A patient with a urea cycle disorder may clinically deteriorate if certain conditions occur that cause a high nitrogen load, decreased hepatic function or impact the capacity of the urea cycle.


Nutritional factors that can cause the patient to deteriorate:

(1) rapid weight loss

(2) poor nutritional intake (starvation)

(3) gastric bypass surgery

(4) malabsorption

(5) high protein diet

(6) short bowel syndrome

(7) parenteral nutrition with high nitrogen load


Systemic stress:

(1) gastrointestinal or internal bleeding

(2) significant infection (viral, bacterial, fungal) with generalized stress

(3) comorbid metabolic disorders (fatty acid oxidation defects, organic acidemia)


Trauma or surgery:

(1) major fracture

(2) major surgery or trauma

(3) postpartum state

(4) major organ transplant


Drug or toxin exposure:

(1) high dose or intravenous glucocorticoids

(2) chemotherapy, including cyclophosphamide

(3) valproic acid

(4) exposure to 5-pentanoic acid (Jamaican vomiting sickness)


Liver disease:

(1) cirrhosis with portal hypertension and shunting

(2) acute hepatitis or liver injury


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