Treatment of urea cycle disorders in neonates and infants

(canceled)

A method of treating a urea cycle disorder (UCD) in a patient under 2 years of age comprising

• administering glycerol phenylbutyrate to said patient at an initial daily dose which is administered at an initial frequency of administration;
• and
• if after said administration, said patient exhibits neurological symptoms, normal ammonia levels, and a ratio of plasma PAA to plasma PAGN of >2.5 or a level of plasma PAA of >500 μg/mL, then administering said glycerol phenylbutyrate to said patient at a reduced daily dose while maintaining said initial frequency of administration.

A method of treating a urea cycle disorder (UCD) in a patient under 2 years of age comprising

• administering glycerol phenylbutyrate to said patient at an initial daily dose which is administered at an initial frequency of administration;
• and
• if after said administration said patient exhibits neurological symptoms, normal ammonia levels, and a ratio of plasma PAA to plasma PAGN of >2.5 or a level of plasma PAA of >500 μg/mL, then administering said glycerol phenylbutyrate to said patient at a lower dose or increased frequency of administration while maintaining said daily dose.

A method of treating a urea cycle disorder (UCD) in a patient under 2 years of age comprising

• administering glycerol phenylbutyrate to said patient at an initial daily dose which is administered at an initial frequency of administration;
• and
• if after said administration said patient exhibits neurological symptoms, normal ammonia levels, and a ratio of plasma PAA to plasma PAGN of <2.5, then said glycerol phenylbutyrate to said patient should continue to be administered at the same dose and frequency and the patient evaluated for other causes of his or her neurological symptoms.

(canceled)

(canceled)