Longitudinal lung function and structural changes in children with primary ciliary dyskinesia
Background and Objectives Functional and structural lung evaluations are part of the follow‐up of patients with primary ciliary dyskinesia (PCD). We aimed to evaluate transversal and longitudinal relationships between lung function test (LFT) and chest computed tomography (CT) in children with PCD,...
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| Veröffentlicht in: | Pediatric pulmonology 2012-08, Vol.47 (8), p.816-825 |
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| container_title | Pediatric pulmonology |
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| creator | Magnin, Marie Lémery Cros, Pierrick Beydon, Nicole Mahloul, Malika Tamalet, Aline Escudier, Estelle Clément, Annick Le Pointe, Hubert Ducou Blanchon, Sylvain |
| description | Background and Objectives
Functional and structural lung evaluations are part of the follow‐up of patients with primary ciliary dyskinesia (PCD). We aimed to evaluate transversal and longitudinal relationships between lung function test (LFT) and chest computed tomography (CT) in children with PCD, in stable clinical condition.
Materials and Methods
Data from children followed in the French National Center were retrospectively collected. Inclusion criteria were (i) definitive diagnosis of PCD, (ii) age less than 15 years at the beginning of follow‐up, (iii) at least 8 years of follow‐up, (iv) at least two couples of concurrent CT and LFT available in a phase of clinical stability of the lung disease without modification of the treatment regimen in the last 4 weeks. Twenty children (median age at entry 4.6 years, median follow‐up 15.4 years) were included. Concurrent LFT (blood gas and spirometry) and CT (score) results were recorded.
Results
LFT indices (PaO2 (n = 210), FVC, FEV1, FEF2575% (n = 195)) significantly decreased with age, and the mean annual decrease (z‐score (% predicted)) was −0.17 (−0.49%), −0.09 (−0.50%), −0.10 (−0.89%), and −0.07 (−1.73%), respectively. First CT (median age 8.7 years) revealed bronchiectasis (70%), mucous plugging (70%), peribronchial thickening (90%), parenchymal abnormalities (65%), and hyperinflation (50%). CT scores (n = 74) significantly increased with age, and was negatively correlated to PaO2, FVC, FEV1, and FEF2575% longitudinal changes.
Conclusion
In stable clinical condition, functional, and structural progressive impairments significantly correlated in children with PCD. Further prospective studies, including large populations of patients with various levels of disease severity, are needed to confirm whether lung function follow‐up can be used to adjust CT frequency and help at minimizing the radiation burden in children with a good life expectancy. Pediatr Pulmonol. 2012. 47:816–825. © 2012 Wiley Periodicals, Inc. |
| doi_str_mv | 10.1002/ppul.22577 |
| format | Article |
| fullrecord | <record><control><sourceid>proquest_cross</sourceid><recordid>TN_cdi_proquest_miscellaneous_1024937488</recordid><sourceformat>XML</sourceformat><sourcesystem>PC</sourcesystem><sourcerecordid>1024937488</sourcerecordid><originalsourceid>FETCH-LOGICAL-c4337-fa6312512ce2af7c638d19068e2d2b971cdd7d2e48626cafed979fa5acc0a0f83</originalsourceid><addsrcrecordid>eNp90VtrFDEUB_Agil2rL34AmRdBhKm5zOTyKEWrsKwtWgRBwmku29hsZptMqPvtnXG39c2nE8jvnBP-QeglwScEY_puu63xhNJeiEdoQbBSLe4Uf4wWUvR9yyVnR-hZKb8wnu4UeYqOZowZUQv0czmkdRirDQliE2taN74mM4YhNZBsU8ZczVjzdGmuIa1daUKajiHa7FJzF8brZpvDBvKuMSGGudpduQnJlQDP0RMPsbgXh3qMLj9--Hb6qV1-Oft8-n7Zmo4x0XrgjNCeUOMoeGE4k5YozKWjll4pQYy1wlLXSU65Ae-sEspDD8ZgwF6yY_RmP3ebh9vqyqg3oRgXIyQ31KIJpp1iopMzfbunJg-lZOf14fkT0nOceo5T_41zwq8Oc-vVxtkHep_fBF4fABQD0WdIJpR_jpPpC3o-ObJ3dyG63X9W6vPzy-X98nbfE8rofj_0QL7RXDDR6--rM33xdbWSP6TUF-wPOumehQ</addsrcrecordid><sourcetype>Aggregation Database</sourcetype><iscdi>true</iscdi><recordtype>article</recordtype><pqid>1024937488</pqid></control><display><type>article</type><title>Longitudinal lung function and structural changes in children with primary ciliary dyskinesia</title><source>MEDLINE</source><source>Access via Wiley Online Library</source><creator>Magnin, Marie Lémery ; Cros, Pierrick ; Beydon, Nicole ; Mahloul, Malika ; Tamalet, Aline ; Escudier, Estelle ; Clément, Annick ; Le Pointe, Hubert Ducou ; Blanchon, Sylvain</creator><creatorcontrib>Magnin, Marie Lémery ; Cros, Pierrick ; Beydon, Nicole ; Mahloul, Malika ; Tamalet, Aline ; Escudier, Estelle ; Clément, Annick ; Le Pointe, Hubert Ducou ; Blanchon, Sylvain</creatorcontrib><description>Background and Objectives
Functional and structural lung evaluations are part of the follow‐up of patients with primary ciliary dyskinesia (PCD). We aimed to evaluate transversal and longitudinal relationships between lung function test (LFT) and chest computed tomography (CT) in children with PCD, in stable clinical condition.
