Givosiran

Givosiran Brand Name– GIVLAARI

What is Givosiran

Givosiran is a parenteral aminolevulinate synthase 1-directed small interfering RNA (siRNA) indicated for the treatment of acute hepatic porphyria (AHP) in adults. In the ENVISION trial (n = 94), a double-blind, placebo-controlled study, patients with acute hepatic porphyria, including acute intermittent porphyria (n = 89), variegate porphyria (n = 2), hereditary coproporphyria (n = 1), and no identified mutation (n = 2), having a minimum of 2 porphyria attacks requiring hospitalization, urgent healthcare visit, or intravenous hemin administration at home in the 6 months before study entry were included.

On average, givosiran-treated patients experienced 70% (95% CI 60% to 80%) fewer porphyria attacks compared to placebo.

Givosiran use also resulted in a reduction in hemin use as well as urinary aminolevulinic acid (ALA) and porphobilinogen (PBG), which are neurotoxic intermediates associated with porphyria attacks and other disease manifestations.

Serious adverse events, including anaphylaxis, hepatic transaminase elevation, and serum creatinine elevation with associated glomerular filtration rate decrease have been reported with givosiran.

Givosiran is intended for administration by a healthcare professional only and where medical support is available to appropriately manage anaphylactic reactions during administration.

Liver and renal function monitoring are recommended.

Indications

  • acute hepatic porphyria

For the treatment of acute hepatic porphyria

Side Effects

  1. anaphylactoid reactions
  2. antibody formation
  3. elevated hepatic enzymes
  4. erythema
  5. fatigue
  6. injection site reaction
  7. nausea
  8. pruritus
  9. rash
  10. skin discoloration
  11. urticaria

Monitoring Parameters

  • LFTs
  • serum creatinine

Contraindications

  • breast-feeding
  • hepatotoxicity
  • pregnancy
  • renal impairment

Interactions

  • Acetaminophen; Aspirin, ASA; Caffeine
  • Acetaminophen; Butalbital; Caffeine
  • Acetaminophen; Butalbital; Caffeine; Codeine
  • Acetaminophen; Caffeine
  • Acetaminophen; Caffeine; Dihydrocodeine
  • Acetaminophen; Caffeine; Magnesium Salicylate; Phenyltoloxamine
  • Acetaminophen; Caffeine; Phenyltoloxamine; Salicylamide
  • Acetaminophen; Chlorpheniramine; Dextromethorphan; Phenylephrine
  • Acetaminophen; Chlorpheniramine; Dextromethorphan; Pseudoephedrine
  • Acetaminophen; Codeine
  • Acetaminophen; Dextromethorphan
  • Acetaminophen; Dextromethorphan; Doxylamine
  • Acetaminophen; Dextromethorphan; Guaifenesin; Phenylephrine
  • Acetaminophen; Dextromethorphan; Phenylephrine
  • Acetaminophen; Dextromethorphan; Pseudoephedrine
  • Alosetron
  • Amitriptyline
  • Amitriptyline; Chlordiazepoxide
  • Amoxapine
  • Aspirin, ASA; Butalbital; Caffeine
  • Aspirin, ASA; Butalbital; Caffeine; Codeine
  • Aspirin, ASA; Caffeine; Dihydrocodeine
  • Aspirin, ASA; Caffeine; Orphenadrine
  • Aspirin, ASA; Carisoprodol; Codeine
  • Atomoxetine
  • Brompheniramine; Dextromethorphan; Guaifenesin
  • Brompheniramine; Dextromethorphan; Phenylephrine
  • Caffeine
  • Caffeine; Ergotamine
  • Carbamazepine
  • Carbinoxamine; Dextromethorphan; Pseudoephedrine
  • Chlorpheniramine; Codeine
  • Chlorpheniramine; Dextromethorphan
  • Chlorpheniramine; Dextromethorphan; Phenylephrine
  • Clomipramine
  • Codeine
  • Codeine; Guaifenesin
  • Codeine; Phenylephrine; Promethazine
  • Codeine; Promethazine
  • Desipramine
  • Dexchlorpheniramine; Dextromethorphan; Pseudoephedrine
  • Dextromethorphan
  • Dextromethorphan; Diphenhydramine; Phenylephrine
  • Dextromethorphan; Guaifenesin
  • Dextromethorphan; Guaifenesin; Phenylephrine
  • Dextromethorphan; Guaifenesin; Potassium Guaiacolsulfonate
  • Dextromethorphan; Guaifenesin; Pseudoephedrine
  • Dextromethorphan; Promethazine
  • Dextromethorphan; Quinidine
  • Doxepin
  • Duloxetine
  • Eliglustat
  • Imipramine
  • Maprotiline
  • Melatonin
  • Methadone
  • Nebivolol
  • Nebivolol; Valsartan
  • Nortriptyline
  • Perphenazine
  • Perphenazine; Amitriptyline
  • Pimozide
  • Protriptyline
  • Ramelteon
  • Tasimelteon
  • Theophylline, Aminophylline
  • Thioridazine
  • Tizanidine
  • Tolterodine
  • Trimipramine
  • Venlafaxine
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