Abstract: The present disclosure provides methods for evaluating daily ammonia exposure based on a single fasting ammonia blood level measurement, as well as methods that utilize this technique to adjust the dosage of a nitrogen scavenging drug, determine whether to administer a nitrogen scavenging drug, and treat nitrogen retention disorders.

Abstract: Provided are methods of administering glycerol phenylbutyrate to a patient in need thereof, wherein said patient is also being treated with a CYP3A4 substrate having a narrow therapeutic index, midazolam or a pharmaceutically acceptable salt thereof, or celecoxib.

Abstract: The present disclosure provides novel methods for determining an effective dosage of a PAA prodrug and for treating a UCD that incorporate body surface area and urinary PAGN concentration. The disclosure further provides novel methods for assessing compliance with PAA prodrug administration that incorporate urinary PAGN concentration, and the subject's current dosing regimen, BSA, or age. The disclosure further provides novel methods of treating a UCD in a subject in need thereof that incorporate urinary PAGN concentration, and the subject's current dosing regimen, BSA, and/or age.

Abstract: Nitrogen scavenging drugs such as glycerol phenylbutyrate can be administered safely to infants and toddlers with urea cycle disorders by adjusting the dosage based on one or more biomarkers selected from the group consisting of urinary-PAGN and plasma PAA:PAGN ratio.

Abstract: Nitrogen scavenging drugs such as glycerol phenylbutyrate can be administered safely to infants and toddlers with urea cycle disorders by adjusting the dosage based on one or more biomarkers selected from the group consisting of urinary-PAGN and plasma PAA:PAGN ratio.

Abstract: The present disclosure provides methods for evaluating daily ammonia exposure based on a single fasting ammonia blood level measurement, as well as methods that utilize this technique to adjust the dosage of a nitrogen scavenging drug, determine whether to administer a nitrogen scavenging drug, and treat nitrogen retention disorders.

Abstract: The present disclosure provides methods for evaluating daily ammonia exposure based on a single fasting ammonia blood level measurement, as well as methods that utilize this technique to adjust the dosage of a nitrogen scavenging drug, determine whether to administer a nitrogen scavenging drug, and treat nitrogen retention disorders.

Abstract: Nitrogen scavenging drugs such as glycerol phenylbutyrate can be administered safely to infants and toddlers with urea cycle disorders by adjusting the dosage based on one or more biomarkers selected from the group consisting of urinary-PAGN and plasma PAA:PAGN ratio.

Abstract: Provided are methods of administering glycerol phenylbutyrate to a patient in need thereof, wherein said patient is also being treated with a CYP3A4 substrate having a narrow therapeutic index, midazolam or a pharmaceutically acceptable salt thereof, or celecoxib.

Abstract: Provided are methods of administering glycerol phenylbutyrate to a patient in need thereof, wherein said patient is also being treated with a CYP3A4 substrate having a narrow therapeutic index, midazolam or a pharmaceutically acceptable salt thereof, or celecoxib.

Abstract: The present disclosure provides methods for evaluating daily ammonia exposure based on a single fasting ammonia blood level measurement, as well as methods that utilize this technique to adjust the dosage of a nitrogen scavenging drug, determine whether to administer a nitrogen scavenging drug, and treat nitrogen retention disorders.

Abstract: The present disclosure provides methods for evaluating daily ammonia exposure based on a single fasting ammonia blood level measurement, as well as methods that utilize this technique to adjust the dosage of a nitrogen scavenging drug, determine whether to administer a nitrogen scavenging drug, and treat nitrogen retention disorders.

Abstract: The present disclosure provides methods for evaluating daily ammonia exposure based on a single fasting ammonia blood level measurement, as well as methods that utilize this technique to adjust the dosage of a nitrogen scavenging drug, determine whether to administer a nitrogen scavenging drug, and treat nitrogen retention disorders.

Abstract: The present disclosure provides methods for evaluating daily ammonia exposure based on a single fasting ammonia blood level measurement, as well as methods that utilize this technique to adjust the dosage of a nitrogen scavenging drug, determine whether to administer a nitrogen scavenging drug, and treat nitrogen retention disorders.

Abstract: The present disclosure provides methods for evaluating daily ammonia exposure based on a single fasting ammonia blood level measurement, as well as methods that utilize this technique to adjust the dosage of a nitrogen scavenging drug, determine whether to administer a nitrogen scavenging drug, and treat nitrogen retention disorders.

Abstract: The present disclosure provides methods for evaluating daily ammonia exposure based on a single fasting ammonia blood level measurement, as well as methods that utilize this technique to adjust the dosage of a nitrogen scavenging drug, determine whether to administer a nitrogen scavenging drug, and treat nitrogen retention disorders.

Abstract: The present disclosure provides methods for adjusting the dosage of PAA prodrugs (e.g., HPN-100, PBA) based on measurement of PAA and PAGN in plasma and calculating the PAA:PAGN ratio so as to determine whether PAA to PAGN conversion is saturated.

Abstract: The present disclosure provides methods for evaluating daily ammonia exposure based on a single fasting ammonia blood level measurement, as well as methods that utilize this technique to adjust the dosage of a nitrogen scavenging drug, determine whether to administer a nitrogen scavenging drug, and treat nitrogen retention disorders.

Abstract: The present disclosure provides methods for evaluating daily ammonia exposure based on a single fasting ammonia blood level measurement, as well as methods that utilize this technique to adjust the dosage of a nitrogen scavenging drug, determine whether to administer a nitrogen scavenging drug, and treat nitrogen retention disorders.

Abstract: The present disclosure provides methods for evaluating daily ammonia exposure based on a single fasting ammonia blood level measurement, as well as methods that utilize this technique to adjust the dosage of a nitrogen scavenging drug, determine whether to administer a nitrogen scavenging drug, and treat nitrogen retention disorders.