Drug information of alpha-1 proteinase inhibitor

alpha-1 proteinase inhibitor


Alpha 1-proteinase inhibitor is a protein, also called alpha 1-antitrypsin. This protein occurs naturally in the body and is important for preventing the breakdown of tissues in the lungs.

Mechanism of effect

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

Source: Human plasma donors

Pharmacodynamic

Prevents excessive accumulation of active neutrophil elastase and consequent proteolysis of elastin tissues in alveolar lung structures. This prevents the development of emphysema.

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

Drug indications

Alpha-1 Antitrypsin Deficiency

Dosage

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

Drug contraindications

Hypersensitivity

-Hypersensitivity

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

-Liver disease associated with alpha 1-proteinase inhibitor deficiency

Side effects

Headache , chest pain , vertigo , Cough , Injection-site reaction , vasodilatation , itching , difficulty urinating , Rash

Increases ALT, AST >2 times upper limit or normal, Infusion Reactions ,Headache ,Pharyngitis ,Increased cough ,Headache ,Somnolence ,Chills and fever ,Chest pain ,Dizziness ,Increased cough ,Pruritus ,Rash ,Vasodilation

Interactions

There are no known significant interactions.

Alerts

- Plasma volume may increase following infusion; use with caution in patients at risk for fluid overload.

-Theoretical risk of pathogen transmission. Product of human plasma; may potentially contain infectious agents which could transmit disease (eg, viruses and theoretically the Creutzfeldt-Jakob disease [CJD]). Screening of donors, as well as testing and/or inactivation or removal of certain viruses, reduces the risk. Infections thought to be transmitted by this product should be reported to the manufacturer

Points of recommendation

-For IV infusion only. Do not mix with other agents or solutions

-Monitoring Parameters: Alpha1-PI serum levels; lung function; vital signs during infusion

Pregnancy level

HAVE NOT BEEN ESTABLISHED

-There is no data on use in pregnant women to know this drugs risks, including the risk of fetal harm or reproductive effects.

Breast feeding warning

not known if distributed into breast milk, use caution

Drug forms

Aralast NP, Glassia, Prolastin C, Zemaira

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