Inborn errors of metabolism:

Indications for ZEMAIRA:

For chronic augmentation and maintenance therapy in individuals with alpha1-proteinase inhibitor (A1-PI) deficiency and clinical evidence of emphysema.


Give by IV infusion at a rate of 0.08mL/kg/min. 60mg/kg once weekly. Monitor infusion rates and clinical state closely during infusion.


Not established.


History of anaphylaxis or severe systemic response to A1-PI products. Selective IgA deficiencies who have known antibodies against IgA.


Contains human plasma; monitor for possible infection transmission. Risk of circulatory overload. Pregnancy (Cat.C). Nursing mothers.

Adverse Reactions:

Asthenia, inj site pain, dizziness, headache, paresthesia, pruritus; hypersensitivity reactions (discontinue if occur).

How Supplied:

Single-use vial—1 (w. diluent + vented transfer device)

Pricing for ZEMAIRA

1000mg vial (Qty: 4)
Appx. price $2055