No

Antitrypsin Deficiency Agent

Congenital alpha₁-antitrypsin deficiency

C

<1%:

Hematologic: Leukocytosis

Alpha₁-proteinase inhibitor should be stored under refrigeration (2°C to 8°C;35°F to 46°F); freezing should be avoided as breakage of the diluent bottle might occur

Each bottle has the functional activity, as determined by inhibition of porcine pancreatic elastase, stated on the label of the bottle

Reconstitution directions:

Warm the unopened diluent and concentrate to room temperature

After removing the plastic flip-top caps, aseptically cleanse rubber stoppers of both bottles

Remove the protective cover from the plastic transfer needle cartridge with tamperproof seal and penetrate the stopper of the diluent bottle

Remove the remaining portion of the plastic cartridge; invert the diluent bottle and penetrate the rubber seal on the concentrate bottle with the needle at an angle

The vacuum will draw the diluent into the concentrate bottle; for best results, and to avoid foaming, hold the diluent bottle at an angle to the concentrate bottle until the powder is completed dissolved

After removing the diluent bottle and transfer needle, gently swirl the concentrate bottle until the powder is completely dissolved

Swab top of reconstituted bottle of alpha₁-proteinase inhibitor again

Attach the sterile filter needle provided to syringe; with filter needle in place, insert syringe into reconstituted bottle and withdraw solution into syringe

To administer, replace filter needle with appropriate injection needle and follow procedure for I.V. administration

The contents of more than one bottle of alpha₁-proteinase inhibitor may be drawn into the same syringe before administration

Human alpha₁-proteinase inhibitor is prepared from the pooled human plasma of normal donors and is intended for use in the therapy of congenital alpha₁-antitrypsin deficiency. Alpha₁-antitrypsin (AAT) is the principal protease inhibitor in the serum and exists as a single polypeptide glycoprotein. Production of AAT occurs in the liver hepatocyte and secretion occurs at a rate to maintain serum concentrations of 150-200 mg/dL. The major physiologic role of the antiprotease is that of combining with proteolytic enzymes to render them inactive. Several proteases can be inactivated by AAT including trypsin, chymotrypsin, coagulation factor XI, plasmin, thrombin, and neutrophil elastase.

Serum half-life, elimination (parent compound): 4.5-5.2 days

Adults: I.V.: 60 mg/kg once weekly (at a rate greater than or equal to 0.08 mL/kg/minute)

Administer at a rate of 0.08 mL/kg/minute or greater intravenously; the recommended dosage of 80 mg/kg takes approximately 30 minutes to infuse

Administer within 3 hours after reconstitution

No information available to require special precautions

No effects or complications reported

Sodium content of 1 L after reconstitution: 100-210 mEq

Injection (human): 500 mg alpha₁-PI (20 mL diluent); 1000 mg alpha ₁-PI (40 mL diluent)