Primary Ciliary Dyskinesia Due to Microtubular Defects is Associated with Worse Lung Clearance Index
Purpose Primary ciliary dyskinesia (PCD) is characterised by repeated upper and lower respiratory tract infections, neutrophilic airway inflammation and obstructive airway disease. Different ultrastructural ciliary defects may affect lung function decline to different degrees. Lung clearance index (...
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| Veröffentlicht in: | Lung 2018-04, Vol.196 (2), p.231-238 |
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| creator | Irving, S. Dixon, M. Fassad, M. R. Frost, E. Hayward, J. Kilpin, K. Ollosson, S. Onoufriadis, A. Patel, M. P. Scully, J. Carr, S. B. Mitchison, H. M. Loebinger, M. R. Hogg, C. Shoemark, A. Bush, A. |
| description | Purpose
Primary ciliary dyskinesia (PCD) is characterised by repeated upper and lower respiratory tract infections, neutrophilic airway inflammation and obstructive airway disease. Different ultrastructural ciliary defects may affect lung function decline to different degrees. Lung clearance index (LCI) is a marker of ventilation inhomogeneity that is raised in some but not all patients with PCD. We hypothesised that PCD patients with microtubular defects would have worse (higher) LCI than other PCD patients.
Methods
Spirometry and LCI were measured in 69 stable patients with PCD. Age at testing, age at diagnosis, ethnicity, ciliary ultrastructure, genetic screening result and any growth of
Pseudomonas aeruginosa
was recorded.
Results
Lung clearance index was more abnormal in PCD patients with microtubular defects (median 10.24) than those with dynein arm defects (median 8.3,
p
= 0.004) or normal ultrastructure (median 7.63,
p
= 0.0004). Age is correlated with LCI, with older patients having worse LCI values (
p
= 0.03,
r
= 0.3).
Conclusion
This study shows that cilia microtubular defects are associated with worse LCI in PCD than dynein arm defects or normal ultrastructure. The patient’s age at testing is also associated with a higher LCI. Patients at greater risk of obstructive lung disease should be considered for more aggressive management. Differences between patient groups may potentially open avenues for novel treatments. |
| doi_str_mv | 10.1007/s00408-018-0086-x |
| format | Article |
| fullrecord | <record><control><sourceid>gale_pubme</sourceid><recordid>TN_cdi_pubmedcentral_primary_oai_pubmedcentral_nih_gov_5854730</recordid><sourceformat>XML</sourceformat><sourcesystem>PC</sourcesystem><galeid>A531222629</galeid><sourcerecordid>A531222629</sourcerecordid><originalsourceid>FETCH-LOGICAL-c606t-5d6ddddd074a09af61f0c34c474d3ccd28a20d7f6337b824939528668aa023c23</originalsourceid><addsrcrecordid>eNp1kl1v0zAUhi0EYl3hB3CDLCGh3WT4K05yg1S1fEwqggsQl5brnKQeqb3ZCXT_HkcZo0XFlmXJ5zmv7XNehF5QckkJKd5EQgQpM0LTIqXM9o_QjArOMlrk5DGaES5oxhJzhs5jvCaEFpLmT9EZq7gsiWAzVH8JdqfDHV7azo776i7-sA6i1Xg1AO49_mRN8P2wGTod8AoaMH3ENuJFjN5Y3UONf9l-i7_7EAGvB9fiZQc6aGcAX7ka9s_Qk0Z3EZ7f73P07f27r8uP2frzh6vlYp0ZSWSf5bWsx0EKoUmlG0kbYrgwohA1N6ZmpWakLhrJebEpmah4lbNSylJrwrhhfI7eTro3w2YHtQHXB92pm-mLymurjiPOblXrf6q8zEXBSRK4uBcI_naA2KudjQa6TjvwQ1S0qigtZSFG9NU_6LUfgkvfGykuUulz9pdqdQfKusane80oqhY5p4wxmXoxR9kJqgUH6ZHeQWPT8RF_eYJPs4adNScTXh8kbEF3_Tb6buitd_EYpBOYWh5jgOaheJSo0XNq8pxKnlOj59Q-5bw8rPpDxh-TJYBNQEwh10I4KNV_VX8DYiTftA</addsrcrecordid><sourcetype>Open