Emergency Department and ICU Clinical Pathway
for Acute Hyperammonemia in Neonates
for Acute Hyperammonemia in Neonates
- Initial Evaluation
- Review referring hospital records, labs, imaging
- Resuscitation
- Airway support as indicated
- Establish IV access
- Initial Laboratory Studies
- Initial Fluid Management
- Stop Protein intake immediately
-
Emergent head CT
- Preferred modality for assessing increased ICP
- Consider Fast MRI depending on child stability
- Consults
- Metabolism
- Interventional Radiology
- Central and hemodialysis access
- Nephrology
- Neonatology
- 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
and/or
Mental status WNL
Ammonia level < 500 umol/L
and/or
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
- GIR
- Glucose infusion rate goal: 10-15 mg/kg/min
- Initiate with Metabolism Guidance
- Avoid fever
- GIR
- Glucose infusion rate goal: 15-20 mg/kg/min
- Initiate
- Prepare for CRRT (Continuous Renal Replacement Therapy) using only a Prisma CRRT machine (ammonia clearance may be inadequate with Aquadex™ or Carpediem™)
- Access
- Obtain central access and HD Catheter
- Initiate CRRT
- Indications include encephalopathy or ammonia > 1,000 or poor response to initial therapies
- Avoid fever
- Consider Targeted Temperature Management
Lab Monitoring |
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Indications for CRRT |
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Glucose Management |
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Continued Care |
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Posted: August 2020
Revised: March 2023
Authors: K. McKenna, MD; B. Laskin, MD; J. Fitzgerald, MD; S. Vatsky, DO; A. Bustin, PharmD
Revised: March 2023
Authors: K. McKenna, MD; B. Laskin, MD; J. Fitzgerald, MD; S. Vatsky, DO; A. Bustin, PharmD
Evidence
- High-dose Continuous Renal Replacement Therapy for Neonatal Hyperammonemia
- Hemodialysis Catheters in Infants: A Retrospective Single-Center Cohort Study
- Alternative Pathway Therapy for Urea Cycle Disorders: Twenty Years Later
- Current Strategies for the Management of Neonatal Urea Cycle Disorders
- Feasibility of Adjunct Therapeutic Hypothermia Treatment for Hyperammonemia and Encephalopathy Due to Urea Cycle Disorders and Organic Acidemias