Version May 2024
Note: The vial strength (mg) of some products (ie, Prolastin-C, Zemaira) varies by manufacturer lot; consult individual vial labels for exact amount prior to preparation for administration.
Alpha1-antitrypsin deficiency: IV: 60 mg/kg once weekly
There are no dosage adjustments provided in the manufacturer's labeling.
There are no dosage adjustments provided in the manufacturer's labeling.
Refer to adult dosing.
The following adverse drug reactions and incidences are derived from product labeling unless otherwise specified. Adverse reactions reported in adults.
Frequency not defined:
Cardiovascular: Chest discomfort, chest pain, peripheral edema, vasodilation
Dermatologic: Pruritus, skin rash, urticaria
Endocrine & metabolic: Hot flash
Gastrointestinal: Abdominal distention, abdominal pain, diarrhea, nausea, oral candidiasis
Genitourinary: Dysmenorrhea, urinary tract infection
Hepatic: Increased liver enzymes (including increased serum alanine aminotransferase, increased serum aspartate aminotransferase)
Hypersensitivity: Hypersensitivity reaction
Immunologic: Antibody development (including neutralizing)
Local: Bleeding at injection site, bruising at injection site
Nervous system: Asthenia, cerebral ischemia, chills, dizziness, drowsiness, fatigue, headache, hypertonia, hypoesthesia, insomnia, lethargy, nervousness, seizure
Neuromuscular & skeletal: Back pain, musculoskeletal pain (discomfort)
Ophthalmic: Conjunctivitis, visual disturbance
Otic: Tinnitus
Respiratory: Asthma, bronchitis, cough, dyspnea, exacerbation of chronic obstructive pulmonary disease, lower respiratory tract infection, nasopharyngitis, pharyngitis, rhinitis, rhinorrhea, sinusitis, upper respiratory tract infection
Miscellaneous: Fever
Postmarketing:
Cardiovascular: Flushing, increased blood pressure, swelling of extremities, tachycardia
Dermatologic: Exfoliative dermatitis, hyperhidrosis
Gastrointestinal: Vomiting
Hypersensitivity: Anaphylaxis, facial edema, hypersensitivity angiitis (Mwirigi 2009), lip edema, nonimmune anaphylaxis
Immunologic: Lymph node pain
Local: Infusion-site reaction
Nervous system: Malaise, pain, paresthesia
Neuromuscular & skeletal: Arthralgia, myalgia
Ophthalmic: Periorbital swelling
Respiratory: Flu-like symptoms, hemoptysis (Mwirigi 2009), wheezing (Mwirigi 2009)
History of anaphylaxis or other severe systemic reaction to A1-proteinase inhibitor or any component of the formulation; IgA deficient patients with antibodies against IgA.
Concerns related to adverse effects:
• Hypersensitivity: Severe hypersensitivity and anaphylactic/anaphylactoid reactions may occur; stop infusion promptly for acute hypersensitivity; immediate treatment (including epinephrine and/or other supportive therapy) should be available. Monitor vital signs and observe the patient throughout the infusion. May contain trace amounts of IgA; patients with known anti-IgA antibody, which may be present in patients with selective or severe IgA deficiency, have an increased risk of developing potentially severe hypersensitivity and anaphylactic reactions.
Disease-related concerns:
• Fluid overload: Plasma volume may increase following infusion; use with caution in patients at risk for fluid overload.
Dosage form specific issues:
• Human plasma: 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.
Strengths expressed with approximate values. Consult individual vial labels for exact potency within each vial.
Excipient information presented when available (limited, particularly for generics); consult specific product labeling.
