Factors associated with pulmonary function test abnormalities

Factors evaluatedOR (95% CI)p-value
DC inheritance: AD non-TINF2 versus AR/XLR/TINF20.04 (<0.001–1.57)0.08
DC triad features: 2 or 3 versus 0–10.90 (0.12–6.55)0.92
Smoker versus nonsmoker70.85 (1.84– >999.999)0.02
Sex: male versus female0.25 (0.02–2.70)0.25
Age in years at PFT1.11 (0.99–1.24)0.07
Lymphocyte telomere Z-score0.63 (0.20–1.94)0.42
BMF: severe versus none or moderate17.36 (1.82–165.30)0.01

The multivariable regression model used includes dyskeratosis congenita (DC) inheritance (autosomal recessive (AR)/X-linked recessive (XLR)/TINF2 versus autosomal dominant (AD) non-TINF2), DC triad (0–1 versus 2–3), smoking, sex, age at pulmonary function testing (PFT) (integer), lymphocyte telomere Z-score, and bone marrow failure (BMF) (severe versus none/moderate). Overall, 38 patients were included in the multivariable regression model; five patients were excluded (four with unknown gene status and one without telomere length Z-score data). Significant p-values are in bold.