Clinical impact of Pseudomonas aeruginosa colonization in patients with Primary Ciliary Dyskinesia

Autor: David Shoseyov, Mareike Price, Elie Picard, Nurith Hiller, Galit Livnat, Angelo Barbato, Deborah Snijders, Nir Weigert, Ugo Pradal, Huda Mussaffi, Mirella Collura, Michal Gur, Natalia Simanovsky, Kris De Boeck, Malena Cohen-Cymberknoh, Israel Amirav, Michal Shteinberg, Micha Aviram, Lea Bentur, Alex Gileles-Hillel, Hannah Blau, Joseph Rivlin, Eitan Kerem, Mieke Boon, Oded Breuer
Rok vydání: 2017
Předmět:
Male
Pathology
Lung deterioration
medicine.disease_cause
Gastroenterology
Severity of Illness Index
Pulmonary function testing
0302 clinical medicine
Forced Expiratory Volume
Colonization
030212 general & internal medicine
Child
Primary ciliary dyskinesia
Middle Aged
medicine.anatomical_structure
Child
Preschool
Carrier State
Pseudomonas aeruginosa
Disease Progression
Female
medicine.symptom
Pseudomonas colonization
Pulmonary and Respiratory Medicine
Adult
medicine.medical_specialty
Adolescent
Subgroup analysis
03 medical and health sciences
Young Adult
Internal medicine
medicine
Humans
Pseudomonas Infections
Aged
Retrospective Studies
Lung
business.industry
Kartagener Syndrome
Infant
Newborn
Sputum
Infant
medicine.disease
Lung function
digestive system diseases
respiratory tract diseases
Airway infection
030228 respiratory system
business
Airway
Tomography
X-Ray Computed
Zdroj: Respiratory medicine. 131
ISSN: 1532-3064
Popis: 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 (FEV 1 ) was lower in the colonized group (72.4 ± 22.0 vs. 80.1 ± 18.9, % predicted, p = 0.015), but FEV 1 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 FEV 1 and CT score. However, the magnitude of decline in pulmonary function was similar in colonized and non-colonized PCD patients.
Databáze: OpenAIRE
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