulminant hepaitis (Figure A). Salicylate and acetaminophen levels, antimitohondrial antibody, antinuclear antibody, and viral hepatitis erologies were negative. By day 2, her aspartate aminotransferse level was 8,063 U/L and her alanine aminotransferase level as 5,578 U/L. She developed anemia, thrombocytopenia, and rogressive liver failure with an international normalized ratio f 8.52 and a bilirubin level of 27.9 mg/dL. By day 4, she was ypotensive, encephalopathic, and required intubation. She reeived successful orthotopic liver transplantation 6 days after dmission. Surgical pathology (Figure B) revealed severe zonal centriobular necrosis with focal bridging hemorrhagic necrosis (arows). A sample of the mushrooms from her backyard was rocured for analysis (Figure C). Identification of the mushooms as Amanita phalloides was made by a mycologist based on dentification of (from clockwise) the cream-colored gills, the olid stalk with a round base, overlaying white volva and oposing thin, white universal veil patch, and the yellowish-brown ap. -Amanitin was identified by mass spectrometry. Ingestion of amatoxin-containing mushrooms (in this case, A halloides), is a rare cause of acute liver failure that may require iver transplantation.1 However, the A phalloides population in orth America is increasing. Two distinct mushroom populaions now exist, one extending from California to British Coumbia and the second extending from Maryland to Maine.2 The 50% lethal dose of -amanitin is low, with as little as 0.1 g/kg of body weight being lethal in the adult. The toxin binds nd inhibits RNA polymerase II, resulting in necrosis primarily n intestinal mucosa, hepatocytes, and proximal tubules of the idney. -Amanitin is thermostable, and is not inactivated by eating or cooking.3 NAC can be an effective treatment in cases f acute A phalloides ingestion.4 In the case of our patient, who presumably ingested an exremely large amount of -amanitin, NAC did not prevent liver ailure, and liver transplantation was a necessary intervention.