.alpha.1-proteinase inhibitor human

PROLASTIN ® -C LIQUID is an Alpha 1 -Proteinase Inhibitor (Human) (Alpha 1 -PI) indicated for chronic augmentation and maintenance therapy in adults with clinical evidence of emphysema due to severe hereditary deficiency of Alpha 1 -PI (alpha 1 -antitrypsin deficiency). Limitations of Use The effect of augmentation therapy with any Alpha 1 -PI, including PROLASTIN-C LIQUID, on pulmonary exacerbations and on the progression of emphysema in Alpha 1 -PI deficiency has not been conclusively demonstrated in randomized, controlled clinical trials. Clinical data demonstrating the long-term effects of chronic augmentation or maintenance therapy with PROLASTIN-C LIQUID are not available. PROLASTIN-C LIQUID is not indicated as therapy for lung disease in patients in whom severe Alpha 1 -PI deficiency has not been established.

Alpha 1 -PI deficiency is an autosomal, co-dominant, hereditary disorder characterized by low serum and lung levels of Alpha 1 -PI. Smoking is an important risk factor for the development of emphysema in patients with Alpha 1 -PI deficiency. Because emphysema affects many, but not all individuals with the more severe genetic variants of Alpha 1 -PI deficiency, augmentation therapy with Alpha 1 -PI is indicated only in patients with severe Alpha 1 -PI deficiency who have clinically evident emphysema. Only some Alpha 1 -PI alleles are associated with clinically apparent Alpha 1 -PI deficiency. Approximately 95% of all severely deficient patients are homozygous for the PiZ allele. Individuals with the PiZZ variant typically have serum Alpha 1 -PI levels less than 35% of the average normal level.

For intravenous use only. Dose: 60 mg/kg body weight intravenously once per week. Dose ranging studies using efficacy endpoints have not been performed with any Alpha 1 -PI product, including PROLASTIN-C LIQUID. Administration: 0.08 mL/kg/min as determined by patient response and comfort.

PROLASTIN-C LIQUID is contraindicated in: IgA deficient patients with antibodies against IgA, due to the risk of severe hypersensitivity. Patients with a history of anaphylaxis or other severe systemic reaction to Alpha 1 -PI. Immunoglobulin A (IgA) deficient patients with antibodies against IgA. History of anaphylaxis or other severe systemic reaction to Alpha 1 -PI.

Severe hypersensitivity and anaphylactic reactions may occur in IgA deficient patients with antibodies against IgA. Discontinue administration of the product and initiate appropriate emergency treatment if hypersensitivity reactions occur. Because PROLASTIN-C LIQUID is made from human plasma, it may carry a risk of transmitting infectious agents, e.g., viruses, the variant Creutzfeldt-Jakob disease (vCJD) agent, and, theoretically, the Creutzfeldt-Jakob disease (CJD) agent.

The most serious adverse reaction observed during clinical trials with PROLASTIN-C was an abdominal and extremity rash in one subject. The most common adverse reactions observed at a rate of > 5% in subjects receiving PROLASTIN-C LIQUID were diarrhea and fatigue, each of which occurred at a rate of 6% (two subjects each). The most common adverse reactions during PROLASTIN-C LIQUID clinical trials in > 5% of subjects were diarrhea and fatigue, each of which occurred in 2 subjects (6%).

Store refrigerated at 2-8°C (36-46°F) for the period indicated by the expiration date on its label. Product may be stored at room temperatures not exceeding 25 o C (77 o F) for up to one month, after which the product must be used or immediately discarded. Do not freeze.