Access Repository</sourcetype><iscdi>true</iscdi><recordtype>article</recordtype><pqid>1993434152</pqid></control><display><type>article</type><title>Primary Ciliary Dyskinesia Due to Microtubular Defects is Associated with Worse Lung Clearance Index</title><source>MEDLINE</source><source>Springer Nature - Complete Springer Journals</source><creator>Irving, S. ; Dixon, M. ; Fassad, M. R. ; Frost, E. ; Hayward, J. ; Kilpin, K. ; Ollosson, S. ; Onoufriadis, A. ; Patel, M. P. ; Scully, J. ; Carr, S. B. ; Mitchison, H. M. ; Loebinger, M. R. ; Hogg, C. ; Shoemark, A. ; Bush, A.</creator><creatorcontrib>Irving, S. ; Dixon, M. ; Fassad, M. R. ; Frost, E. ; Hayward, J. ; Kilpin, K. ; Ollosson, S. ; Onoufriadis, A. ; Patel, M. P. ; Scully, J. ; Carr, S. B. ; Mitchison, H. M. ; Loebinger, M. R. ; Hogg, C. ; Shoemark, A. ; Bush, A.</creatorcontrib><description>Purpose
Primary ciliary dyskinesia (PCD) is characterised by repeated upper and lower respiratory tract infections, neutrophilic airway inflammation and obstructive airway disease. Different ultrastructural ciliary defects may affect lung function decline to different degrees. Lung clearance index (LCI) is a marker of ventilation inhomogeneity that is raised in some but not all patients with PCD. We hypothesised that PCD patients with microtubular defects would have worse (higher) LCI than other PCD patients.
Methods
Spirometry and LCI were measured in 69 stable patients with PCD. Age at testing, age at diagnosis, ethnicity, ciliary ultrastructure, genetic screening result and any growth of
Pseudomonas aeruginosa
was recorded.
Results
Lung clearance index was more abnormal in PCD patients with microtubular defects (median 10.24) than those with dynein arm defects (median 8.3,
p
= 0.004) or normal ultrastructure (median 7.63,
p
= 0.0004). Age is correlated with LCI, with older patients having worse LCI values (
p
= 0.03,
r
= 0.3).
Conclusion
This study shows that cilia microtubular defects are associated with worse LCI in PCD than dynein arm defects or normal ultrastructure. The patient’s age at testing is also associated with a higher LCI. Patients at greater risk of obstructive lung disease should be considered for more aggressive management. Differences between patient groups may potentially open avenues for novel treatments.</description><identifier>ISSN: 0341-2040</identifier><identifier>EISSN: 1432-1750</identifier><identifier>DOI: 10.1007/s00408-018-0086-x</identifier><identifier>PMID: 29368042</identifier><language>eng</language><publisher>New York: Springer US</publisher><subject>Adolescent ; Adult ; Age ; Age Factors ; Care and treatment ; Child ; Child, Preschool ; Cilia ; Cilia - ultrastructure ; Ciliary Motility Disorders - complications ; Ciliary Motility Disorders - genetics ; Ciliary Motility Disorders - pathology ; Ciliary Motility Disorders - physiopathology ; Complications and side effects ; Defects ; Diagnosis ; Disease control ; Dynein ; Female ; Forced Expiratory Volume ; Genetic screening ; Health risk assessment ; Health risks ; Humans ; Infant ; Infant, Newborn ; Inhomogeneity ; Leukocytes (neutrophilic) ; Lung - physiopathology ; Lung - ultrastructure ; Lung diseases ; Lung