Summary: <a href="https://i2.wp.com/littlebigmed.com/wp-content/uploads/2017/08/cropped-Little-Big-Med-Logo-2.png"></a>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.<br> Important points<br> <br> * If an ill neonate presents and there is any concern for inherited metabolic disorder, 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.<br> * If there are concerns for inherited metabolic disorder, in addition to glucose, checking ketones (urine or serum, though serum preferred), ammonia, and lactate can be very helpful.<br> * If the ammonia level is >200 micromols/L and rising, dialysis will likely be needed and you need to start making plans for this.<br> * Neonates generally can not generate a significant ketosis, even if fasting or ill, without an inherited metabolic disorder.<br> * Other labs to consider obtaining in an ill child with hypoglycemia or concern for metabolic disorder<br> <br> * Cortisol<br> * Serum Amino Acids<br> * Urine Organic Acids<br> * Serum ketones<br> * Free fatty acids<br> * Growth Hormone<br> * Insulin<br> * Acylcarnitine profile<br> <br> <br> <br> Point of Care Resources<br> <br> * <a href="http://newenglandconsortium.org">New England Consortium</a> for treatment protocols<br> * <a href="https://books.google.com/books?id=OHo69LGhCYIC&pg=PA25&lpg=PA25&dq=vademecum+medicum+manual+of+metabolic+pediatrics&source=bl&ots=ayMQHBcxIz&sig=ipvub96ZJO-BIIbfaHoAFm2Na10&hl=en&sa=X&ved=0ahUKEwikkOuJsrHbAhVLxlQKHT7iCJQQ6AEIOTAC#v=onepage&q&f=false">Vademecum Metabolicum handbook in book form</a><br> * <a href="https://itunes.apple.com/us/app/evm-vademecum-metabolicum/id1123172322?mt=8">App version of Vademecum Metabolicum – eVM</a><br> <br> Guests<br> Austin Larson, MD – Assistant Professor of Pediatrics and Metabolic and Clinical Genetics, University of Colorado School of Medicine and Children’s Hospital Colorado<br>
