1. Ericksson,1965
2. Longstreth (1975)
3. Current case (1994) 1
4. Although no clear risk for the development of bronchiectasis in homozygous ol-antitrypsin deficient individuals has ever been established, a few reports have helped to confirm an association between this deficiency and bronchiectasis and pulmonary infections.' It has been suggested that the absence of this protease inhibitor accentuates the damage due to the unopposed protease activity which occurs in bronchitis and its accompanying neutrophilic infiltration of the bronchial wall.34 Repeated bronchopulmonary infection with severe PI, ZZ have been reported.5 It has been postulated that in the axl-antitrypsin-deficient individual repeated episodes of ordinary bronchitis, of whatever cause, may lead to the development of bronchiectasis.4 This suggestion seems to be supported by the study by Ericksson6 in which most of the 35 cases who underwent necropsy had bronchitis, although only two of the 35 had bronchiectasis
5. Studies from Sweden indicate that the frequency of the association of oc,-antitrypsin deficiency and bronchiectasis may be as high as 10%.i8 However, the true frequency of bronchiectasis in ocl-antitrypsin-deficient individuals remains to be determined. Conversely, the frequency of ac,-antitrypsin deficiency in series,unknown.4