alpha1-proteinase inhibitor, human

REMS

Replacement therapy (chronic) in patients with emphysema associated with alpha₁-antitrypsin deficiency.

Contraind./Precautions ⬆ ⬇

Contraindicated in:

Hypersensitivity to polyethylene glycol (Aralast);
Emphysema associated with alpha₁-antitrypsin deficiency, where risk of panacinar emphysema is small (PiMZ and PiMS phenotype). ;
Selective IgA deficiency with known anti-IgA antibody (↑ risk of allergic reactions).

Use Cautiously in:

Patients at risk for volume overload;
OB: Safety not established.

Adv. Reactions/Side Effects ⬆ ⬇

GI: ↑liver enzymes

Hemat: transient leukocytosis

Neuro: dizziness, headache

Resp: cough, respiratory tract infection, sore throat

Misc: delayed fever

Interactions ⬆ ⬇

Drug-drug:

None known.

Availability ⬆ ⬇

Injection: 500 mg/vial (Aralast, Aralast NP); 1000 mg/vial (Aralast, Aralast NP, Glassia, Prolastin-C, Zemaira)

Route/Dosage ⬆ ⬇

IV (Adults ): 60 mg/kg once weekly.

US Brand Names ⬆ ⬇

alpha₁-antitrypsin, Aralast, Aralast NP, Glassia, Prolastin-C, Zemaira

Action ⬆ ⬇

Prevents the destructive action of elastase on alveolar tissue in patients who have alpha₁-antitrypsin deficiency.

Therapeutic effects:

Slowing of the destructive process on lung tissue.

Classifications ⬆ ⬇

Therapeutic Classification: pulmonary replacement enzyme inhibitor

Pharmacologic Classification: enzyme inhibitors

Pharmacokinetics ⬆ ⬇

Absorption: Following IV administration, absorption is essentially complete.

Distribution: Achieves high concentration in epithelial fluid of the lungs.

Metabolism/Excretion: Broken down in the intravascular space.

Half-Life: 4.5–5.9 days.

Time/Action Profile ⬆ ⬇

(↑ serum levels of alpha₁-proteinase inhibitor)

ROUTE	ONSET	PEAK	DURATION
IV	2–6 days	within 3 wks	unknown

Patient/Family Teaching ⬆ ⬇

Explain purpose of medication and need for long term weekly therapy to patient and family. Patient should avoid smoking and notify health care professional of any changes in breathing pattern or sputum production.
Inform patient that fever may occur within 12 hr after infusion and will resolve by 24 hr. Fever may be treated symptomatically.
Advise patient of need for periodic pulmonary function tests to determine disease progression and response to therapy.
Explain purpose of hepatitis B vaccine prior to beginning therapy. A small risk of hepatitis is caused by the manufacturing process, and vaccination is recommended.

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Indications ⬇