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Alpha 1 proteinase Inhibitor Brand Names
Aralast | Aralast NP | Glassia | Prolastin | Prolastin C | Zemaira
What is Alpha 1 proteinase Inhibitor
Alpha 1 proteinase inhibitor (A1-PI) is a sterile, parenteral preparation of purified human alpha-1-proteinase inhibitor, which is also known as alpha-1-antitrypsin (AAT).
The product is derived from pooled human plasma of healthy donors. A1-PI is FDA-approved for the augmentation treatment of patients with congenital AAT-deficiency (AATD) who have clinically evident emphysema.
Weekly infusions raise plasma and lung epithelial fluid levels of AAT to normal in AAT-deficient individuals. Findings from the RAPID/RAPID Extension program have confirmed the benefits of A1-PI therapy in slowing disease progression and provided evidence of a disease-modifying effect in patients with AATD; the study suggests that the early introduction of treatment may delay the time to death, lung transplantation, or crippling respiratory complaints in patients with emphysema related to severe AATD.
In humans, A1-PI is a broad-spectrum anti-inflammatory, immunomodulatory, and tissue-repair molecule, and the products are under investigation for other purposes.
The drug has received orphan drug designation by the FDA in the off-label treatment of graft versus host disease, and also for patients with recent onset (less than 15 years) with Type 1A diabetes mellitus with residual beta-cell function.
Several different A1-PI products are available and the first product was FDA-approved in 1987.
Indications
- alpha-1 proteinase inhibitor (A1PI) deficiency
- emphysema
For chronic augmentation and maintenance therapy in adults with emphysema due to alpha-1 proteinase inhibitor (A1PI) deficiency
Side Effects
- abdominal pain
- anaphylactic shock
- anaphylactoid reactions
- arthralgia
- asthenia
- back pain
- bronchospasm
- candidiasis
- chest pain (unspecified)
- chills
- cholangitis
- cough
- diarrhea
- dizziness
- drowsiness
- dyspepsia
- dyspnea
- ecchymosis
- elevated hepatic enzymes
- fatigue
- fever
- flushing
- headache
- hot flashes
- hypertension
- hypotension
- infection
- injection site reaction
- malaise
- migraine
- musculoskeletal pain
- myalgia
- nausea
- paresthesias
- peripheral edema
- peripheral vasodilation
- pharyngitis
- pruritus
- rash
- rhinitis
- rhinorrhea
- sinus tachycardia
- sinusitis
- syncope
- urticaria
- vomiting
- wheezing
Monitoring Parameters
- pulmonary function tests (PFTs)
Contraindications
- breast-feeding
- children
- geriatric
- IgA deficiency
- infants
- neonates
- pregnancy
- viral infection
Interactions
There are no drug interactions associated with Alpha-1-proteinase Inhibitor products.