1. Indications And Usage Carbaglu Is A Carbamoyl Phosphate Synthetase 1 (Cps 1) Activator Indicated In Pediatric And Adult Patients As: Adjunctive Therapy For The Treatment Of Acute Hyperammonemia Due To The Deficiency Of The Hepatic Enzyme N-Acetylglutamate Synthase (Nags). ( 1.1 ) Maintenance Therapy For The Treatment Of Chronic Hyperammonemia Due To The Deficiency Of The Hepatic Enzyme N-Acetylglutamate Synthase (Nags). ( 1.2 ) 1.1 Acute Hyperammonemia In Patients With Nags Deficiency Carbaglu Is Indicated As An Adjunctive Therapy In Pediatric And Adult Patients For The Treatment Of Acute Hyperammonemia Due To The Deficiency Of The Hepatic Enzyme N-Acetylglutamate Synthase (Nags). During Acute Hyperammonemic Episodes, Concomitant Administration Of Carbaglu With Other Ammonia Lowering Therapies, Such As Alternate Pathway Medications, Hemodialysis, And Dietary Protein Restriction, Is Recommended. 1.2 Chronic Hyperammonemia In Patients With Nags Deficiency Carbaglu Is Indicated As Maintenance Therapy In Pediatric And Adult Patients For The Treatment Of Chronic Hyperammonemia Due To Deficiency Of The Hepatic Enzyme N-Acetylglutamate Synthase (Nags). During Maintenance Therapy, The Concomitant Use Of Other Ammonia Lowering Therapies And Protein Restriction May Be Needed Based On Plasma Ammonia Levels.
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