Hyperammonemia, Neonatal — Patient Cohort — Clinical Pathway: Emergency Department and ICU

Neonatal Hyperammonemia Clinical Pathway — PICU and Emergency Department

Patient Cohort

This pathway guides the care for infants ≤ 30 days of age referred or presenting to CHOP for acute care for any abnormal or elevated ammonia level.

Differential Diagnoses

  • Urea cycle defects
  • Organic acidemias
  • Fatty acid oxidation defects
  • Mitochondrial defects
  • Infection (sepsis, disseminated HSV, meningitis)
  • Perinatal asphyxia with hepatic involvement
  • Transient hyperammonemia of the newborn
  • Acute or chronic hepatic insufficiency
  • Drug-related side effect or toxicity

Exclusion Criteria

  • Children with liver failure:
    • Nitrogen scavengers are not effective in liver disease because they are processed by the liver.
    • These drugs can become toxic if the liver is completely nonfunctional.
    • Lactulose and other strategies may be of benefit in children with severe liver failure.
  • Infants older than 30 days of age.