Diseases - etiology ; Lung Diseases - pathology ; Lung Diseases - physiopathology ; Male ; Maximal Midexpiratory Flow Rate ; Mechanical ventilation ; Medicine ; Medicine & Public Health ; Microscopy, Electron, Transmission ; Microtubules - ultrastructure ; Minority & ethnic groups ; Movement disorders ; Mucociliary Clearance ; Obstructive lung disease ; Patients ; Pneumology/Respiratory System ; Primary ciliary dyskinesia ; Pseudomonas aeruginosa ; Respiratory function ; Respiratory Physiology ; Respiratory tract ; Respiratory tract diseases ; Respiratory tract infections ; Risk Factors ; Risk management ; Spirometry ; Ultrastructure ; Ventilation ; Young Adult</subject><ispartof>Lung, 2018-04, Vol.196 (2), p.231-238</ispartof><rights>The Author(s) 2018</rights><rights>COPYRIGHT 2018 Springer</rights><rights>Lung is a copyright of Springer, (2018). All Rights Reserved.</rights><lds50>peer_reviewed</lds50><oa>free_for_read</oa><woscitedreferencessubscribed>false</woscitedreferencessubscribed><citedby>FETCH-LOGICAL-c606t-5d6ddddd074a09af61f0c34c474d3ccd28a20d7f6337b824939528668aa023c23</citedby><cites>FETCH-LOGICAL-c606t-5d6ddddd074a09af61f0c34c474d3ccd28a20d7f6337b824939528668aa023c23</cites><orcidid>0000-0003-1861-592X</orcidid></display><links><openurl>$$Topenurl_article</openurl><openurlfulltext>$$Topenurlfull_article</openurlfulltext><thumbnail>$$Tsyndetics_thumb_exl</thumbnail><linktopdf>$$Uhttps://link.springer.com/content/pdf/10.1007/s00408-018-0086-x$$EPDF$$P50$$Gspringer$$Hfree_for_read</linktopdf><linktohtml>$$Uhttps://link.springer.com/10.1007/s00408-018-0086-x$$EHTML$$P50$$Gspringer$$Hfree_for_read</linktohtml><link.rule.ids>230,314,776,780,881,27901,27902,41464,42533,51294</link.rule.ids><backlink>$$Uhttps://www.ncbi.nlm.nih.gov/pubmed/29368042$$D View this record in MEDLINE/PubMed$$Hfree_for_read</backlink></links><search><creatorcontrib>Irving, S.</creatorcontrib><creatorcontrib>Dixon, M.</creatorcontrib><creatorcontrib>Fassad, M. R.</creatorcontrib><creatorcontrib>Frost, E.</creatorcontrib><creatorcontrib>Hayward, J.</creatorcontrib><creatorcontrib>Kilpin, K.</creatorcontrib><creatorcontrib>Ollosson, S.</creatorcontrib><creatorcontrib>Onoufriadis, A.</creatorcontrib><creatorcontrib>Patel, M. P.</creatorcontrib><creatorcontrib>Scully, J.</creatorcontrib><creatorcontrib>Carr, S. B.</creatorcontrib><creatorcontrib>Mitchison, H. M.</creatorcontrib><creatorcontrib>Loebinger, M. R.</creatorcontrib><creatorcontrib>Hogg, C.</creatorcontrib><creatorcontrib>Shoemark, A.</creatorcontrib><creatorcontrib>Bush, A.</creatorcontrib><title>Primary Ciliary Dyskinesia Due to Microtubular Defects is Associated with Worse Lung Clearance Index</title><title>Lung</title><addtitle>Lung</addtitle><addtitle>Lung</addtitle><description>Purpose
Primary ciliary dyskinesia (PCD) is characterised by repeated upper and lower respiratory tract infections, neutrophilic airway inflammation and obstructive airway disease. Different ultrastructural ciliary defects may affect lung function decline to different degrees. Lung clearance index (LCI) is a marker of ventilation inhomogeneity that is raised in some but not all patients with PCD. We hypothesised that PCD patients with microtubular defects would have worse (higher) LCI than other PCD patients.