Materials and Methods
Data from children followed in the French National Center were retrospectively collected. Inclusion criteria were (i) definitive diagnosis of PCD, (ii) age less than 15 years at the beginning of follow‐up, (iii) at least 8 years of follow‐up, (iv) at least two couples of concurrent CT and LFT available in a phase of clinical stability of the lung disease without modification of the treatment regimen in the last 4 weeks. Twenty children (median age at entry 4.6 years, median follow‐up 15.4 years) were included. Concurrent LFT (blood gas and spirometry) and CT (score) results were recorded.
Results
LFT indices (PaO2 (n = 210), FVC, FEV1, FEF2575% (n = 195)) significantly decreased with age, and the mean annual decrease (z‐score (% predicted)) was −0.17 (−0.49%), −0.09 (−0.50%), −0.10 (−0.89%), and −0.07 (−1.73%), respectively. First CT (median age 8.7 years) revealed bronchiectasis (70%), mucous plugging (70%), peribronchial thickening (90%), parenchymal abnormalities (65%), and hyperinflation (50%). CT scores (n = 74) significantly increased with age, and was negatively correlated to PaO2, FVC, FEV1, and FEF2575% longitudinal changes.
Conclusion
In stable clinical condition, functional, and structural progressive impairments significantly correlated in children with PCD. Further prospective studies, including large populations of patients with various levels of disease severity, are needed to confirm whether lung function follow‐up can be used to adjust CT frequency and help at minimizing the radiation burden in children with a good life expectancy. Pediatr Pulmonol. 2012. 47:816–825. © 2012 Wiley Periodicals, Inc.</description><identifier>ISSN: 8755-6863</identifier><identifier>EISSN: 1099-0496</identifier><identifier>DOI: 10.1002/ppul.22577</identifier><identifier>PMID: 22570319</identifier><identifier>CODEN: PEPUES</identifier><language>eng</language><publisher>Hoboken: Wiley Subscription Services, Inc., A Wiley Company</publisher><subject>Adolescent ; Adult ; Biological and medical sciences ; blood gas ; Blood Gas Analysis ; Child ; Child, Preschool ; Complex syndromes ; computed tomography ; Disease Progression ; Female ; General aspects ; Humans ; Infant ; Kartagener syndrome ; Kartagener Syndrome - diagnostic imaging ; Kartagener Syndrome - physiopathology ; Longitudinal Studies ; Lung - diagnostic imaging ; Lung - physiopathology ; lung function test ; Male ; Medical genetics ; Medical sciences ; Pneumology ; Respiratory Function Tests ; Retrospective Studies ; Tomography, X-Ray Computed</subject><ispartof>Pediatric pulmonology, 2012-08, Vol.47 (8), p.816-825</ispartof><rights>Copyright © 2012 Wiley Periodicals, Inc.