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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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Ten patients (two male) suffering from acute exacerbations of long-standing chronic obstructive pulmonary disease and admitted in hypoxic, hypercapnic respiratory failure were treated with Nasal Intermittent Positive Pressure Ventilation (NIPPV) plus supplemental oxygen, on a general medical ward. The median (range) pH on admission was 7.30 (7.20-7.35), the median age was 67 years (47-77) with an FEV1 (percent of predicted) of 30 (17-39). On admission the median arterial oxygen tension (PaO2) was 4.71 kPa (3.45-6.26) on air, and the carbon dioxide tension (PaCO2) was 7.68 kPa (6.85-9.83). With controlled oxygen therapy there was no significant improvement in PaO2, but the median PaCO2 increased significantly to 9.75 kPa (7.04-11.70) (P < 0.05). By using NIPPV with supplemental oxygen it was possible to significantly improve the median PaO2 to 11.25 kPa (6.70-26.90) (P < 0.01) without worsening PaCO2 levels (8.96 kPa; 6.85-13.10). NIPPV was applied by a senior, respiratory physiotherapist and used intermittently depending on patient tolerance and clinical response. The median total time on NIPPV was 27 h, delivered over 1-5 days. One patient found the mask difficult to tolerate beyond a short period of time. NIPPV was well accepted on a general ward by nursing staff. Three patients later died with progressive hypercapnia, despite an initial response; with one of these patients also receiving intubation and mechanical ventilation. A further patient also received intubation and mechanical ventilation and was eventually discharged. NIPPV plus supplemental oxygen offers a method to correct hypoxaemia on a general medical ward without worsening hypercapnia for acute on chronic, hypoxic, hypercapnic respiratory failure, and warrants further investigation.