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October 8, 1982

Intravenous Replacement Therapy for Patients With Severe α1-Antitrypsin Deficiency

JAMA. 1982;248(14):1693. doi:10.1001/jama.1982.03330140013005

A small group of patients with severe α1-antitrypsin deficiency and advanced emphysema has been treated with intravenous (IV) replacement therapy consisting of a partially purified preparation of human α1-antitrypsin from pooled plasma at the National Institutes of Health (NIH) Clinical Center.

The patients received 4 g of enriched α1-antitrypsin preparation IV during a six- to eight-hour period at weekly intervals for four consecutive weeks. All five patients tolerated the infusions—which were free of hepatitis B antigen and immunoglobulins—without serious adverse effects.

Drs James E. Gadek and Ronald G. Crystal of the National Heart, Lung, and Blood Institute (NHLBI) and Drs Harvey G. Klein and Paul V. Holland of the NIH Blood Bank developed the relatively rapid and simple method for partially purifying α1-antitrypsin from human plasma and administering it to patients.

α1-Antitrypsin is a protein that prevents the WBC enzyme,