</rights><rights>2015 INIST-CNRS</rights><lds50>peer_reviewed</lds50><oa>free_for_read</oa><woscitedreferencessubscribed>false</woscitedreferencessubscribed><citedby>FETCH-LOGICAL-c4337-fa6312512ce2af7c638d19068e2d2b971cdd7d2e48626cafed979fa5acc0a0f83</citedby><cites>FETCH-LOGICAL-c4337-fa6312512ce2af7c638d19068e2d2b971cdd7d2e48626cafed979fa5acc0a0f83</cites></display><links><openurl>$$Topenurl_article</openurl><openurlfulltext>$$Topenurlfull_article</openurlfulltext><thumbnail>$$Tsyndetics_thumb_exl</thumbnail><linktopdf>$$Uhttps://onlinelibrary.wiley.com/doi/pdf/10.1002%2Fppul.22577$$EPDF$$P50$$Gwiley$$H</linktopdf><linktohtml>$$Uhttps://onlinelibrary.wiley.com/doi/full/10.1002%2Fppul.22577$$EHTML$$P50$$Gwiley$$H</linktohtml><link.rule.ids>314,780,784,1417,27924,27925,45574,45575</link.rule.ids><backlink>$$Uhttp://pascal-francis.inist.fr/vibad/index.php?action=getRecordDetail&idt=26175556$$DView record in Pascal Francis$$Hfree_for_read</backlink><backlink>$$Uhttps://www.ncbi.nlm.nih.gov/pubmed/22570319$$D View this record in MEDLINE/PubMed$$Hfree_for_read</backlink></links><search><creatorcontrib>Magnin, Marie Lémery</creatorcontrib><creatorcontrib>Cros, Pierrick</creatorcontrib><creatorcontrib>Beydon, Nicole</creatorcontrib><creatorcontrib>Mahloul, Malika</creatorcontrib><creatorcontrib>Tamalet, Aline</creatorcontrib><creatorcontrib>Escudier, Estelle</creatorcontrib><creatorcontrib>Clément, Annick</creatorcontrib><creatorcontrib>Le Pointe, Hubert Ducou</creatorcontrib><creatorcontrib>Blanchon, Sylvain</creatorcontrib><title>Longitudinal lung function and structural changes in children with primary ciliary dyskinesia</title><title>Pediatric pulmonology</title><addtitle>Pediatr. Pulmonol</addtitle><description>Background and Objectives
Functional and structural lung evaluations are part of the follow‐up of patients with primary ciliary dyskinesia (PCD). We aimed to evaluate transversal and longitudinal relationships between lung function test (LFT) and chest computed tomography (CT) in children with PCD, in stable clinical condition.
Materials and Methods
Data from children followed in the French National Center were retrospectively collected. Inclusion criteria were (i) definitive diagnosis of PCD, (ii) age less than 15 years at the beginning of follow‐up, (iii) at least 8 years of follow‐up, (iv) at least two couples of concurrent CT and LFT available in a phase of clinical stability of the lung disease without modification of the treatment regimen in the last 4 weeks. Twenty children (median age at entry 4.6 years, median follow‐up 15.4 years) were included. Concurrent LFT (blood gas and spirometry) and CT (score) results were recorded.
Results
LFT indices (PaO2 (n = 210), FVC, FEV1, FEF2575% (n = 195)) significantly decreased with age, and the mean annual decrease (z‐score (% predicted)) was −0.17 (−0.49%), −0.09 (−0.50%), −0.10 (−0.89%), and −0.07 (−1.73%), respectively. First CT (median age 8.7 years) revealed bronchiectasis (70%), mucous plugging (70%), peribronchial thickening (90%), parenchymal abnormalities (65%), and hyperinflation (50%). CT scores (n = 74) significantly increased with age, and was negatively correlated to PaO2, FVC, FEV1, and FEF2575% longitudinal changes.