Methods
Spirometry and LCI were measured in 69 stable patients with PCD. Age at testing, age at diagnosis, ethnicity, ciliary ultrastructure, genetic screening result and any growth of
Pseudomonas aeruginosa
was recorded.
Results
Lung clearance index was more abnormal in PCD patients with microtubular defects (median 10.24) than those with dynein arm defects (median 8.3,
p
= 0.004) or normal ultrastructure (median 7.63,
p
= 0.0004). Age is correlated with LCI, with older patients having worse LCI values (
p
= 0.03,
r
= 0.3).
Conclusion
This study shows that cilia microtubular defects are associated with worse LCI in PCD than dynein arm defects or normal ultrastructure. The patient’s age at testing is also associated with a higher LCI. Patients at greater risk of obstructive lung disease should be considered for more aggressive management. Differences between patient groups may potentially open avenues for novel treatments.</description><subject>Adolescent</subject><subject>Adult</subject><subject>Age</subject><subject>Age Factors</subject><subject>Care and treatment</subject><subject>Child</subject><subject>Child, Preschool</subject><subject>Cilia</subject><subject>Cilia - ultrastructure</subject><subject>Ciliary Motility Disorders - complications</subject><subject>Ciliary Motility Disorders - genetics</subject><subject>Ciliary Motility Disorders - pathology</subject><subject>Ciliary Motility Disorders - physiopathology</subject><subject>Complications and side effects</subject><subject>Defects</subject><subject>Diagnosis</subject><subject>Disease control</subject><subject>Dynein</subject><subject>Female</subject><subject>Forced Expiratory Volume</subject><subject>Genetic screening</subject><subject>Health risk assessment</subject><subject>Health risks</subject><subject>Humans</subject><subject>Infant</subject><subject>Infant, Newborn</subject><subject>Inhomogeneity</subject><subject>Leukocytes (neutrophilic)</subject><subject>Lung - physiopathology</subject><subject>Lung - ultrastructure</subject><subject>Lung diseases</subject><subject>Lung Diseases - etiology</subject><subject>Lung Diseases - pathology</subject><subject>Lung Diseases - physiopathology</subject><subject>Male</subject><subject>Maximal Midexpiratory Flow Rate</subject><subject>Mechanical ventilation</subject><subject>Medicine</subject><subject>Medicine & Public Health</subject><subject>Microscopy, Electron, Transmission</subject><subject>Microtubules - ultrastructure</subject><subject>Minority & ethnic groups</subject><subject>Movement disorders</subject><subject>Mucociliary Clearance</subject><subject>Obstructive lung disease</subject><subject>Patients</subject><subject>Pneumology/Respiratory System</subject><subject>Primary ciliary dyskinesia</subject><subject>Pseudomonas aeruginosa</subject><subject>Respiratory function</subject><subject>Respiratory Physiology</subject><subject>Respiratory tract</subject><subject>Respiratory tract diseases</subject><subject>Respiratory tract infections</subject><subject>Risk Factors</subject><subject>Risk management</subject><subject>Spirometry</subject><subject>Ultrastructure</subject><subject>Ventilation</subject><subject>Young