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 |