Conclusion
In stable clinical condition, functional, and structural progressive impairments significantly correlated in children with PCD. Further prospective studies, including large populations of patients with various levels of disease severity, are needed to confirm whether lung function follow‐up can be used to adjust CT frequency and help at minimizing the radiation burden in children with a good life expectancy. Pediatr Pulmonol. 2012. 47:816–825. © 2012 Wiley Periodicals, Inc.</description><subject>Adolescent</subject><subject>Adult</subject><subject>Biological and medical sciences</subject><subject>blood gas</subject><subject>Blood Gas Analysis</subject><subject>Child</subject><subject>Child, Preschool</subject><subject>Complex syndromes</subject><subject>computed tomography</subject><subject>Disease Progression</subject><subject>Female</subject><subject>General aspects</subject><subject>Humans</subject><subject>Infant</subject><subject>Kartagener syndrome</subject><subject>Kartagener Syndrome - diagnostic imaging</subject><subject>Kartagener Syndrome - physiopathology</subject><subject>Longitudinal Studies</subject><subject>Lung - diagnostic imaging</subject><subject>Lung - physiopathology</subject><subject>lung function test</subject><subject>Male</subject><subject>Medical genetics</subject><subject>Medical sciences</subject><subject>Pneumology</subject><subject>Respiratory Function Tests</subject><subject>Retrospective Studies</subject><subject>Tomography, X-Ray Computed</subject><issn>8755-6863</issn><issn>1099-0496</issn><fulltext>true</fulltext><rsrctype>article</rsrctype><creationdate>2012</creationdate><recordtype>article</recordtype><sourceid>EIF</sourceid><recordid>eNp90VtrFDEUB_Agil2rL34AmRdBhKm5zOTyKEWrsKwtWgRBwmku29hsZptMqPvtnXG39c2nE8jvnBP-QeglwScEY_puu63xhNJeiEdoQbBSLe4Uf4wWUvR9yyVnR-hZKb8wnu4UeYqOZowZUQv0czmkdRirDQliE2taN74mM4YhNZBsU8ZczVjzdGmuIa1daUKajiHa7FJzF8brZpvDBvKuMSGGudpduQnJlQDP0RMPsbgXh3qMLj9--Hb6qV1-Oft8-n7Zmo4x0XrgjNCeUOMoeGE4k5YozKWjll4pQYy1wlLXSU65Ae-sEspDD8ZgwF6yY_RmP3ebh9vqyqg3oRgXIyQ31KIJpp1iopMzfbunJg-lZOf14fkT0nOceo5T_41zwq8Oc-vVxtkHep_fBF4fABQD0WdIJpR_jpPpC3o-ObJ3dyG63X9W6vPzy-X98nbfE8rofj_0QL7RXDDR6--rM33xdbWSP6TUF-wPOumehQ</recordid><startdate>201208</startdate><enddate>201208</enddate><creator>Magnin, Marie Lémery</creator><creator>Cros, Pierrick</creator><creator>Beydon, Nicole</creator><creator>Mahloul, Malika</creator><creator>Tamalet, Aline</creator><creator>Escudier, Estelle</creator><creator>Clément, Annick</creator><creator>Le Pointe, Hubert Ducou</creator><creator>Blanchon, Sylvain</creator><general>Wiley Subscription Services, Inc., A Wiley Company</general><general>Wiley-Liss</general><scope>BSCLL</scope><scope>IQODW</scope><scope>CGR</scope><scope>CUY</scope><scope>CVF</scope><scope>ECM</scope><scope>EIF</scope><scope>NPM</scope><scope>AAYXX</scope><scope>CITATION</scope><scope>7X8</scope></search><sort><creationdate>201208</creationdate><title>Longitudinal lung function and structural changes in children with primary ciliary dyskinesia</title><author>Magnin, Marie Lémery ; Cros, Pierrick ; Beydon, Nicole ; Mahloul, Malika ; Tamalet, Aline ; Escudier, Estelle ; Clément, Annick ; Le Pointe, Hubert Ducou ; Blanchon, Sylvain</author></sort><facets><frbrtype>5</frbrtype><frbrgroupid>cdi_FETCH-LOGICAL-c4337-fa6312512ce2af7c638d19068e2d2b971cdd7d2e48626cafed979fa5acc0a0f83</frbrgroupid><rsrctype>articles</rsrctype><prefilter>articles</prefilter><language>eng</language><creationdate>2012</creationdate><topic>Adolescent</topic><topic>Adult</topic><topic>Biological and medical sciences</topic><topic>blood gas</topic><topic>Blood Gas Analysis</topic><topic>Child</topic><topic>Child, Preschool</topic><topic>Complex syndromes</topic><topic>computed tomography</topic><topic>Disease Progression</topic><topic>Female</topic><topic>General aspects</topic><topic>Humans</topic><topic>Infant</topic><topic>Kartagener syndrome</topic><topic>Kartagener Syndrome - diagnostic imaging</topic><topic>Kartagener Syndrome - physiopathology</topic><topic>Longitudinal Studies</topic><topic>Lung - diagnostic imaging</topic><topic>Lung - physiopathology</topic><topic>lung function test</topic><topic>Male</topic><topic>Medical genetics</topic><topic>Medical sciences</topic><topic>Pneumology</topic><topic>Respiratory Function Tests</topic><topic>Retrospective Studies</topic><topic>Tomography, X-Ray Computed</topic><toplevel>peer_reviewed</toplevel><toplevel>online_resources</toplevel><creatorcontrib>Magnin, Marie