Non-cirrhotic Hyperammonemia - Differential Diagnosis Framework
Hematologic & Vascular:
• Multiple myeloma & Acute leukemia (plasma cells —Y t amino acid metabolism)
• Congenital portosystemic shunt (Abernethy Malformation)
Infections:
• Urease-producing bacteria: Proteus mirabilis, Escherichia coli, Klebsiella
• Results in severe hyper-NH3 if elderly + urinary retention
Unmasked Urea Cycle Defects
• OTC deficiency (XLR)
• Deficiency unmasked by:
- Seizure, Starvation (↑ muscle catabolism)
- TPN, GI bleeding (↑ nitrogen loading)
- Infections (direct ↑)
Drugs:
• Valproic acid
• Carbamazepine
• Salicylates
• Sulfadiazine
By Andrew Sanchez M.D. @ASanchez_PS
#Hyperammonemia #ammonia #Differential #Diagnosis #Pathophysiology