Solution, Intravenous [preservative free]:
Glassia: 1000 mg/50 mL (1 ea) [latex free]
Prolastin-C: 1000 mg/20 mL (1 ea) [latex free, pyrogen free]
Solution Reconstituted, Intravenous [preservative free]:
Aralast NP: 500 mg (1 ea); 1000 mg (1 ea) [contains polyethylene glycol (macrogol), polysorbate 80]
Prolastin-C: 1000 mg (1 ea)
Zemaira: 1000 mg (1 ea); 4000 mg (1 ea); 5000 mg (1 ea)
No
Solution (Glassia Intravenous)
1000 mg/50 mL (0 Price provided is per milligram): $0.77
Solution (Prolastin-C Intravenous)
1000 mg/20 mL (0 Price provided is per milligram): $0.68
Solution (reconstituted) (Aralast NP Intravenous)
500 mg (0 Price provided is per milligram): $0.76
1000 mg (0 Price provided is per milligram): $0.76
Solution (reconstituted) (Prolastin-C Intravenous)
1000 mg (0 Price provided is per milligram): $0.62
Solution (reconstituted) (Zemaira Intravenous)
1000 mg (0 Price provided is per milligram): $0.67
4000 mg (0 Price provided is per milligram): $0.67
5000 mg (0 Price provided is per milligram): $0.67
Disclaimer: A representative AWP (Average Wholesale Price) price or price range is provided as reference price only. A range is provided when more than one manufacturer's AWP price is available and uses the low and high price reported by the manufacturers to determine the range. The pricing data should be used for benchmarking purposes only, and as such should not be used alone to set or adjudicate any prices for reimbursement or purchasing functions or considered to be an exact price for a single product and/or manufacturer. Medi-Span expressly disclaims all warranties of any kind or nature, whether express or implied, and assumes no liability with respect to accuracy of price or price range data published in its solutions. In no event shall Medi-Span be liable for special, indirect, incidental, or consequential damages arising from use of price or price range data. Pricing data is updated monthly.
Excipient information presented when available (limited, particularly for generics); consult specific product labeling.
Suspension Reconstituted, Intravenous:
Prolastin-C: 1000 mg (1 ea)
IV: For IV infusion only. Do not mix with other agents or solutions. If adverse reactions occur during administration, rate may be decreased or temporarily interrupted. Some products require an in-line filter for administration; consult specific product labeling.
Aralast NP, Glassia: Infuse at rate of ≤0.2 mL/kg/minute. Glassia may be self-administered by the patient/caregiver at home after appropriate training
Prolastin-C, Zemaira: Infuse at ~0.08 mL/kg/minute (rate may be increased or decreased based on patient comfort).
Prolastin-C Liquid: Infuse at 0.08 mL/kg/minute (takes ~15 minutes to infuse) as determined by patient response and comfort. Allow vial to warm to room temperature before administration; solution may contain a few protein particles.
Alpha1-proteinase inhibitor deficiency: Long-term augmentation and maintenance therapy in adults with severe hereditary deficiency of alpha1-antitrypsin (AAT) with clinically evident emphysema.
Limitations of use:
Not indicated as therapy for patients with lung disease in whom hereditary AAT deficiency has not been established; long-term effects of chronic augmentation or maintenance therapy of individuals with alpha1-proteinase inhibitor are not available.
The effect of augmentation therapy with any alpha1-proteinase inhibitor on pulmonary exacerbations and the progression of emphysema in alpha1-proteinase inhibitor deficiency has not been demonstrated in randomized, controlled clinical trials. However, one randomized controlled trial showed a reduction in emphysema progression with alpha1-proteinase inhibitor augmentation therapy when CT lung density was measured at total lung capacity (Chapman 2015).
None known.
There are no known significant interactions.
Animal reproduction studies have not been conducted.
It is not known if alpha1-proteinase inhibitor is present in breast milk.
According to the manufacturer, the decision to continue or discontinue breastfeeding during therapy should take into account the risk of infant exposure, the benefits of breastfeeding to the infant, and benefits of treatment to the mother.
Some products may contain sodium.
Alpha1-PI serum levels; lung function; vital signs during infusion
Alpha1-antitrypsin (AAT) is the principle protease inhibitor in serum. Its major physiologic role is to render proteolytic enzymes (secreted during inflammation) inactive. A decrease in AAT, as seen in congenital AAT deficiency, leads to increased elastic damage in the lung, causing emphysema.
Distribution: Vd: 3.2 ± 0.3 L.
Half-life elimination: Metabolic: ~5 to 6 days; terminal: 111 ± 33 hours.
Time to peak, serum: ~1 hour; threshold levels achieved after 3 weeks.
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