Lémery</creatorcontrib><creatorcontrib>Cros, Pierrick</creatorcontrib><creatorcontrib>Beydon, Nicole</creatorcontrib><creatorcontrib>Mahloul, Malika</creatorcontrib><creatorcontrib>Tamalet, Aline</creatorcontrib><creatorcontrib>Escudier, Estelle</creatorcontrib><creatorcontrib>Clément, Annick</creatorcontrib><creatorcontrib>Le Pointe, Hubert Ducou</creatorcontrib><creatorcontrib>Blanchon, Sylvain</creatorcontrib><collection>Istex</collection><collection>Pascal-Francis</collection><collection>Medline</collection><collection>MEDLINE</collection><collection>MEDLINE (Ovid)</collection><collection>MEDLINE</collection><collection>MEDLINE</collection><collection>PubMed</collection><collection>CrossRef</collection><collection>MEDLINE - Academic</collection><jtitle>Pediatric pulmonology</jtitle></facets><delivery><delcategory>Remote Search Resource</delcategory><fulltext>fulltext</fulltext></delivery><addata><au>Magnin, Marie Lémery</au><au>Cros, Pierrick</au><au>Beydon, Nicole</au><au>Mahloul, Malika</au><au>Tamalet, Aline</au><au>Escudier, Estelle</au><au>Clément, Annick</au><au>Le Pointe, Hubert Ducou</au><au>Blanchon, Sylvain</au><format>journal</format><genre>article</genre><ristype>JOUR</ristype><atitle>Longitudinal lung function and structural changes in children with primary ciliary dyskinesia</atitle><jtitle>Pediatric pulmonology</jtitle><addtitle>Pediatr. Pulmonol</addtitle><date>2012-08</date><risdate>2012</risdate><volume>47</volume><issue>8</issue><spage>816</spage><epage>825</epage><pages>816-825</pages><issn>8755-6863</issn><eissn>1099-0496</eissn><coden>PEPUES</coden><abstract>Background and Objectives
Functional and structural lung evaluations are part of the follow‐up of patients with primary ciliary dyskinesia (PCD). We aimed to evaluate transversal and longitudinal relationships between lung function test (LFT) and chest computed tomography (CT) in children with PCD, in stable clinical condition.
Materials and Methods
Data from children followed in the French National Center were retrospectively collected. Inclusion criteria were (i) definitive diagnosis of PCD, (ii) age less than 15 years at the beginning of follow‐up, (iii) at least 8 years of follow‐up, (iv) at least two couples of concurrent CT and LFT available in a phase of clinical stability of the lung disease without modification of the treatment regimen in the last 4 weeks. Twenty children (median age at entry 4.6 years, median follow‐up 15.4 years) were included. Concurrent LFT (blood gas and spirometry) and CT (score) results were recorded.
Results
LFT indices (PaO2 (n = 210), FVC, FEV1, FEF2575% (n = 195)) significantly decreased with age, and the mean annual decrease (z‐score (% predicted)) was −0.17 (−0.49%), −0.09 (−0.50%), −0.10 (−0.89%), and −0.07 (−1.73%), respectively. First CT (median age 8.7 years) revealed bronchiectasis (70%), mucous plugging (70%), peribronchial thickening (90%), parenchymal abnormalities (65%), and hyperinflation (50%). CT scores (n = 74) significantly increased with age, and was negatively correlated to PaO2, FVC, FEV1, and FEF2575% longitudinal changes.
Conclusion
In stable clinical condition, functional, and structural progressive impairments significantly correlated in children with PCD. Further prospective studies, including large populations of patients with various levels of disease severity, are needed to confirm whether lung function follow‐up can be used to adjust CT frequency and help at minimizing the radiation burden in children with a good life expectancy. Pediatr Pulmonol. 2012. 47:816–825. © 2012 Wiley Periodicals, Inc.</abstract><cop>Hoboken</cop><pub>Wiley Subscription Services, Inc., A Wiley Company</pub><pmid>22570319</pmid><doi>10.1002/ppul.22577</doi><tpages>10</tpages><oa>free_for_read</oa></addata></record> |
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| subjects | Adolescent Adult Biological and medical sciences blood gas Blood Gas Analysis Child Child, Preschool Complex syndromes computed tomography Disease Progression Female General aspects Humans Infant Kartagener syndrome Kartagener Syndrome - diagnostic imaging Kartagener Syndrome - physiopathology Longitudinal Studies Lung - diagnostic imaging Lung - physiopathology lung function test Male Medical genetics Medical sciences Pneumology Respiratory Function Tests Retrospective Studies Tomography, X-Ray Computed |
| title | Longitudinal lung function and structural changes in children with primary ciliary dyskinesia |
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