Abstract: The present disclosure provides methods for preventing hyperammonemia crises (HACs) in subjects with a urea cycle disorder (UCD) and for optimizing and adjusting nitrogen scavenging drug dosage for subjects with UCDs to prevent HACs.

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, systems, and instruments for the determination by a physician or medical professional whether a subject is experiencing or has recently experienced an overt HE episode and for grading overt HE episodes as well as methods, systems, instruments and tools for screening for overt HE episodes by a non-medical professional such as a caregiver. Also provided are methods of treating HE episodes and methods of monitoring HE episode treatment that incorporate these methods, systems, instruments, and tools.

Abstract: The present disclosure provides methods for treating hepatic encephalopathy (HE) and for optimizing and adjusting nitrogen scavenging drug dosage for subjects with HE.

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 treating hepatic encephalopathy (HE) and for optimizing and adjusting nitrogen scavenging drug dosage for subjects with HE.

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: 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 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: The present disclosure provides methods, systems, and instruments for the determination by a physician or medical professional whether a subject is experiencing or has recently experienced an overt HE episode and for grading overt HE episodes as well as methods, systems, instruments and tools for screening for overt HE episodes by a non-medical professional such as a caregiver. Also provided are methods of treating HE episodes and methods of monitoring HE episode treatment that incorporate these methods, systems, instruments, and tools.

Abstract: The invention provides a method for determining a dose and schedule and making dose adjustments of PBA prodrugs used to treat nitrogen retention states, or ammonia accumulation disorders, by measuring urinary excretion of phenylacetylglutamine and/or total urinary nitrogen. The invention provides methods to select an appropriate dosage of a PBA prodrug based on the patient's dietary protein intake, or based on previous treatments administered to the patient. The methods are applicable to selecting or modifying a dosing regimen for a subject receiving an orally administered ammonia scavenging drug.

Abstract: The present disclosure provides methods for treating hepatic encephalopathy (HE) and for optimizing and adjusting nitrogen scavenging drug dosage for subjects with HE.

Abstract: The invention provides a method for determining a dose and schedule and making dose adjustments of PBA prodrugs used to treat nitrogen retention states, or ammonia accumulation disorders, by measuring urinary excretion of phenylacetylglutamine and/or total urinary nitrogen. The invention provides methods to select an appropriate dosage of a PBA prodrug based on the patient's dietary protein intake, or based on previous treatments administered to the patient. The methods are applicable to selecting or modifying a dosing regimen for a subject receiving an orally administered ammonia scavenging drug.