Describe the acute nutritional interventions used for urea cycle disorders
Initial treatment includes administration of intravenous fluids because many infants are dehydrated at presentation due to anorexia and poor oral intake. Over-hydration 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 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, total parenteral nutrition (TPN) should be started. When protein is reintroduced into the diet, a standard TPN solution with a low percentage of amino acids may be used if TPN is only needed for a short duration. If enteral feedings are not resumed quickly, then the use of a special TPN solution rich in the essential branched-chain amino acids should be considered.
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Macronutrients provide __________.
Fill in the blank(s) with the appropriate word(s).