1/2. Intermediate alpha 1-antitrypsin deficiency with apical lung bullae and spontaneous pneumothorax. Presence of a Z variant in an American black.A 43-year-old black man had an 18-year history of apical lung cystic-bullous disease. Following two episodes of spontaneous pneumothorax and two instances of thoracotomy for bullectomy and pleural abrasion, he was found to have an intermediate AAT deficiency with an MZ phenotype. It is believed that this is the first case of localized bullous lung disease to be reported in association with any degree of AAT deficiency. There is evidence that the cystic lesions progressed to form upper lobe bullae. It is postulated that the AAT deficiency may have played a role in this progression, as did the patient's cigarette smoking. Following two instances of surgery, CT scans of the lungs, compliance studies and complete pulmonary function tests show no further evidence of lung bullae or emphysema. The rarity of the Z variant of AAT in blacks is discussed.- - - - - - - - - - ranking = 1keywords = black (Clic here for more details about this article) |
2/2. alpha 1-antitrypsin deficiency and status asthmaticus in a black teen-ager.The rare occurrence of alpha 1-antitrypsin deficiency in black Americans has led to the suggestion that routine screening of such subjects in needless. Our report of a black teen-ager with deficiency of this protease inhibitor in association with status asthmaticus suggests that at least relatively young black patients with otherwise unexplained chronic pulmonary disease should be tested for this defect.- - - - - - - - - - ranking = 1.1666666666667keywords = black (Clic here for more details about this article) |