Describe the Acute Nutritional Interventions Used for Urea Cycle Disorders

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The Answer is Initial treatment includes administration of intravenous fluids because many infants are dehydrated at presentation due to anorexia and poor oral intake. Overhydration should be avoided because most patients with urea cycle defects have some degree of cerebral swelling. Caloric supplementation should be maximized to reverse catabolism and nitrogen turnover. In addition to glucose, Intralipid administration can provide the much-needed kilocalories in a smaller volume. Complete protein restriction is recommended only for a 24- to 48-hour period in order to avoid depletion of essential amino acids, which would result in further protein catabolism and nitrogen release. If enteral feedings cannot be tolerated, parenteral nutrition (PN) should be started. When protein is reintroduced into the diet, a standard PN solution with a low percentage of amino acids may be used if PN is only needed for a short duration. If enteral feedings are not resumed quickly, then the use of a special PN solution rich in the essential branched-chain amino acids should be considered.

Describe the Acute Nutritional Interventions Used for Urea Cycle Disorders

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Describe the Acute Nutritional Interventions Used for Urea Cycle Disorders

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