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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Sixty-six patients with chronic obstructive pulmonary disease (COPD) were evaluated for neuropsychological functioning. While the patients showed normal skills on broad intellectual tasks, they displayed mild cerebral deficits on several neuropsychological tests. Multiple regression analyses revealed that the neuropsychological test battery scores were significantly related to partial pressure of arterial oxygen (PaO2), and to degree of pulmonary impairment. Although the cognitive deficits were real, they were generally small. This result may explain why the widely assumed link between chronic lung disorders and cognitive deficit has been difficult to demonstrate. The general pattern was similar to deficits produced by chronic alcoholism and childhood asthma, with complex skills suffering the greatest insult. Because the cognitive deficits among COPD patients in stable condition are small, it is uncertain whether they have importance clinically or for accomplishing daily activities.

An earlier study by the authors had demonstrated neuropsychological impairment among 66 clinically stable patients suffering from chronic obstructive pulmonary disease (COPD), and had found that the deficits were directly associated with the amount of oxygen available to body tissue. We were able to follow 62 of the patients for three years when retrospective analyses were performed to determine the relationship of the neuropsychological test scores with the patients' three-year survival status. Patients who survived had scored significantly (p less than 0.01) higher on the neuropsychological tests than those who had died. Individual analyses of variance indicated that the significant relationship between the neuropsychological tests and survival status was attributable primarily to the Benton Visual Motor Retention Test and the digit symbol subtest of the Wechsler Adult Intelligence Scale. The latter test had, in the earlier study, shown the strongest relationship with partial pressure of oxygen and with a pulmonary function test. These findings suggest that certain neuropsychological tests may reflect both the progress of COPD and three-year survival probability among the patients.

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