alpha 1 proteinase inhibitor (Aralast NP, Glassia, Prolastin C, Zemaira)

Dosing and uses of Aralast NP, Glassia (alpha 1 proteinase inhibitor)

• Adult
• Pediatric

 

Adult dosage forms and strengths

powder for reconstitution

• 0.4g/vial
• 0.5g/vial
• 0.8g/vial
• 1g/vial

IV solution

• 1g/50mL vial

 

Alpha-1 Antitrypsin Deficiency

Indicated for chronic augmentation and maintenance therapy in adults with emphysema caused by congenital alpha-1-proteinase inhibitor deficiency

60 mg/kg IV infusion qWeek

IV infusion rate

• Aralast NP, Prolastin C, Zemaira: Not to exceed 0.08 mL/kg/min
• Glassia: 0.04 mL/kg/min

 

Orphan Designations

Diabetes Mellitus, Type-1 (Orphan)

• Glassia (IV): Treatment of recent onset (<15 yr) with type 1A diabetes mellitus with residual beta-cell function
• Prolastin C: Orphan designation for treatment of type 1 diabetes mellitus patients with residual beta-cell function
• Sponsors
  • Glassia: Kamada, Ltd.; Kiryat Weizmann, Science Park; Ness-Ziona 74036 Israel
  • Prolastin C: Grifols Therapeutics, Inc; 8368 US Business Hwy 70 West; Clayton, NC 27520

Alpha-1 Proteinase Inhibitor Deficiency (Orphan)

• Inhalation therapy for treatment of congenital deficiency of alpha-1 proteinase inhibitor
• Sponsor: Kamada, Ltd.; Kiryat Weizmann, Science Park;  Ness-Ziona 74036 Israel

Bronchopulmonary Dysplasia (Orphan)

• Prevention of bronchopulmonary dysplasia
• Sponsor: Arriva Pharmaceuticals, Inc; 1010 Atlantic Avenue; Alameda, CA 94501

Graft vs Host Disease (Orphan)

• Glassia: Treatment of GVHD
• Sponsor: Kamada, Ltd.; Kiryat Weizmann, Science Park;  Ness-Ziona 74036 Israel

Cystic Fibrosis (Orphan)

• Sponsor: CSL Behring LLC; 1020 First Avenue; King of Prussia, PA 19406
• Sponsor: Arriva Pharmacueticals, Inc; 1010 Atlantic Avenue; Alameda, CA 94501
• Sponsor: PPL Therapeutics (Scotland) Limited; Roslin, Edinburgh EH259PP, Scotland, United Kingdom

 

Pediatric dosage forms and strengths

Safety and efficacy not established

 

Aralast NP, Glassia (alpha 1 proteinase inhibitor) adverse (side) effects

>10%

Increases ALT, AST >2 times upper limit or normal (11%)

 

1-10%

Infusion Reactions (9.6%)

• Headache (0.7%)
• Pharyngitis (1.6%)
• Increased cough (0.6%)

 

<1%

Headache (0.3%)

Somnolence (0.3%)

Chills & fever (0.1%)

Chest pain (0.1%)

Dizziness (0.1%)

Increased cough (0.1%)

Pruritus (0.1%)

Rash (0.1%)

Vasodilation (0.1%)

 

Warnings

Contraindications

Hypersensitivity

Selective IgA deficiencies (IgA <15 mg/dL) with known IgA antibodies

Liver disease associated with alpha 1-proteinase inhibitor deficiency

 

Cautions

Patients at risk of circulatory overload

D/C immediately if anaphylactic or severe anaphylactoid reaction

Theoretical risk of pathogen transmission

• Report suspected infection
• Aralast to Alpha Therapeutic Corp. 1-888-675-2762 (US) or 1-323-225-9735 (international)
• Prolastin to Bayer Corp 1-800-288-8371
• Zemaira to ZLB Behring 1-800-504-5434

 

Pregnancy and lactation

Pregnancy category: C

Lactation: not known if distributed into breast milk, use caution

 

Pregnancy categories

A: Generally acceptable. Controlled studies in pregnant women show no evidence of fetal risk.

B: May be acceptable. Either animal studies show no risk but human studies not available or animal studies showed minor risks and human studies done and showed no risk.

C: Use with caution if benefits outweigh risks. Animal studies show risk and human studies not available or neither animal nor human studies done.

D: Use in LIFE-THREATENING emergencies when no safer drug available. Positive evidence of human fetal risk.

X: Do not use in pregnancy. Risks involved outweigh potential benefits. Safer alternatives exist.

NA: Information not available.

 

Pharmacology of Aralast NP, Glassia (alpha 1 proteinase inhibitor)

Mechanism of action

Purified human alpha-1-proteinase inhibitor (alpha-1-antitrypsin, ATT) inhibits serine proteases such as neutrophil elastase in lungs; alpha1-antitrypsin deficienty leads to the development of emphysema resulting from increased elastic damage in the lung

Source: Human plasma donors

 

Pharmacokinetics

Half-life elimination: 5-6 days

Vd: 3.5 L

Peak plasma time: 1 hr (3 weeks to achieve threshold levels)

Metabolism: Undergoes catabolism in the intravascular space; 33% of AAT is catabolized per day

 

Administration

IV Preparation

Use aseptic technique

Reconstitution requires no more than 5 min for 0.5 g vial and no more than 10 min for a 1 g viaL

Do not shake contents of the vial; do not invert vial until ready to withdraw contents

When reconstitution procedure is strictly followed, a few small particles may remain; will be removed by microaggregate filter

 

IV Administration

For IV infusion only

IV infusion rate

• Aralast NP, Prolastin C, Zemaira: Not to exceed 0.08 mL/kg/min
• Glassia: 0.04 mL/kg/min

 

Storage

Unopened vials

• Aralast NP, Prolastin C, Zemaira: Temperatures not to exceed 25°C (77°F); avoid freezing (may damage diluent vial)
• Glassia: 2-8 °C (36-46 °F); do not freeze

Opened vials

• Store at room temperature
• Use within 3 hr of entering/reconstituting the vials to avoid the potential ill effect of any inadvertent microbial contamination