Adult</subject><issn>0341-2040</issn><issn>1432-1750</issn><fulltext>true</fulltext><rsrctype>article</rsrctype><creationdate>2018</creationdate><recordtype>article</recordtype><sourceid>C6C</sourceid><sourceid>EIF</sourceid><sourceid>8G5</sourceid><sourceid>BENPR</sourceid><sourceid>GUQSH</sourceid><sourceid>M2O</sourceid><recordid>eNp1kl1v0zAUhi0EYl3hB3CDLCGh3WT4K05yg1S1fEwqggsQl5brnKQeqb3ZCXT_HkcZo0XFlmXJ5zmv7XNehF5QckkJKd5EQgQpM0LTIqXM9o_QjArOMlrk5DGaES5oxhJzhs5jvCaEFpLmT9EZq7gsiWAzVH8JdqfDHV7azo776i7-sA6i1Xg1AO49_mRN8P2wGTod8AoaMH3ENuJFjN5Y3UONf9l-i7_7EAGvB9fiZQc6aGcAX7ka9s_Qk0Z3EZ7f73P07f27r8uP2frzh6vlYp0ZSWSf5bWsx0EKoUmlG0kbYrgwohA1N6ZmpWakLhrJebEpmah4lbNSylJrwrhhfI7eTro3w2YHtQHXB92pm-mLymurjiPOblXrf6q8zEXBSRK4uBcI_naA2KudjQa6TjvwQ1S0qigtZSFG9NU_6LUfgkvfGykuUulz9pdqdQfKusane80oqhY5p4wxmXoxR9kJqgUH6ZHeQWPT8RF_eYJPs4adNScTXh8kbEF3_Tb6buitd_EYpBOYWh5jgOaheJSo0XNq8pxKnlOj59Q-5bw8rPpDxh-TJYBNQEwh10I4KNV_VX8DYiTftA</recordid><startdate>20180401</startdate><enddate>20180401</enddate><creator>Irving, S.</creator><creator>Dixon, M.</creator><creator>Fassad, M. R.</creator><creator>Frost, E.</creator><creator>Hayward, J.</creator><creator>Kilpin, K.</creator><creator>Ollosson, S.</creator><creator>Onoufriadis, A.</creator><creator>Patel, M. P.</creator><creator>Scully, J.</creator><creator>Carr, S. B.</creator><creator>Mitchison, H. M.</creator><creator>Loebinger, M. R.</creator><creator>Hogg, C.</creator><creator>Shoemark, A.</creator><creator>Bush, A.</creator><general>Springer US</general><general>Springer</general><general>Springer Nature B.V</general><scope>C6C</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>3V.</scope><scope>7QL</scope><scope>7RV</scope><scope>7T7</scope><scope>7U9</scope><scope>7X7</scope><scope>7XB</scope><scope>88E</scope><scope>8AO</scope><scope>8FD</scope><scope>8FI</scope><scope>8FJ</scope><scope>8FK</scope><scope>8G5</scope><scope>ABUWG</scope><scope>AFKRA</scope><scope>AZQEC</scope><scope>BENPR</scope><scope>C1K</scope><scope>CCPQU</scope><scope>DWQXO</scope><scope>FR3</scope><scope>FYUFA</scope><scope>GHDGH</scope><scope>GNUQQ</scope><scope>GUQSH</scope><scope>H94</scope><scope>K9-</scope><scope>K9.</scope><scope>M0R</scope><scope>M0S</scope><scope>M1P</scope><scope>M2O</scope><scope>M7N</scope><scope>MBDVC</scope><scope>NAPCQ</scope><scope>P64</scope><scope>PQEST</scope><scope>PQQKQ</scope><scope>PQUKI</scope><scope>PRINS</scope><scope>Q9U</scope><scope>7X8</scope><scope>5PM</scope><orcidid>https://orcid.org/0000-0003-1861-592X</orcidid></search><sort><creationdate>20180401</creationdate><title>Primary Ciliary Dyskinesia Due to Microtubular Defects is Associated with Worse Lung Clearance Index</title><author>Irving, S. ; Dixon, M. ; Fassad, M. R. ; Frost, E. ; Hayward, J. ; Kilpin, K. ; Ollosson, S. ; Onoufriadis, A. ; Patel, M. P. ; Scully, J. ; Carr, S. B. ; Mitchison, H. M. ; Loebinger, M. R. ; Hogg, C. ; Shoemark, A. ; Bush, A.</author></sort><facets><frbrtype>5</frbrtype><frbrgroupid>cdi_FETCH-LOGICAL-c606t-5d6ddddd074a09af61f0c34c474d3ccd28a20d7f6337b824939528668aa023c23</frbrgroupid><rsrctype>articles</rsrctype><prefilter>articles</prefilter><language>eng</language><creationdate>2018</creationdate><topic>Adolescent</topic><topic>Adult</topic><topic>Age</topic><topic>Age Factors</topic><topic>Care and treatment</topic><topic>Child</topic><topic>Child, Preschool</topic><topic>Cilia</topic><topic>Cilia - ultrastructure</topic><topic>Ciliary Motility Disorders - complications</topic><topic>Ciliary Motility Disorders - genetics</topic><topic>Ciliary Motility Disorders - pathology</topic><topic>Ciliary Motility Disorders - physiopathology</topic><topic>Complications and side effects</topic><topic>Defects</topic><topic>Diagnosis</topic><topic>Disease control</topic><topic>Dynein</topic><topic>Female</topic><topic>Forced Expiratory Volume</topic><topic>Genetic screening</topic><topic>Health risk assessment</topic><topic>Health risks</topic><topic>Humans</topic><topic>Infant</topic><topic>Infant, Newborn</topic><topic>Inhomogeneity</topic><topic>Leukocytes (neutrophilic)</topic><topic>Lung - physiopathology</topic><topic>Lung - ultrastructure</topic><topic>Lung diseases</topic><topic>Lung Diseases - etiology</topic><topic>Lung Diseases - pathology</topic><topic>Lung Diseases - physiopathology</topic><topic>Male</topic><topic>Maximal Midexpiratory Flow Rate</topic><topic>Mechanical ventilation</topic><topic>Medicine</topic><topic>Medicine & Public Health</topic><topic>Microscopy, Electron, Transmission</topic><topic>Microtubules - ultrastructure</topic><topic>Minority & ethnic groups</topic><topic>Movement disorders</topic><topic>Mucociliary Clearance</topic><topic>Obstructive lung disease</topic><topic>Patients</topic><topic>Pneumology/Respiratory System</topic><topic>Primary ciliary dyskinesia</topic><topic>Pseudomonas aeruginosa</topic><topic>Respiratory function</topic><topic>Respiratory Physiology</topic><topic>Respiratory tract</topic><topic>Respiratory tract diseases</topic><topic>Respiratory tract infections</topic><topic>Risk Factors</topic><topic>Risk management</topic><topic>Spirometry</topic><topic>Ultrastructure</topic><topic>Ventilation</topic><topic>Young Adult</topic><toplevel>peer_reviewed</toplevel><toplevel>online_resources</toplevel><creatorcontrib>Irving, S.</creatorcontrib><creatorcontrib>Dixon, M.</creatorcontrib><creatorcontrib>Fassad, M. R.</creatorcontrib><creatorcontrib>Frost, E.</creatorcontrib><creatorcontrib>Hayward, J.</creatorcontrib><creatorcontrib>Kilpin, K.</creatorcontrib><creatorcontrib>Ollosson, S.</creatorcontrib><creatorcontrib>Onoufriadis, A.</creatorcontrib><creatorcontrib>Patel, M. P.</creatorcontrib><creatorcontrib>Scully, J.</creatorcontrib><creatorcontrib>Carr, S. B.</creatorcontrib><creatorcontrib>Mitchison, H. M.</creatorcontrib><creatorcontrib>Loebinger, M. R.</creatorcontrib><creatorcontrib>Hogg, C.</creatorcontrib><creatorcontrib>Shoemark, A.</creatorcontrib><creatorcontrib>Bush, A.</creatorcontrib><collection>Springer Nature OA Free Journals</collection><collection>Medline</collection><collection>MEDLINE</collection><collection>MEDLINE (Ovid)</collection><collection>MEDLINE</collection><collection>MEDLINE</collection><collection>PubMed</collection><collection>CrossRef</collection><collection>ProQuest Central (Corporate)</collection><collection>Bacteriology Abstracts (Microbiology B)</collection><collection>Nursing & Allied Health Database</collection><collection>Industrial and Applied Microbiology Abstracts (Microbiology A)</collection><collection>Virology and AIDS Abstracts</collection><collection>Health & Medical Collection</collection><collection>ProQuest Central (purchase pre-March 2016)</collection><collection>Medical Database (Alumni Edition)</collection><collection>ProQuest Pharma Collection</collection><collection>Technology Research Database</collection><collection>Hospital Premium Collection</collection><collection>Hospital Premium Collection (Alumni Edition)</collection><collection>ProQuest Central (Alumni) (purchase pre-March 