Primary ciliary dyskinesia (PCD) is a rare disease, due to malfunctioning of motile cilia, characterized by chronic airway infection. Aim of the study was to evaluate factors associated to radiological disease severity and lung function deterioration in PCD patients. A retrospective analysis of 58 PCD patients, 37 adults and 21 children, was performed. Patients were stratified according to the number of exacerbations (≥2/year versus < 2/year) and chronic Pseudomonas aeruginosa (PA) colonization. Demographic and clinical data, respiratory function and sputum microbiology were recorded. A chest CT and modified Bhalla score (mBhalla) were obtained for each patient. Univariate and multivariable linear regression models were used to evaluate possible correlations between lung function and chest CT-scores. High mBhalla scores were significantly associated with FEV1 (p=0.0002), age (p<0.0001), BMI (p=0.0002) and number of lung lobes involved (p<0.0001). PA colonization had an overall prevalence of 32.6 %; no significant difference in FEV1 between colonized and non-colonized patients was found (p=0.70), while chest CT score resulted significantly higher in chronic PA colonized patients (p=0.009). Patients with more frequent exacerbations (36/58) were older (p=0.01), had lower FEV1 (p=0.03), greater number of lobes involved (p=0.000) and higher mBhalla scores when compared to low exacerbation patients (p=0.0007); they also had higher prevalence of PA chronic bronchial infection (33.3 % versus 13.6 %, p=0.10). The number of exacerbations (≥ 2/year) and PA colonization were the two most relevant factors conditioning the radiological severity of disease.

Number of exacerbations and Pseudomonas aeruginosa colonization negatively impact on the radiological severity in Primary Ciliary Dyskinesia / A:farolfi, L. Barcellini, G. Zuccotti, U. Ambrosetti, M. DE SANTI, M. Patria, D. Consonni, G. Piatti. - In: EUROPEAN RESPIRATORY JOURNAL SUPPLEMENT. - ISSN 0904-1850. - 54:Suppl. 63(2019 Oct). ((Intervento presentato al convegno ERS International Congress 2019 abstracts : 28 September nel 2019 [10.1183/13993003.congress-2019.PA4581].

Number of exacerbations and Pseudomonas aeruginosa colonization negatively impact on the radiological severity in Primary Ciliary Dyskinesia

L. Barcellini
Secondo
;
G. Zuccotti;U. Ambrosetti;G. Piatti
Ultimo
2019

Abstract

Primary ciliary dyskinesia (PCD) is a rare disease, due to malfunctioning of motile cilia, characterized by chronic airway infection. Aim of the study was to evaluate factors associated to radiological disease severity and lung function deterioration in PCD patients. A retrospective analysis of 58 PCD patients, 37 adults and 21 children, was performed. Patients were stratified according to the number of exacerbations (≥2/year versus < 2/year) and chronic Pseudomonas aeruginosa (PA) colonization. Demographic and clinical data, respiratory function and sputum microbiology were recorded. A chest CT and modified Bhalla score (mBhalla) were obtained for each patient. Univariate and multivariable linear regression models were used to evaluate possible correlations between lung function and chest CT-scores. High mBhalla scores were significantly associated with FEV1 (p=0.0002), age (p<0.0001), BMI (p=0.0002) and number of lung lobes involved (p<0.0001). PA colonization had an overall prevalence of 32.6 %; no significant difference in FEV1 between colonized and non-colonized patients was found (p=0.70), while chest CT score resulted significantly higher in chronic PA colonized patients (p=0.009). Patients with more frequent exacerbations (36/58) were older (p=0.01), had lower FEV1 (p=0.03), greater number of lobes involved (p=0.000) and higher mBhalla scores when compared to low exacerbation patients (p=0.0007); they also had higher prevalence of PA chronic bronchial infection (33.3 % versus 13.6 %, p=0.10). The number of exacerbations (≥ 2/year) and PA colonization were the two most relevant factors conditioning the radiological severity of disease.
Primary ciliary dyskinesia, Pseudomonas aeuriginosa, exacerbations
Settore MED/10 - Malattie dell'Apparato Respiratorio
Article (author)
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Utilizza questo identificativo per citare o creare un link a questo documento: https://hdl.handle.net/2434/822949
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