Episode 11b: Acute Illness and Known Metabolic Disease with Austin Larson

On this episode, host Jason Woods speaks with Dr. Austin Larson, a specialist in pediatric inherited metabolic disorders (and also someone way smarter than I), about what do do with the crashing neonate if there are concerns for a metabolic disorder. We review the diagnostic approach, emergent treatment, and what resources are available to read/consult in this situation.

Important points

  1. Most children with known inherited metabolic disorder will have a letter with them, from their metabolic doc, about what to do if they show up to the with a new illness. If they do not, then the New England Consortium website is a great resource.
  2. Most protocols recommend starting D10 fluids at 1.5 x maintenance is generally the recommendation, unless there is a reason why glucose/fluids will otherwise harm the patient.
  3. Be careful with patients who are on a ketogenic diet as treatment for their seizures, as giving them glucose can cause refractory status epilepticus. In those patients, the preferred calorie support would be lipids.

Point of Care Resources

  1. New England Consortium for treatment protocols
  2. Vademecum Metabolicum handbook in book form
  3. App version of Vademecum Metabolicum – eVM

Guests

Austin Larson, MD – Assistant Professor of Pediatrics and Metabolic and Clinical Genetics, University of Colorado School of Medicine and Children’s Hospital Colorado

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