Hyperammonemia, Neonatal — Initial Laboratory Studies and Fluid Management — Clinical Pathway: Emergency Department and ICU

Neonatal Hyperammonemia Clinical Pathway — PICU and Emergency Department

Initial Laboratory Studies and Fluid Management

Initial Laboratory Studies

Labs
Listed in order
of priority
  • Plasma:
    • Ammonia level (free flowing, on ice)
    • Gas with lactate and ionized calcium
    • Glucose level
    • Amino acids
      • Draw before arginine started
    • CBC with platelet count
    • Coagulation studies
    • Electrolytes/LFTs (CMP with Phosphorus)
    • Acylcarnitine panel
  • Urine:
    • Ketones
    • Organic acids
    • Orotic acid
    • Amino acids
  • Anion gap measurement
  • Lactic acid
  • Carnitine
  • Newborn screen
  • DNA extraction

Initial Fluid Management

  • IVF recommendations are similar between lower and higher-risk children
  • The GIR differs (Glucose Infusion Rate)
    • Glucose provokes a hyperinsulinemic response that inhibits protein and fat catabolism and decreases toxic intermediates.
  • Electrolyte supplementation is started at the discretion of the clinical care team based on age, clinical status
Fluid Timing of Initiation Vascular Access
  • Dextrose 10% in Water
  • Rate:
    • 6 mL/kg or 1.5x maintenance
  • Any dextrose-containing fluids can be started emergently and increased to the appropriate dextrose concentration as available
  • ED, Transport, OSH
    • D10W is generally readily available
    • Start as soon as possible
  • ED and Transport (grey bags for ground only) have D10W in stock; PICU does not
  • On arrival to PICU, continue D10 fluids from ED or transport until child transitions to fluids below
 Peripheral or central
  • Dextrose 12.5% in Water
  • Electrolyte addition, as indicated
  • Based on clinical status, at discretion of care team
 Peripheral or central
  • Dextrose 20% in Water
  • Electrolytes as indicated
  • Once central line is established
 Central ONLY