TY - JOUR
T1 - Clinical impact of Pseudomonas aeruginosa colonization in patients with Primary Ciliary Dyskinesia
AU - Cohen-Cymberknoh, Malena
AU - Weigert, Nir
AU - Gileles-Hillel, Alex
AU - Breuer, Oded
AU - Simanovsky, Natalia
AU - Boon, Mieke
AU - De Boeck, Kris
AU - Barbato, Angelo
AU - Snijders, Deborah
AU - Collura, Mirella
AU - Pradal, Ugo
AU - Blau, Hannah
AU - Mussaffi, Huda
AU - Price, Mareike
AU - Bentur, Lea
AU - Gur, Michal
AU - Aviram, Micha
AU - Picard, Elie
AU - Shteinberg, Michal
AU - Livnat, Galit
AU - Rivlin, Joseph
AU - Hiller, Nurith
AU - Shoseyov, David
AU - Amirav, Israel
AU - Kerem, Eitan
N1 - Publisher Copyright:
© 2017 Elsevier Ltd
PY - 2017/10/1
Y1 - 2017/10/1
N2 - Background Airway infections in Primary Ciliary Dyskinesia (PCD) are caused by different microorganisms, including pseudomonas aeruginosa (PA). The aim of this study was to investigate the association of PA colonization and the progression of lung disease in PCD. Methods Data from 11PCD centers were retrospectively collected from 2008 to 2013. Patients were considered colonized if PA grew on at least two separate sputum cultures; otherwise, they were classified as non-colonized. These two groups were compared on the lung function computed tomography (CT) Brody score and other clinical parameters. Results Data were available from 217 patients; 60 (27.6%) of whom were assigned to the colonized group. Patients colonized with PA were older and were diagnosed at a later age. Baseline forced expiratory volume at 1 s (FEV1) was lower in the colonized group (72.4 ± 22.0 vs. 80.1 ± 18.9, % predicted, p = 0.015), but FEV1 declined throughout the study period was similar in both groups. The colonized group had significantly worse CT-Brody scores (36.07 ± 24.38 vs. 25.56 ± 24.2, p = 0.034). A subgroup analysis with more stringent definitions of colonization revealed similar results. Conclusions Lung PA colonization in PCD is associated with more severe disease as shown by the FEV1 and CT score. However, the magnitude of decline in pulmonary function was similar in colonized and non-colonized PCD patients.
AB - Background Airway infections in Primary Ciliary Dyskinesia (PCD) are caused by different microorganisms, including pseudomonas aeruginosa (PA). The aim of this study was to investigate the association of PA colonization and the progression of lung disease in PCD. Methods Data from 11PCD centers were retrospectively collected from 2008 to 2013. Patients were considered colonized if PA grew on at least two separate sputum cultures; otherwise, they were classified as non-colonized. These two groups were compared on the lung function computed tomography (CT) Brody score and other clinical parameters. Results Data were available from 217 patients; 60 (27.6%) of whom were assigned to the colonized group. Patients colonized with PA were older and were diagnosed at a later age. Baseline forced expiratory volume at 1 s (FEV1) was lower in the colonized group (72.4 ± 22.0 vs. 80.1 ± 18.9, % predicted, p = 0.015), but FEV1 declined throughout the study period was similar in both groups. The colonized group had significantly worse CT-Brody scores (36.07 ± 24.38 vs. 25.56 ± 24.2, p = 0.034). A subgroup analysis with more stringent definitions of colonization revealed similar results. Conclusions Lung PA colonization in PCD is associated with more severe disease as shown by the FEV1 and CT score. However, the magnitude of decline in pulmonary function was similar in colonized and non-colonized PCD patients.
KW - Airway infection
KW - Lung deterioration
KW - Lung function
KW - Primary ciliary dyskinesia
KW - Pseudomonas colonization
UR - http://www.scopus.com/inward/record.url?scp=85029378381&partnerID=8YFLogxK
U2 - 10.1016/j.rmed.2017.08.028
DO - 10.1016/j.rmed.2017.08.028
M3 - Article
C2 - 28947038
AN - SCOPUS:85029378381
SN - 0954-6111
VL - 131
SP - 241
EP - 246
JO - Respiratory Medicine
JF - Respiratory Medicine
ER -
