Alpha1-Antitrypsin Deficiency and Pulmonary Deficiency
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Data is presented to substantiate the hypothesis that serum alpha 1- antitrypsin deficiency is sometimes a factor in the occurrence of pulmonary emphysema. This theory is particularly valid when emphysema is manifest in patients under 40 years of age. The proposed role of alpha 1 -antitrypsin is to destroy the proteolytic enzymes released by leukocytes in the process of fighting infection. Diminished amounts of alpha 1 - antitrypsin are not sufficient to protect the lungs from the inflammatory exudate; this results in destruction of lung tissue. It is further postulated that alpha 1 -antitrypsin deficiency is transmitted by an autosomal recessive gene, a theory borne out by the pedigrees of the families studied.
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