A1-Proteinase Inhibitor (Human)

Name: A1-Proteinase Inhibitor (Human)

a1-Proteinase Inhibitor (Human) Dosage and Administration

Administration

IV Administration

Administer by IV infusion.1 3 11

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

Monitor infusion rate and clinical status (e.g., vital signs, infusion-related reactions) of the patient continuously throughout the infusion.1 11

Administer with caution; percutaneous puncture with a needle contaminated with blood may transmit infectious agents.3 15 (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.1 3 11

Reconstitute vials of lyophilized α1-proteinase inhibitor with manufacturer-supplied sterile water for injection without preservatives.b c d 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.)b c d Swirl vial gently to ensure dissolution; do not shake.b c d

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

Manufacturer-supplied sterile water for injection without preservatives.b c d

Table 1. Reconstitution of α1-Proteinase Inhibitor Preparations

α1-Proteinase Inhibitor Preparation

Dosage Strength Labeled on Vial

Diluent Volume

Aralast

500 mg

25 mL1 16

Aralast

1 g

50 mL1 16

Prolastin

500 mg

20 mL3 9 15

Prolastin

1 g

40 mL3 9 15

Zemaira

1 g

20 mL11 15

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.1 3 15

For administration of large doses, several reconstituted vials may be pooled into an empty, sterile IV infusion container (e.g., empty IV bag or glass bottle) using aseptic technique.1 3 11 15

Withdraw reconstituted solutions of Aralast and Prolastin from the vial using a filter needle provided by the manufacturer.1 3 9 16

Reconstituted solutions contain no preservatives; administer within 3 hours after reconstitution.1 3 11

Any unused solution should be discarded; discard administration equipment in accordance with biohazard waste procedures.3 11 16 1 3 11

Rate of Administration

Administer Aralast at an infusion rate ≤0.08 mL/kg per minute.1 11

Administer Zemaira at an infusion rate of approximately 0.08 mL/kg per minute.b

Administer Prolastin at an infusion rate of ≥0.08 mL/kg per minute.b

If adverse effects occur, reduce infusion rate or temporarily interrupt infusion until manifestations subside.1 15 Infusion may then be resumed at a rate tolerated by the patient.1 15

Dosage

Dosage of α1-proteinase inhibitor in mg is expressed in terms of functionally active α1-proteinase inhibitor, as determined by human neutrophil (Zemaira) or porcine pancreatic (Aralast, Prolastin) elastase inhibitory activity.1 3 5 10 11 15 16

Number of mg of functionally active α1-proteinase inhibitor is indicated on the label of each vial.1 3 11 15 16

Specific activity of functional α1-proteinase inhibitor in Aralast, Prolastin, or Zemaira is NLT 0.55, NLT 0.35, or NLT 0.7 mg, respectively, per mg of protein.1 3 11

Adults

Congenital α1-Proteinase Inhibitor Deficiency IV

60 mg/kg by IV infusion once weekly.1 3 11

Cautions for a1-Proteinase Inhibitor (Human)

Contraindications

  • Individuals with selective IgA deficiencies1 3 11 (IgA concentrations <15 mg/dL) who have antibodies to IgA.1

  • History of anaphylaxis or severe systemic reaction to α1-proteinase inhibitors.b

  • Known hypersensitivity to α1-proteinase inhibitor or any ingredient in the formulation.b

Warnings/Precautions

Warnings

Risk of Transmissible Agents in Plasma-derived Preparations

Potential vehicle for transmission of human viruses (i.e., hepatitis A [HAV] or C virus [HCV]; HIV-1 or HIV-2; parvovirus B19)1 3 5 11 or other infectious agents.1 3 5 11

Despite stringent procedures (e.g., screening of plasma donors, application of a number of viral elimination/reduction steps) to prevent transmission of infectious agents, a risk of transmission still remains.1 3 11 12

Risk of viral infection should be weighed against the benefits of α1-proteinase inhibitor therapy.1 3 11

All infections thought possible to have been transmitted by α1-proteinase inhibitor products should be reported to the appropriate manufacturer.1 3 11

Risk of Creutzfeldt-Jakob Disease

May carry a risk of transmitting the causative agent of Creutzfeldt-Jakob disease (CJD), although transmission via human blood, blood components, or plasma derivatives (including α1-proteinase inhibitor) has not been documented.b c d e CJD is a rare, but invariably fatal, degenerative disease of the CNS associated with a poorly understood transmissable agent.e

There remains a theoretical risk that CJD can be transmitted through blood or blood products, although the risk is considered extremely remote.b c d e

Sensitivity Reactions

Hypersensitivity Reactions

Potential serious hypersensitivity reactions (e.g., anaphylactic or anaphylactoid reactions).1 11

If acute hypersensitivity reactions (e.g., hives, generalized urticaria, tightness of the chest, dyspnea, wheezing, faintness, hypotension, anaphylaxis) occur, discontinue immediately and institute appropriate therapy as indicated (e.g., epinephrine, corticosteroids, maintenance of an adequate airway, oxygen).1 11

General Precautions

Expansion of Plasma Volume

Transient expansion of plasma volume may occur during infusion; administer cautiously to patients at risk for circulatory overload.3 11

Specific Populations

Pregnancy

Category C.b c d

Lactation

Not known whether α1-proteinase inhibitor is distributed into milk.1 3 11 Caution advised if α1-proteinase inhibitor is used.1 3 11

Pediatric Use

Safety and efficacy not established.1

Hepatic Impairment

Use not recommended in patients with liver disease associated with α1-proteinase inhibitor deficiency.17

Common Adverse Effects

Headache,1 11 somnolence,1 delayed fever,3 lightheadedness,3 dizziness,3 11 asthenia,11 injection site pain,11 paresthesia,11 pruritus.11

Stability

Storage

Parenteral

Powder for Injection

Prolastin and Zemaira: ≤25°C; do not freeze.b d

Aralast: 2–8°C (may be exposed to ≤25°C); do not freeze.c Use within 1 month of removing from refrigeration.c

Actions

  • Inhibits serine proteases, which thereby helps prevent proteolytic destruction of the connective tissue framework of the lung parenchyma.1 3 11

Advice to Patients

  • Importance of patients understanding potential risks of therapy, including hypersensitivity reactions and possible transmission of infectious agents.1 3 11

  • Importance of women informing clinicians if they are or plan to become pregnant or plan to breast-feed.1 3 11

  • Importance of informing patients of other important precautionary information. (See Cautions.)

Liver Dose Adjustments

Data not available

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