Characteristics of alpha-1 antitrypsin-deficient individuals in the long-term oxygen treatment trial and comparison with other subjects with chronic obstructive pulmonary disease

James K. Stoller, Loutfi S. Aboussouan, Richard E. Kanner, Laura A. Wilson, Phil Diaz, Robert Wise

Research output: Contribution to journalArticlepeer-review

Abstract

Rationale: Alpha-1 antitrypsin deficiency (AATD) predisposes to chronic obstructive pulmonary disease, but is underrecognized. Oxygenation and exercise desaturation in individuals with AATDassociated chronic obstructive pulmonary disease has been sparsely studied. The Long-term Oxygen Treatment Trial (LOTT) permits comparing these features of individuals with AATD with alpha-1 antitrypsin-replete (called "usual chronic obstructive pulmonary disease") LOTT participants. Objectives: Compare demographic, clinical, baseline oxygenation, and exercise desaturation features in participating AATD subjects with those of other LOTT subjects. Methods: LOTT is a multicenter randomized controlled trial comparing use of supplemental oxygen versus not in subjects with chronic obstructive pulmonary disease and moderate hypoxemia (resting oxygen saturation as measured by pulse oximetry, 89-93%) or normal oxygen saturation at rest and significant exercise desaturation. Measurement and Main Results: Among the 597 LOTT participants with nonmissing alpha-1 antitrypsin levels, 11 (1.8%) had severe AATD and 44 (7.4%) had mild/moderate AATD. Comparison of the 11 severely AAT-deficient individuals with the 542 LOTT participants with usual chronic obstructive pulmonary disease showed that theAATDsubjects were younger and despite less smoking, had lower FEV1/FVC (mean post-bronchodilator FEV1/ FVC, 0.3860.06 vs. 0.4660.13; P = 0.002). Comparison with 27 age-, sex-, and FEV1-matched alpha-1 antitrypsin-normal LOTT participants showed no baseline difference in resting room air pulse oximetry saturation (AATD, 93.6%62.3% vs. 92.7%62.2%; P = 0.64). Exercise-related desaturation was more severe in the individuals with AATD based on desaturation to 88% or less sooner during a 6-minute-walk test, having a higher percentage of desaturation points (e.g., ,90%) during exercise, and having a higher distance-saturation product (defined as the distance walked in 6 min multiplied by the nadir saturation achieved during the 6-minute-walk test). Conclusions: These data suggest that individuals with AATD experience more profound desaturation with exercise than age-, sex-, race-, and FEV1-matched control subjects with usual chronic obstructive pulmonary disease.

Original languageEnglish (US)
Pages (from-to)1796-1804
Number of pages9
JournalAnnals of the American Thoracic Society
Volume12
Issue number12
DOIs
StatePublished - Dec 2015

Keywords

  • Alpha-1 antitrypsin deficiency
  • Desaturation
  • Longterm oxygen treatment trial
  • Oxygenation

ASJC Scopus subject areas

  • Pulmonary and Respiratory Medicine

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