Exacerbations and Pseudomonas aeruginosa colonization are associated with altered lung structure and function in primary ciliary dyskinesia.
Chest CT scores
Primary ciliary dyskinesia
Pseudomonas aeruginosa colonization
Respiratory exacerbations
Journal
BMC pediatrics
ISSN: 1471-2431
Titre abrégé: BMC Pediatr
Pays: England
ID NLM: 100967804
Informations de publication
Date de publication:
13 04 2020
13 04 2020
Historique:
received:
26
08
2019
accepted:
31
03
2020
entrez:
15
4
2020
pubmed:
15
4
2020
medline:
1
5
2021
Statut:
epublish
Résumé
Recurrent bacterial infections of the respiratory tract are one of the major clinical features of the primary ciliary dyskinesia (PCD), a rare genetic disease due to malfunctioning of motile cilia. Chronic infections and persistent inflammation of the respiratory system result in progressive lung disease. Aim of the study was to highlight the main factors associated with clinical, functional and anatomical deterioration in PCD patients. We retrospectively analyzed data from 58 patients with PCD, 37 adults and 21 children. The demographic and clinical data, forced expiratory volume at 1 s (FEV Chest CT scores showed a significant correlation with FEV In our PCD population the number of exacerbations (≥ 2/year) and PA colonization were the two most relevant factors associated with severity of disease.
Sections du résumé
BACKGROUND
Recurrent bacterial infections of the respiratory tract are one of the major clinical features of the primary ciliary dyskinesia (PCD), a rare genetic disease due to malfunctioning of motile cilia. Chronic infections and persistent inflammation of the respiratory system result in progressive lung disease. Aim of the study was to highlight the main factors associated with clinical, functional and anatomical deterioration in PCD patients.
METHODS
We retrospectively analyzed data from 58 patients with PCD, 37 adults and 21 children. The demographic and clinical data, forced expiratory volume at 1 s (FEV
RESULTS
Chest CT scores showed a significant correlation with FEV
CONCLUSIONS
In our PCD population the number of exacerbations (≥ 2/year) and PA colonization were the two most relevant factors associated with severity of disease.
Identifiants
pubmed: 32284045
doi: 10.1186/s12887-020-02062-4
pii: 10.1186/s12887-020-02062-4
pmc: PMC7153224
doi:
Types de publication
Journal Article
Langues
eng
Sous-ensembles de citation
IM
Pagination
158Références
Pediatr Pulmonol. 2002 Aug;34(2):91-100
pubmed: 12112774
Am J Respir Crit Care Med. 2005 Apr 15;171(8):912-30
pubmed: 15817806
Thorax. 2017 Feb;72(2):154-160
pubmed: 27382041
Eur Respir J. 2010 May;35(5):1057-63
pubmed: 19840971
Am J Respir Crit Care Med. 2000 Oct;162(4 Pt 1):1277-84
pubmed: 11029331
Eur Respir J. 2020 Apr 16;55(4):
pubmed: 32060067
Am J Respir Crit Care Med. 2004 Feb 15;169(4):459-67
pubmed: 14656747
Pediatr Pulmonol. 2012 Aug;47(8):816-25
pubmed: 22570319
Radiology. 1991 Jun;179(3):783-8
pubmed: 2027992
Chron Respir Dis. 2017 Nov;14(4):392-406
pubmed: 29081265
Ann Am Thorac Soc. 2013 Dec;10(6):574-81
pubmed: 24024753
Eur Respir J. 2016 Mar;47(3):829-36
pubmed: 26585432
Clin Microbiol Infect. 2015 Dec;21(12):1093.e1-7
pubmed: 26341913
Cilia. 2015 Jan 22;4(1):2
pubmed: 25610612
Ann Otol Rhinol Laryngol. 1997 Oct;106(10 Pt 1):854-8
pubmed: 9342982
Chest. 2008 Aug;134(2):351-357
pubmed: 18403663
Am J Respir Crit Care Med. 2019 Jan 15;199(2):190-198
pubmed: 30067075
Breathe (Sheff). 2017 Sep;13(3):166-178
pubmed: 28894478
Pharmacol Res. 2005 Feb;51(2):165-8
pubmed: 15629263
Hum Reprod. 2008 Aug;23(8):1957-62
pubmed: 18492703
Pediatr Pulmonol. 2016 Dec;51(12):1362-1366
pubmed: 27273679
Eur Respir J. 2016 Aug;48(2):441-50
pubmed: 27288033
Respir Med. 2017 Oct;131:241-246
pubmed: 28947038
Eur Respir J. 2011 Sep;38(3):603-7
pubmed: 21406509
J Comput Assist Tomogr. 1982 Jun;6(3):437-44
pubmed: 7096687
Eur Respir J. 2016 Mar;47(3):837-48
pubmed: 26647444
Chest. 2007 Nov;132(5):1565-72
pubmed: 17998359
Science. 1976 Jul 23;193(4250):317-9
pubmed: 1084576
Thorax. 1998 Oct;53(10):894-7
pubmed: 10193380
ERJ Open Res. 2019 Feb 01;5(1):
pubmed: 30723730
Eur Respir J. 2017 Jan 4;49(1):
pubmed: 27836958
Eur Respir J. 2014 May;43(5):1357-67
pubmed: 24232697
Am J Respir Crit Care Med. 2013 Oct 15;188(8):913-22
pubmed: 23796196
Am J Respir Crit Care Med. 2014 Mar 1;189(5):576-85
pubmed: 24328736
Ann Am Thorac Soc. 2015 Nov;12(11):1602-11
pubmed: 26356317
COPD. 2017 Mar 15;14(sup1):S12-S18
pubmed: 28306354
Clin Microbiol Rev. 2010 Apr;23(2):299-323
pubmed: 20375354
Pediatr Pulmonol. 2016 Feb;51(2):115-32
pubmed: 26418604
Int J Chron Obstruct Pulmon Dis. 2017 Jan 18;12:275-284
pubmed: 28182132
Front Pediatr. 2017 Apr 24;5:84
pubmed: 28596950