Pediatric emergency medicine trisk 2571 2571

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.