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Subjects >= 18 yr of age with serum alpha1-antitrypsin (alpha1-AT) levels <= 11 microM or a ZZ genotype were followed for 3.5 to 7 yr with spirometry measurements every 6 to 12 mo as part of a National Heart, Lung, and Blood Institute Registry of Patients with Severe Deficiency of Alpha-1-Antitrypsin. Among all 1,129 enrollees, 5-yr mortality was 19% (95% CI: 16 to 21%). In multivariate analyses of 1, 048 subjects who had been contacted >= 6 mo after enrolling, age and baseline FEV1% predicted were significant predictors of mortality. Results also showed that those subjects receiving augmentation therapy had decreased mortality (risk ratio [RR] = 0.64, 95% CI: 0. 43 to 0.94, p = 0.02) as compared with those not receiving therapy. Among 927 subjects with two or more FEV1 measurements >= 1 yr apart, the mean FEV1 decline was 54 ml/yr, with more rapid decline in males, those aged 30 to 44 yr, current smokers, those with FEV1 35 to 79% predicted, and those who ever had a bronchodilator response. Among all subjects, FEV1 decline was not different between augmentation-therapy groups (p = 0.40). However, among subjects with a mean FEV1 35 to 49% predicted, FEV1 decline was significantly slower for subjects receiving than for those not receiving augmentation therapy (mean difference = 27 ml/yr, 95% CI: 3 to 51 ml/yr; p = 0.03). Because this was not a randomized trial, we cannot exclude the possibility that these differences may have been due to other factors for which we could not control.

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The purpose of this paper is to examine the evidence, clinical, histological and experimental to try to determine the effect on bone of cholesteatosis. The term, cholesteatosis, borrowed from our English colleagues, is used in preference to cholesteatoma because the latter term indicates a neoplasm. Although primary cholesteatoma, the result of misplaced embryonic epithelial rests, does occur in the cranial cavity, it is a rare condition. Primary cholesteatoma is even more rare in the temporal bone and should not be confused with cholesteatosis which is the result of inflammation and altered aural physiology.The destructive effect of cholesteatosis on bone has long been recognized. Many considered the effect to be one of pressure due to accumulation of desquamated epithelial cells which were unable to escape through a narrow communication with the external ear. Others felt that there was some definite chemical process responsible for the lytic effect on the bone. Cholesterol was thought to play a part in this lytic process. Still others supposed that the connective tissue matrix had an effect on the underlying bone and was responsible for its destruction. It is important to determine what factors are really responsible for bone destruction so that surgical treatment may be based on sound premises.There are many cases which seem to lend credibility to the theory of pressure necrosis. It is not uncommon to see patients with a small attic perforation through which a small amount of discharge

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