Survival and FEV1 decline in individuals with severe deficiency of alpha1-antitrypsin. The Alpha-1-Antitrypsin Deficiency Registry Study Group. Academic Article uri icon

Overview

MeSH

  • Adult
  • Female
  • Humans
  • Male
  • Middle Aged
  • Multivariate Analysis
  • Phenotype
  • Predictive Value of Tests
  • Spirometry
  • Survival Analysis

MeSH Major

  • Forced Expiratory Volume
  • alpha 1-Antitrypsin
  • alpha 1-Antitrypsin Deficiency

abstract

  • 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.

authors

publication date

  • July 1998

has subject area

  • Adult
  • Female
  • Forced Expiratory Volume
  • Humans
  • Male
  • Middle Aged
  • Multivariate Analysis
  • Phenotype
  • Predictive Value of Tests
  • Spirometry
  • Survival Analysis
  • alpha 1-Antitrypsin
  • alpha 1-Antitrypsin Deficiency

Research

keywords

  • Journal Article

Identity

Language

  • eng

Digital Object Identifier (DOI)

  • 10.1164/ajrccm.158.1.9712017

PubMed ID

  • 9655706

Additional Document Info

start page

  • 49

end page

  • 59

volume

  • 158

number

  • 1