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- Alpha1-Proteinase Inhibitor, Human
Excipient information presented when available (limited, particularly for generics); consult specific product labeling. [DSC] = Discontinued product
Solution, Intravenous [preservative free]:
Glassia: 1000 mg/50 mL (1 ea) [latex free]
Solution Reconstituted, Intravenous [preservative free]:
Aralast NP: 400 mg (1 ea [DSC]); 500 mg (1 ea); 800 mg (1 ea [DSC]); 1000 mg (1 ea) [contains polyethylene glycol, polysorbate 80]
Prolastin-C: 1000 mg (1 ea)
Zemaira: 1000 mg (1 ea)
Brand Names: U.S.
- Aralast NP
- Antitrypsin Deficiency Agent
- Blood Product Derivative
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.
Vd: ~3.5 L
Time to Peak
Serum: ~1 hour; threshold levels achieved after 3 weeks
Metabolic: ~5-6 days
Use: Labeled Indications
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:
Clinical data demonstrating the 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 on the progression of emphysema in AAT deficiency has not been demonstrated in randomized, controlled clinical trials.
Alpha1-proteinase inhibitors are not indicated as therapy for patients with lung disease in whom hereditary AAT deficiency has not been established.
Hypersensitivity to any component of the formulation or other A1-proteinase inhibitor products; IgA deficient patients with known anti-IgA antibody.
Alpha1-antitrypsin deficiency: IV: 60 mg/kg once weekly
Refer to adult dosing.
Dosing: Renal Impairment
There are no dosage adjustments provided in the manufacturer’s labeling.
Dosing: Hepatic Impairment
There are no dosage adjustments provided in the manufacturer’s labeling.
Reconstitute lyophilized powder with provided diluent (SWFI). Allow product and diluent to reach room temperature (if refrigerated) prior to reconstitution. Filtering requirements during preparation vary by product; consult manufacturer recommendations. May pool vial contents by transferring into a sterile container for IV infusion. To mix, swirl; do not shake. Administer within 3 hours of preparation or within 3 hours of entering the vial (Glassia); products do not contain a preservative.
Aralast NP: Each 500 mg and 1,000 mg vial should be diluted with 25 mL and 50 mL of diluent, respectively
Prolastin-C: Each 1,000 mg vial should be diluted with 20 mL of diluent
Zemaira: Each 1,000 mg vial should be diluted with 20 mL of diluent
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).
Some products may contain sodium.
Aralast NP: Prior to reconstitution, store intact vials up to 25°C (77°F); do not freeze. Keep vial in carton to protect from light.
Glassia: Prior to use, store intact vials between 2°C and 8°C (36°F and 46°F); do not freeze. May also be stored up to 25°C (77°F) for up to 1 month. Keep vial in carton until ready for use.
Prolastin-C, Zemaira: Prior to reconstitution, store intact vials up to 25°C (77°F); avoid freezing.
There are no known significant interactions.
Actual incidence may vary by product.
Cardiovascular: Chest pain (8%), chest discomfort (6%), vasodilatation (≥5%), peripheral edema (1% to ≤4%)
Central nervous system: Headache (1% to 16%), dizziness (5% to 6%), chills (≤5%)
Dermatologic: Urticaria (1% to 5%), pruritus (1% to 4%)
Endocrine & metabolic: Hot flash (5%)
Gastrointestinal: Nausea (1% to 11%), oral candidiasis (5%), sore throat (≥5%)
Genitourinary: Urinary tract infection (13%)
Hematologic & oncologic: Hemorrhage (≥5%)
Hepatic: Increased liver enzymes (6%)
Local: Bruising at injection site (8%)
Neuromuscular & skeletal: Musculoskeletal pain (discomfort: 16%), back pain (5%), weakness (≥5%)
Respiratory: Upper respiratory tract infection (2% to 16%), cough (5% to 9%), rhinorrhea (4% to 6%), bronchitis (≥5%), rhinitis (≥5%), sinusitis (≥5%), dyspnea (5%)
Miscellaneous: Fever (1% to ≥5%)
Postmarketing and/or case reports: Abdominal pain, asthma, back pain, confusion, diarrhea, drowsiness, flu-like symptoms, flushing, hypersensitivity reaction, hypoesthesia, hypoxia, injection site reaction, paresthesia, skin rash (exfoliative, generalized), syncope, tachycardia, visual disturbance, vomiting
Concerns related to adverse effects:
• Hypersensitivity: Severe hypersensitivity and anaphylactic reactions may occur; stop infusion promptly for acute hypersensitivity; immediate treatment (including epinephrine and/or other supportive therapy) should be available. 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.
• 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.
Alpha1-PI serum levels; lung function; vital signs during infusion
Pregnancy Risk Factor
Animal reproduction studies have not been conducted.
• Discuss specific use of drug and side effects with patient as it relates to treatment. (HCAHPS: During this hospital stay, were you given any medicine that you had not taken before? Before giving you any new medicine, how often did hospital staff tell you what the medicine was for? How often did hospital staff describe possible side effects in a way you could understand?)
• Patient may experience headache, signs of common cold, sinus pain, loss of strength and energy, muscle pain, nausea, rhinorrhea, or injection site irritation. Have patient report immediately to prescriber signs of parvovirus B19 or hepatitis A infection (chills, severe fatigue, rhinorrhea, rash, joint pain, lack of appetite, nausea, vomiting, abdominal pain, or jaundice), angina, severe dizziness, passing out, or shortness of breath (HCAHPS).
• Educate patient about signs of a significant reaction (eg, wheezing; chest tightness; fever; itching; bad cough; blue skin color; seizures; or swelling of face, lips, tongue, or throat). Note: This is not a comprehensive list of all side effects. Patient should consult prescriber for additional questions.
Intended Use and Disclaimer: Should not be printed and given to patients. This information is intended to serve as a concise initial reference for healthcare professionals to use when discussing medications with a patient. You must ultimately rely on your own discretion, experience and judgment in diagnosing, treating and advising patients.
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- Drug class: miscellaneous respiratory agents