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Table 1 Mechanism leading to nonhepatic hyperammonemia: increased production and decreased metabolism

From: Malnutrition-related hyperammonemic encephalopathy presenting with burst suppression: a case report

Overproduction Undermetabolism
Excessive protein supply (enteral or parenteral) Portosystemic shunting
Muscles catabolism: cachexia, starvation, seizure Medications interfering with urea cycle: valproic acid, carbamazepine, salicylate, glycine, ribavirin
Urease producing bacteria urinary tract infections: Proteus mirabilis, Klebsiella species, Escherichia coli, Morganella morganii, Providencia rettgeri, diphtheroids Inborn error of metabolism: inherited defects of the urea cycle, amino acid transporters, fatty acid oxidation, organic acid disorders
Hematological malignancies: multiple myeloma, leukemia