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Neonatal hyperammonemia is a feature of many different inborn errors of metabolism that may be individually rare but have about a 1:5000 cumulative incidence.1,2 It can also be a feature of fulminant liver failure of any cause and of structural anomalies leading to portosystemic shunting. Neonatal hyperammonemia represents a true metabolic emergency as rapid identification and intervention are critical to a positive neurologic outcome. It is essential that neonatal centers have a protocol and plan in place to address these patients. A representative protocol is provided in this chapter (Table 106-1) and details are provided.

Table 106-1Timeline for Management of Hyperammonemia

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