Management The infant should be stabilized and if there are signs of respiratory distress, should be supported with assisted ventilation Patients with signs of global asphyxial injury should be supported with fluid boluses or inotropes if there are signs of poor cardiac output For select patients presenting with encephalopathy since birth, the consideration of therapeutic hypothermia should be discussed with a neonatologist A history that is concerning for trauma or asphyxia, or infants with evidence of trauma or increased intracranial pressure, should undergo emergent head imaging (noncontrast head CT) If there is evidence of intracranial hemorrhage, cerebral edema, and/or herniation, pediatric neurosurgery should be consulted In addition, a complete blood count and coagulation studies should be sent and abnormalities should be corrected aggressively with transfusion of blood products to stabilize any hemorrhage Any metabolic derangements should be corrected—particularly hypoglycemia, with the rapid infusion of parenteral dextrose bolus and then an ongoing infusion Tight control of serum sodium should be achieved to decrease the effects of cerebral edema Broad-spectrum antibiotics should be administered once the cultures have been obtained Acute hyperbilirubinemia with encephalopathy is treated with hydration and exchange transfusion Hyperammonemia is treated with scavenger drugs and/or dialysis in conjunction with a pediatric nephrologist Once stabilized, many IEM can be managed by diet in conjunction with a metabolic geneticist Degenerative diseases such as Tay–Sachs, Menkes, Neiman–Pick, Guacher, Crabber, and peroxisomal and mitochondrial disorders have no effective treatments other than supportive care