2016)</collection><collection>Research Library (Alumni Edition)</collection><collection>ProQuest Central (Alumni Edition)</collection><collection>ProQuest Central UK/Ireland</collection><collection>ProQuest Central Essentials</collection><collection>ProQuest Central</collection><collection>Environmental Sciences and Pollution Management</collection><collection>ProQuest One Community College</collection><collection>ProQuest Central Korea</collection><collection>Engineering Research Database</collection><collection>Health Research Premium Collection</collection><collection>Health Research Premium Collection (Alumni)</collection><collection>ProQuest Central Student</collection><collection>Research Library Prep</collection><collection>AIDS and Cancer Research Abstracts</collection><collection>Consumer Health Database (Alumni Edition)</collection><collection>ProQuest Health & Medical Complete (Alumni)</collection><collection>Consumer Health Database</collection><collection>Health & Medical Collection (Alumni Edition)</collection><collection>Medical Database</collection><collection>Research Library</collection><collection>Algology Mycology and Protozoology Abstracts (Microbiology C)</collection><collection>Research Library (Corporate)</collection><collection>Nursing & Allied Health Premium</collection><collection>Biotechnology and BioEngineering Abstracts</collection><collection>ProQuest One Academic Eastern Edition (DO NOT USE)</collection><collection>ProQuest One Academic</collection><collection>ProQuest One Academic UKI Edition</collection><collection>ProQuest Central China</collection><collection>ProQuest Central Basic</collection><collection>MEDLINE - Academic</collection><collection>PubMed Central (Full Participant titles)</collection><jtitle>Lung</jtitle></facets><delivery><delcategory>Remote Search Resource</delcategory><fulltext>fulltext</fulltext></delivery><addata><au>Irving, S.</au><au>Dixon, M.</au><au>Fassad, M. R.</au><au>Frost, E.</au><au>Hayward, J.</au><au>Kilpin, K.</au><au>Ollosson, S.</au><au>Onoufriadis, A.</au><au>Patel, M. P.</au><au>Scully, J.</au><au>Carr, S. B.</au><au>Mitchison, H. M.</au><au>Loebinger, M. R.</au><au>Hogg, C.</au><au>Shoemark, A.</au><au>Bush, A.</au><format>journal</format><genre>article</genre><ristype>JOUR</ristype><atitle>Primary Ciliary Dyskinesia Due to Microtubular Defects is Associated with Worse Lung Clearance Index</atitle><jtitle>Lung</jtitle><stitle>Lung</stitle><addtitle>Lung</addtitle><date>2018-04-01</date><risdate>2018</risdate><volume>196</volume><issue>2</issue><spage>231</spage><epage>238</epage><pages>231-238</pages><issn>0341-2040</issn><eissn>1432-1750</eissn><abstract>Purpose
Primary ciliary dyskinesia (PCD) is characterised by repeated upper and lower respiratory tract infections, neutrophilic airway inflammation and obstructive airway disease. Different ultrastructural ciliary defects may affect lung function decline to different degrees. Lung clearance index (LCI) is a marker of ventilation inhomogeneity that is raised in some but not all patients with PCD. We hypothesised that PCD patients with microtubular defects would have worse (higher) LCI than other PCD patients.
Methods
Spirometry and LCI were measured in 69 stable patients with PCD. Age at testing, age at diagnosis, ethnicity, ciliary ultrastructure, genetic screening result and any growth of
Pseudomonas aeruginosa
was recorded.
