Emergency Department and ICU Clinical Pathway
for Acute Hyperammonemia in Neonates
  • Neonates with hyperammonemia are at risk for severe neurologic impairment.
  • Rapid diagnosis and aggressive treatment by a coordinated multidisciplinary team are critical.
  • Priority management goals:
  • Obtain adequate vascular access
  • Initiate metabolic therapy
  • Provide Continuous Renal
    • Replacement Therapy
Admit to PICU
  • Ready Giraffe® warmer bed, temperature probes available
  • Consider intubation
    • Use cuffed ETT for tight control of CO2
    • Facilitate central catheter placement
Lower Risk
Ammonia level < 500 umol/L
Mental status WNL
Higher Risk
  • Ammonia level ≥ 500 umol/L or
  • Mental status changes and/or
  • Tachypnea, respiratory alkalosis and/or
  • Evidence of cerebral edema, seizures
Continued Medical Management
Lab Monitoring
  • Lower risk: Ammonia level every 3-4 hrs
  • Higher risk: Ammonia level every 1-2 hrs initially (use STAT order priority)
  • Lab frequency may be spaced once ammonia has decreased and child has stabilized
Indications for CRRT
  • Consider if ammonia level not improving after 1-2 hours of medical management or
  • Persistent encephalopathy
  • If there are no relative contraindications (infant size, presence of coagulopathy)
Glucose Management
  • For persistent hyperglycemia > 200 mg/dL, do not decrease GIR
    • Start insulin infusion 0.01 units/kg/hr
    • Titrate by 0.01 unit/kg/hr to maintain euglycemia (goal: 100-200 mg/dL)
Continued Care
  • Following stabilization with improving labs and physical exam, care plan is coordinated by Critical Care Team with Metabolism and Nephrology consultation
Posted: August 2020
Revised: March 2023
Authors: K. McKenna, MD; B. Laskin, MD; J. Fitzgerald, MD; S. Vatsky, DO; A. Bustin, PharmD