Pronunciation:
giv-o-si-ran
Trade Name(s)
Ther. Class.
none assigned
Pharm. Class.
aminolevulinate synthase 1 directed small interfering ribonucleic acids
Acute hepatic porphyria.
Causes degradation of aminolevulinate synthase 1 (ALAS1) mRNA in hepatocytes through RNA interference, which subsequently reduces circulating levels of aminolevulinic acid and porphobilinogen, both of which accumulate in acute hepatic porphyria.
Therapeutic Effect(s):
Reduction of porphyria attacks associated with hospitalizations, urgent healthcare visits, or IV hemin administration at home.
Absorption: Unknown.
Distribution: Distributed primarily to liver.
Protein Binding: 90%.
Metabolism and Excretion: Metabolized by nucleases to oligonucleotides of shorter lengths, including an active metabolite. Primarily excreted in urine (5–14% as unchanged drug; 4–13% as active metabolite).
Half-life: Givosiran– 6 hr. Active metabolite– 6 hr.
TIME/ACTION PROFILE (plasma concentrations)
ROUTE | ONSET | PEAK | DURATION |
---|---|---|---|
Subcut | unknown | 3 hr (givosiran); 7 hr (active metabolite) | unknown |
Contraindicated in:
Use Cautiously in:
Derm: rash
GI: ↑ liver enzymes, nausea
GU: renal impairment
Local: injection site reactions
Misc: HYPERSENSITIVITY REACTIONS (including anaphylaxis), fatigue
* CAPITALS indicate life-threatening.
Underline indicate most frequent.
Drug-Drug
SUBQ (Adults): 2.5 mg/kg (actual body weight) once monthly.
Solution for injection: 189 mg/mL
Monitor for signs and symptoms of anaphylactic reactions (rash, hives, dyspnea, swelling of lips, face and throat) during therapy.
Lab Test Considerations:
Measure liver function tests before starting therapy, repeat monthly during first 6 mo, and as clinically indicated. If clinically significant transaminase elevations occur, hold or discontinue therapy.
Administer by health care professional only. Have medical support available during injections to manage anaphylactic reactions.
Advise patient to notify health care professional immediately if signs and symptoms of anaphylaxis occur.
Reduction of porphyria attacks associated with hospitalizations, urgent healthcare visits, or IV hemin administration at home.