a1-Proteinase Inhibitor

Class: Respiratory Tract Agents, Miscellaneous
VA Class: RE900
Brands: Aralast, Prolastin, Zemaira

Medically reviewed by Drugs.com on Mar 22, 2022. Written by ASHP.

Introduction

A naturally occurring serine protease inhibitor.

Uses for a1-Proteinase Inhibitor

Congenital α1-Proteinase Inhibitor Deficiency

Replacement therapy in patients with congenital α₁-proteinase inhibitor (also called α₁-antitrypsin) deficiency and clinically evident emphysema.

Not indicated as therapy for patients with lung disease in whom congenital α₁-proteinase inhibitor deficiency has not been established.

The American Thoracic Society and the European Respiratory Society (ATS/ERS) state that α₁-proteinase inhibitor therapy does not confer benefit in, and is not recommended for, patients who have α₁-proteinase-associated liver disease.

a1-Proteinase Inhibitor Dosage and Administration

Administration

IV Administration

Administer by IV infusion.

Administer IV infusions of Zemaira through an IV line using an administration set that contains an inline filter (pore size 5 µm).

Monitor infusion rate and clinical status (e.g., vital signs, infusion-related reactions) of the patient continuously throughout the infusion.

Administer with caution; percutaneous puncture with a needle contaminated with blood may transmit infectious agents. (See Risk of Transmissible Agents in Plasma-derived Preparations under Cautions.)

Reconstitution

Vials of lyophilized α₁-proteinase inhibitor and diluent should be at room temperature before reconstitution.

Reconstitute vials of lyophilized α₁-proteinase inhibitor with manufacturer-supplied sterile water for injection without preservatives. Using the supplied transfer needle or device, add the appropriate volume of the supplied diluent to a vial containing α₁-proteinase inhibitor. (See Table 1.) Swirl vial gently to ensure dissolution; do not shake.

Approximate amount (mg or g) of functionally active α₁-proteinase inhibitor.

Manufacturer-supplied sterile water for injection without preservatives.

Resultant solution of Aralast, Prolastin, or Zemaira contains not less than (NLT) 16 mg, NLT 20 mg, or approximately 50 mg of α₁-proteinase inhibitor per mL, respectively.

For administration of large dose...