Hyperammonemia, Neonatal — Guidance for Referred Children — Clinical Pathway: Emergency Department and ICU

Neonatal Hyperammonemia Clinical Pathway — PICU and Emergency Department

Guidance for Referred Children

Transport Call for Child at Referring Institution

Initial Communication
  • Medical Command: PICU for PICU admission due to high risk for need for CRRT
  • Metabolism: For initial management
  • Nephrology: If CRRT is likely (Metabolism to call Nephrology)
  • Interventional Radiology
    • For potential central access and possible dialysis catheter placement
Triage Information
  • Child presentation/pertinent history
  • Physical Exam
    • Neurological status: AMS, encephalopathy
    • Respiratory status: Respiratory support, tachypnea, work of breathing
  • Labs
    • Ammonia level
    • Blood gas
    • Glucose level
    • Amino acids
  • Current vascular access
  • Infusions and medications
Time Sensitive Management
  • Assure IV access, central access if able
  • Respiratory
    • Consider for intubation with cuffed ETT if child is higher risk
  • NPO
    • Stop all enteral intake as soon as possible
  • GIR
    • Start high dextrose-containing IV fluids as soon as possible
    • Preferably D10W or D12.5W IV at 120 cc/kg/day to start
Preparations for Transport and Receipt of Child
  • Giraffe warmer bed is preferred, but availability should not delay admission to PICU
  • Securing appropriate bed should not delay admission to PICU