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- What is alpha-1-proteinase inhibitor-injection, and how does it work (mechanism of action)?
- What are the side effects of alpha-1-proteinase inhibitor?
- What is the dosage for alpha-1-proteinase inhibitor?
- Which drugs or supplements interact with alpha-1-proteinase inhibitor?
- Is alpha-1-proteinase inhibitor safe to take if I'm pregnant or breastfeeding?
- What else should I know about alpha-1-proteinase inhibitor?
What is alpha-1-proteinase inhibitor-injection, and how does it work (mechanism of action)?
Alpha-1 proteinase inhibitor (Prolastin-C) is a prescription medicine used to treat alpha-1 antitrypsin (AAT) deficiency. AAT deficiency is a genetic disorder in which abnormally low levels of the protein AAT allow certain enzymes to attack healthy tissues, especially the lungs. Over time, significant lung damage occurs, and patients develop serious lung diseases like emphysema.
Alpha-1 proteinase inhibitor contains AAT donated by human subjects. AAT is derived from the plasma, the fluid part of the blood, of healthy human donors. Prolastin-C is a new formulation of Prolastin, the original alpha-1 proteinase inhibitor that has been available since 1988. Prolastin-C is a more purified and concentrated formulation that can be infused over a shorter period of time. When administered at the recommended rate, Prolastin-C can be infused in approximately 15 minutes. Additionally, unlike Prolastin, Prolastin-C does not contain prions, the infectious agent that causes mad cow disease or Creutzfeldt-Jakob disease. However, as alpha-1 proteinase inhibitor is derived from human plasma, it carries the potential risk of transmitting other infectious agents like viruses.
There is no cure for AAT deficiency but alpha-1 proteinase inhibitor can help patients manage their condition. Alpha-1 proteinase inhibitor works in the lower respiratory tract where it inhibits neutrophil elastase (NE), the enzyme that damages lung tissue. In clinical trials, alpha-1 proteinase inhibitor therapy increased the plasma levels of AAT. The effects of augmentation therapy on lung symptoms and disease progression have yet to be proven in well-controlled clinical trials.
What brand names are available for alpha-1-proteinase inhibitor-injection?
Is alpha-1-proteinase inhibitor-injection available as a generic drug?
Do I need a prescription for alpha-1-proteinase inhibitor-injection?
What are the side effects of alpha-1-proteinase inhibitor?
The most common side effects of alpha-1 proteinase inhibitor are:
What is the dosage for alpha-1-proteinase inhibitor?
The recommended dose of alpha-1 proteinase inhibitor is 60 mg/kg administered into the vein (intravenously) once weekly. Alpha-1 proteinase inhibitor should be administered at a rate of up to 0.08 milliliters for each kilogram per minute. On average, each infusion takes about 15 minutes.
Patients, family members, or caregivers can be trained to administer alpha1-proteinase inhibitor at home.
Which drugs or supplements interact with alpha-1-proteinase inhibitor?
Information on drug-drug interaction is not found in the medical literature and is not provided by the manufacturer.
Is alpha-1-proteinase inhibitor safe to take if I'm pregnant or breastfeeding?
Alpha-1 proteinase inhibitor has not been adequately evaluated in pregnant women. Due to the lack of conclusive safety data, alpha-1 proteinase inhibitor should be used in pregnancy only if the potential benefit justifies the potential risk to the fetus. Alpha-1 proteinase inhibitor is classified as FDA pregnancy risk category C.
It is not known if alpha-1 proteinase inhibitor is excreted in breast milk. Because many drugs enter human milk and can potentially cause harm to the nursing infant, alpha-1 proteinase inhibitor should be used cautiously in nursing mothers.
What else should I know about alpha-1-proteinase inhibitor?
What preparations of alpha-1-proteinase inhibitor are available?
Alpha-1 proteinase inhibitor is available in a kit containing a single-use vial of 1000 mg alpha-1 proteinase inhibitor as a lyophilized powder, one 20 ml vial of sterile water for dilution, a transfer needle, and a filter needle.
How should I keep alpha-1-proteinase inhibitor stored?
Alpha-1 proteinase inhibitor should be stored below 25 C (77 F). This medicine does not require refrigeration and should not be stored in the freezer. After mixing, the solution can be kept at room temperature for administration within 3 hours.
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Alpha-1 proteinase inhibitor (Prolastin-C) is an injectable drug derived from human blood plasma that helps correct genetic deficiencies of the alpha-1 antitrypsin (AAT) protein, which can lead to serious lung disease like emphysema. Side effects, drug interactions, and patient safety information should be reviewed prior to taking this medication.
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Related Disease Conditions
COPD (Chronic Obstructive Pulmonary Disease)
COPD or chronic obstructive pulmonary disease is a lung condition caused by smoking tobacco, exposure to secondhand smoke, and/or air pollutants. Conditions that accompany COPD include chronic bronchitis, chronic cough, and emphysema. Symptoms of COPD include shortness of breath, wheezing, and chronic cough. Treatment of COPD includes GOLD guidelines, smoking cessation, medications, and surgery. The life expectancy of a person with COPD depends on the stage of the disease.
Emphysema is a COPD (chronic obstructive pulmonary disease) that often occurs with other obstructive pulmonary problems and chronic bronchitis. Causes of emphysema include chronic cigarette smoking, exposure to secondhand smoke, air pollution, and in the underdeveloped parts of the world. Symptoms of emphysema include chronic cough, chest discomfort, breathlessness, and wheezing. Treatments include medication and lifestyle changes.
Alpha-1 Antitrypsin Deficiency (Symptoms, Treatment, Life Expectancy)
Alpha-1 antitrypsin deficiency (AATD) is an inherited disorder caused by mutations in the SERPINA1 gene. People with the condition are at risk for developing serious lung and liver disease. Symptoms and signs of lung disease caused by this condition include:The earliest symptoms and signs of lung disease usually develop between 20 and 50 years of age, and are Wheezing The reduced ability to exercise Shortness of breath (dyspnea) following mild activity Other symptoms and signs of alpha-1 antitrypsin deficiency are: Fatigue Rapid heartbeat when going from sitting to standing Recurring respiratory infections Unintentional weight loss Lung disease: People with this condition often develop emphysema, with symptoms of a hacking cough, barrel-shaped chest, and difficulty breathing. If you have this condition and smoke or are exposed to tobacco smoke, it accelerates the appearance of emphysema symptoms and lung damage.Liver disease: Alpha-1 antitrypsin deficiency also cause liver disease in some people with the condition, that include liver cancer, cirrhosis of the liver, an abnormally large liver (hepatomegaly), liver failure, and hepatitis. Liver damage from alpha-1 antitrypsin deficiency causes symptom of a swollen abdomen, swollen legs or feet, and jaundice. Treatment of AATD depends upon the severity of symptoms. FDA approved drug for AATD is an orphan product called alpha-1-proteinase inhibitor (human), sold under the brand name "Prolastin."
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