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Sample methylmalonic acidemia emergency department form

Sample methylmalonic acidemia emergency department form
Guidelines for emergency department management
Diagnosis: Methylmalonic acidemia (MMA)
Name:
DOB:
MRN:
Patient is followed at ______________________ for the diagnosis of methylmalonic acidemia. Patient is at risk for life-threatening complications during times of illness.
We recommend patient is triaged IMMEDIATELY upon arrival to the emergency department
  1. Common acute complications:
    • Metabolic acidosis, ketoacidosis, hyperammonemia, hypoglycemia, seizures, encephalopathy, coma, pancytopenia, pancreatitis
  2. Initial management:
    • Place peripheral IV and initiate IV fluids
    • If signs of dehydration: IV bolus of 10 to 20 cc/kg NS
    • Hypoglycemia: IV bolus of 10 to 25% glucose
      • Neonates: 2 cc/kg, 10% solution
      • Children: 5 to 10 cc/kg, 10% solution
    • Acidosis: Correct acidosis based on ABG/VBG
    • Continuous IV fluids*: D10% with 1/4 NS, 1/2 NS, NS; Rate: 1.5x maintenance
  3. Laboratory studies:
    • Comprehensive metabolic panel
    • Blood ammonia (STAT, free flowing sample)
    • Dextrose stick
    • Dip all urine voids for ketones
    • Plasma amino acid quantitation
    • Acylcarnitine profile
    • Plasma methylmalonic acid
    • Urine organic acid quantitation
    • Urine analysis with microscopy
    • If indicated:
      • ABG/VBG if patient is ill appearing or has respiratory decompensation
      • Blood culture if patient is ill appearing or has a central line in place
      • Complete blood count with differential if patient has signs of infection or abnormal bleeding
      • Lipase and amylase if patient has vomiting or abdominal pain
  4. Contraindicated medications:
    • Systemic glucocorticoids (unless otherwise specified by metabolism staff)
  5. Discuss further management with a metabolic disease specialist at the hospital or contact the patient's specialist
DOB: date of birth; MRN: medical record number; IV: intravenous; NS: normal saline (isotonic saline, 0.9% solution of sodium chloride); ABG: arterial blood gas; VBG: venous blood gas; D10%: 10% dextrose.
* Monitor for signs of increased intracranial pressure.
Courtesy of Sarah Sheppard, MD, PhD and Can Ficicioglu, MD, PhD.
Graphic 120354 Version 1.0