PEDIATRICS Vol. 63 No. 5 May 1979, pp. 821
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agr1-ANTITRYPSIN AND EMPHYSEMA

J. Lieberman M.D.

Preliminary data from a study of 917 California seventh graders reinforces the suspicion that Caucasians are at most risk of both an inherited deficiency of agr1-antitrypsin, and the emphysema with which the genetically-formulated blood chemical has been linked.

A serum protein, agr1-antitrypsin inactivates trypsin, one of the enzymes that checks bacteria infecting the lungs. When agr1-antitrypsin is reduced or absent, the trypsin can attack and destroy the lung tissue itself. The result is a particularly devastating form of emphysema that strikes during the productive years. An inherited deficiency of agr1-antitrypsin may be one reason why some nonsmokers develop emphysema.

All 25 of the students in this study found to have deficiencies of agr1-antitrypsin were Caucasian, as were nearly 90% of those discovered to have "variants" of agr1-antitrypsin molecules....Abnoraml results include a severe deficiency, inherited from both parents; intermediate deficiencies, inherited from one parent; and a number of antitrypsin variants also suspect in the development of emphysema. Although those with severe deficiencies nearly always develop emphysema, intermediate deficiencies and combinations of variants also predispose a person to the disease, particularly if they smoke cigarett.