alpha-1 proteinase inhibitor, Prolastin-C (cont.)

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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.

PRESCRIBED FOR: Alpha-1 proteinase inhibitor is a prescription medicine used to treat alpha-1 antitrypsin (AAT) deficiency, a genetic disorder in which low levels of AAT allows certain enzymes to attack healthy tissues in the body, especially the lungs. It is approved for the long term treatment of adults with clinically evident emphysema (a type of serious lung disease) caused by severe AAT deficiency. Alpha-1 proteinase inhibitor is used to replace AAT in the blood and lungs, and hence is known as “augmentation therapy” or “replacement therapy.”

SIDE EFFECTS: The most common side effects of alpha-1 proteinase inhibitor are:



PREPARATIONS: 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.

STORAGE: 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.

DOSING: 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.

Medically Reviewed by a Doctor on 3/20/2015

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