Singh listed a number of recommendations for managing a patient with a urea cycle disorder (UCD) on a "sick day". Early and appropriate mangement of an illness, injury or other catabolic state can avoid decompensation and the need for hospitalization. The author is from Emory University.


Sickness increases catabolism, and catabolic breakdown of protein can result in hyperammonemia in these patients.



(1) Provide instructions for handling of a sick day, including ways to reduce protein intake while providing for total energy needs.

(2) Have protein-free food available.

(3) Increase oral phenylbutyrate intake or other nitrogen scavenging drugs (sodium phenylacetate, etc) being careful not to exceed the maximum age-appropriate dose.

(4) Have an antiemetic drug available if needed.

(5) Instruct the caregiver on how to insert a nasogastric tube.

(6) Monitor the urine for urine ketones in order to detect catabolism.

(7) Provide instructions for when to bring the patient in for medical evaluation.


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