RT Journal Article
SR Electronic
T1 Late-breaking abstract: A relative plasma elafin deficiency in children with cystic fibrosis (CF) is associated with pulmonary disease
JF European Respiratory Journal
JO Eur Respir J
FD European Respiratory Society
SP P1200
VO 44
IS Suppl 58
A1 Alex Yacob
A1 Jacquelyn Zirbes
A1 Colleen Dunn
A1 Roham Zamanian
A1 Richard Bland
A1 Marlene Rabinovitch
A1 Mark Nicolls
A1 Carlos Milla
YR 2014
UL http://erj.ersjournals.com/content/44/Suppl_58/P1200.abstract
AB Elafin is an inducible potent and selective serine elastase inhibitor with additional anti-inflammatory and anti-microbial properties. We hypothesized that a relative deficiency in circulating Elafin levels in relation to elastase, reflecting an imbalance in anti-elastolytic capacity, will be associated with the severity of lung disease in children with CF. Plasma samples were obtained from 35 children with CF free from exacerbation and compared to a group of 82 healthy individuals. Samples were assayed in duplicate by ELISA for Elafin and elastase concentrations, and the ratio of circulating elastase to Elafin (e:E) was calculated for each subject. Lung disease severity was assessed by forced expiratory volume in 1 second (FEV1). CF patients (17F, 18M) had a mean (SD) age of 12.8 (4) years, a FEV1 Z-score of -1.64 (1.7) and a Body Mass Index (BMI) z-score of 0.12 (0.9); 22% were infected with Pseudomonas, 75% with S. aureus and 8% were co-infected. Elafin levels were significantly lower in the CF patients compared to controls (17.2 vs. 75.4 ng/mL, p<0.001), whereas elastase levels were comparable (80.4 vs. 94.5 ng/mL, p=0.3). Mean e:E ratio in the CF children was 5.9 (3.1), which was significantly higher than the control group at 2.3 (1.8), (p<0.001). FEV1 Z-scores were not correlated with Elafin levels (p=0.4) but inversely correlated with elastase and e:E ratio (p <0.05 for both). Infection status, age or gender did not modify these associations. We conclude that in children with CF a relative deficiency in Elafin as reflected in a high e:E ratio is associated with the severity of lung disease. Elafin replenishment therapy could be a potential effective intervention.