Pegzilarginase-Nbln (1 brand name, 1 dosage form, 1 route)
Available dosage forms: Injectable.
Routes of administration: Injection.
Brand Names
- LOARGYS
Boxed Warning
Patients treated with enzyme replacement therapies have experienced life-threatening hypersensitivity reactions, including anaphylaxis. Anaphylaxis has occurred during the early course of enzyme replacement therapy and after extended duration of therapy. Initiate LOARGYS in a healthcare setting with appropriate medical monitoring and support measures, including access to cardiopulmonary resuscitation equipment. If a severe hypersensitivity reaction (e.g. anaphylaxis) occurs, discontinue LOARGYS and immediately initiate appropriate medical treatment, including use of epinephrine. Inform patients of the symptoms of life-threatening hypersensitivity reactions, including anaphylaxis and to seek immediate medical care should symptoms occur [see Warnings and Precautions (5.1)].
Indications and Usage
LOARGYS is indicated for the treatment of hyperargininemia in adult and pediatric patients 2 years of age and older with Arginase 1 Deficiency (ARG1-D), in conjunction with dietary protein restriction. This indication is approved under accelerated approval based on reduction of plasma arginine [see Clinical Studies (14)]. Continued approval for this indication may be contingent upon verification and description of clinical benefit in a confirmatory trial.
Contraindications
None.
Adverse Reactions
The following adverse reactions are described below and elsewhere in the labeling: •Hypersensitivity Reactions [see Warnings and Precautions (5.1)].
Mechanism of Action
ARG1-D is an inherited metabolic disease characterized by deficiency of the arginase 1 enzyme and associated with the persistent elevation of plasma arginine. LOARGYS provides an exogenous source of the deficient human arginase 1 enzyme activity in patients with ARG1-D and reduces plasma arginine by converting it to urea and ornithine.