Results
Lung clearance index was more abnormal in PCD patients with microtubular defects (median 10.24) than those with dynein arm defects (median 8.3,
p
= 0.004) or normal ultrastructure (median 7.63,
p
= 0.0004). Age is correlated with LCI, with older patients having worse LCI values (
p
= 0.03,
r
= 0.3).
Conclusion
This study shows that cilia microtubular defects are associated with worse LCI in PCD than dynein arm defects or normal ultrastructure. The patient’s age at testing is also associated with a higher LCI. Patients at greater risk of obstructive lung disease should be considered for more aggressive management. Differences between patient groups may potentially open avenues for novel treatments.</abstract><cop>New York</cop><pub>Springer US</pub><pmid>29368042</pmid><doi>10.1007/s00408-018-0086-x</doi><tpages>8</tpages><orcidid>https://orcid.org/0000-0003-1861-592X</orcidid><oa>free_for_read</oa></addata></record> |
| fulltext | fulltext |
| identifier | ISSN: 0341-2040 |
| ispartof | Lung, 2018-04, Vol.196 (2), p.231-238 |
| issn | 0341-2040 1432-1750 |
| language | eng |
| recordid | cdi_pubmedcentral_primary_oai_pubmedcentral_nih_gov_5854730 |
| source | MEDLINE; Springer Nature - Complete Springer Journals |
| subjects | Adolescent Adult Age Age Factors Care and treatment Child Child, Preschool Cilia Cilia - ultrastructure Ciliary Motility Disorders - complications Ciliary Motility Disorders - genetics Ciliary Motility Disorders - pathology Ciliary Motility Disorders - physiopathology Complications and side effects Defects Diagnosis Disease control Dynein Female Forced Expiratory Volume Genetic screening Health risk assessment Health risks Humans Infant Infant, Newborn Inhomogeneity Leukocytes (neutrophilic) Lung - physiopathology Lung - ultrastructure Lung diseases Lung Diseases - etiology Lung Diseases - pathology Lung Diseases - physiopathology Male Maximal Midexpiratory Flow Rate Mechanical ventilation Medicine Medicine & Public Health Microscopy, Electron, Transmission Microtubules - ultrastructure Minority & ethnic groups Movement disorders Mucociliary Clearance Obstructive lung disease Patients Pneumology/Respiratory System Primary ciliary dyskinesia Pseudomonas aeruginosa Respiratory function Respiratory Physiology Respiratory tract Respiratory tract diseases Respiratory tract infections Risk Factors Risk management Spirometry Ultrastructure Ventilation Young Adult |
| title | Primary Ciliary Dyskinesia Due to Microtubular Defects is Associated with Worse Lung Clearance Index |
| url | https://sfx.bib-bvb.de/sfx_tum?ctx_ver=Z39.88-2004&ctx_enc=info:ofi/enc:UTF-8&ctx_tim=2025-02-12T23%3A59%3A00IST&url_ver=Z39.88-2004&url_ctx_fmt=infofi/fmt:kev:mtx:ctx&rfr_id=info:sid/primo.exlibrisgroup.com:primo3-Article-gale_pubme&rft_val_fmt=info:ofi/fmt:kev:mtx:journal&rft.genre=article&rft.atitle=Primary%20Ciliary%20Dyskinesia%20Due%20to%20Microtubular%20Defects%20is%20Associated%20with%20Worse%20Lung%20Clearance%20Index&rft.jtitle=Lung&rft.au=Irving,%20S.&rft.date=2018-04-01&rft.volume=196&rft.issue=2&rft.spage=231&rft.epage=238&rft.pages=231-238&rft.issn=0341-2040&rft.eissn=1432-1750&rft_id=info:doi/10.1007/s00408-018-0086-x&rft_dat=%3Cgale_pubme%3EA531222629%3C/gale_pubme%3E%3Curl%3E%3C/url%3E&disable_directlink=true&sfx.directlink=off&sfx.report_link=0&rft_id=info:oai/&rft_pqid=1993434152&rft_id=info:pmid/29368042&rft_galeid=A531222629&rfr_iscdi=true |