Sensitive structural and functional measurements and 1-year pulmonary outcomes in pediatric cystic fibrosis
•LCI2.5, 129Xe VDP, and UTE more sensitive to pediatric CF disease than spirometry.•1H MRI suggests increased odds for airway damage in lobes with mucus plugging.•Most spirometry measures correlated with consolidations and ground glass.•LCI2.5 and 129Xe VDP correlated with mucus plugging.•Increased...
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| Veröffentlicht in: | Journal of cystic fibrosis 2021-05, Vol.20 (3), p.533-539 |
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| creator | Willmering, Matthew M. Roach, David J. Kramer, Elizabeth L. Walkup, Laura L. Cleveland, Zackary I. Woods, Jason C. |
| description | •LCI2.5, 129Xe VDP, and UTE more sensitive to pediatric CF disease than spirometry.•1H MRI suggests increased odds for airway damage in lobes with mucus plugging.•Most spirometry measures correlated with consolidations and ground glass.•LCI2.5 and 129Xe VDP correlated with mucus plugging.•Increased exacerbations most strongly correlated with mucus plugs and airway damage.
Two functional measurements (multiple breath washout [MBW] and hyperpolarized 129Xe ventilation magnetic resonance imaging [129Xe MRI]) have been shown to be more sensitive to cystic fibrosis (CF) lung obstruction than traditional spirometry. However, functional techniques may be sensitive to different underlying structural abnormalities. The purpose of this study was to determine relationships between these functional markers, their pathophysiology, and 1-year clinical outcomes.
Spirometry, MBW, 129Xe MRI, and ultrashort echo-time (UTE) MRI were obtained in a same-day assessment of 27 pediatric CF patients (ages 11.5±5.0) who had not begun CFTR modulator therapies. UTE MRI was scored for structural abnormalities and functional metrics obtained via spirometry, MBW and 129Xe MRI. 1-year outcomes (ΔFEV1 and pulmonary exacerbations), during which ≈50% initiated modulator therapy, were obtained from the electronic medical record.
MBW, 129Xe MRI, and UTE MRI detected clinically significant disease in more subjects (>78%) compared to spirometry ( |
| doi_str_mv | 10.1016/j.jcf.2020.11.019 |
| format | Article |
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Two functional measurements (multiple breath washout [MBW] and hyperpolarized 129Xe ventilation magnetic resonance imaging [129Xe MRI]) have been shown to be more sensitive to cystic fibrosis (CF) lung obstruction than traditional spirometry. However, functional techniques may be sensitive to different underlying structural abnormalities. The purpose of this study was to determine relationships between these functional markers, their pathophysiology, and 1-year clinical outcomes.
Spirometry, MBW, 129Xe MRI, and ultrashort echo-time (UTE) MRI were obtained in a same-day assessment of 27 pediatric CF patients (ages 11.5±5.0) who had not begun CFTR modulator therapies. UTE MRI was scored for structural abnormalities and functional metrics obtained via spirometry, MBW and 129Xe MRI. 1-year outcomes (ΔFEV1 and pulmonary exacerbations), during which ≈50% initiated modulator therapy, were obtained from the electronic medical record.
MBW, 129Xe MRI, and UTE MRI detected clinically significant disease in more subjects (>78%) compared to spirometry (<30%). UTE MRI suggests increased odds of bronchial changes when mucus plugging is present in the same lobe. MBW and 129Xe MRI correlated best with mucus plugging, while spirometry correlated best with consolidations. Bronchial abnormalities were associated with future pulmonary exacerbations.
MBW, 129Xe MRI, and UTE MRI are more sensitive for detection of pediatric CF lung disease when compared to spirometry. MBW and 129Xe MRI correlated with structural abnormalities which occur in early CF disease, suggesting MBW and 129Xe MRI are valuable tools in mild CF lung disease that can guide clinical decision making.</description><identifier>ISSN: 1569-1993</identifier><identifier>EISSN: 1873-5010</identifier><identifier>DOI: 10.1016/j.jcf.2020.11.019</identifier><identifier>PMID: 33288474</identifier><language>eng</language><publisher>AMSTERDAM: Elsevier B.V</publisher><subject>Child ; Cystic Fibrosis - diagnostic imaging ; Cystic Fibrosis - physiopathology ; Female ; Function ; Humans ; Life Sciences & Biomedicine ; Magnetic Resonance Imaging - methods ; Male ; MRI ; Multiple breath washout ; Prospective Studies ; Respiratory System ; Science & Technology ; Sensitivity and Specificity ; Spirometry ; Structure ; Xenon ; Xenon Isotopes</subject><ispartof>Journal of cystic fibrosis, 2021-05, Vol.20 (3), p.533-539</ispartof><rights>2020 European Cystic Fibrosis Society</rights><rights>Copyright © 2020 European Cystic Fibrosis Society. Published by Elsevier B.V. All rights reserved.</rights><lds50>peer_reviewed</lds50><oa>free_for_read</oa><woscitedreferencessubscribed>true</woscitedreferencessubscribed><woscitedreferencescount>12</woscitedreferencescount><woscitedreferencesoriginalsourcerecordid>wos000659128900030</woscitedreferencesoriginalsourcerecordid><citedby>FETCH-LOGICAL-c451t-d165efbd7778bc4afd1f758ea45447bf2dda05fd333acac8acf1f0a9e9495e713</citedby><cites>FETCH-LOGICAL-c451t-d165efbd7778bc4afd1f758ea45447bf2dda05fd333acac8acf1f0a9e9495e713</cites><orcidid>0000-0001-6195-9061 ; 0000-0002-3278-2161</orcidid></display><links><openurl>$$Topenurl_article</openurl><openurlfulltext>$$Topenurlfull_article</openurlfulltext><thumbnail>$$Tsyndetics_thumb_exl</thumbnail><linktohtml>$$Uhttps://dx.doi.org/10.1016/j.jcf.2020.11.019$$EHTML$$P50$$Gelsevier$$H</linktohtml><link.rule.ids>230,315,782,786,887,3554,27933,27934,39267,46004</link.rule.ids><backlink>$$Uhttps://www.ncbi.nlm.nih.gov/pubmed/33288474$$D View this record in MEDLINE/PubMed$$Hfree_for_read</backlink></links><search><creatorcontrib>Willmering, Matthew M.</creatorcontrib><creatorcontrib>Roach, David J.</creatorcontrib><creatorcontrib>Kramer, Elizabeth L.</creatorcontrib><creatorcontrib>Walkup, Laura L.</creatorcontrib><creatorcontrib>Cleveland, Zackary I.</creatorcontrib><creatorcontrib>Woods, Jason C.</creatorcontrib><title>Sensitive structural and functional measurements and 1-year pulmonary outcomes in pediatric cystic fibrosis</title><title>Journal of cystic fibrosis</title><addtitle>J CYST FIBROS</addtitle><addtitle>J Cyst Fibros</addtitle><description>•LCI2.5, 129Xe VDP, and UTE more sensitive to pediatric CF disease than spirometry.•1H MRI suggests increased odds for airway damage in lobes with mucus plugging.•Most spirometry measures correlated with consolidations and ground glass.•LCI2.5 and 129Xe VDP correlated with mucus plugging.•Increased exacerbations most strongly correlated with mucus plugs and airway damage.
Two functional measurements (multiple breath washout [MBW] and hyperpolarized 129Xe ventilation magnetic resonance imaging [129Xe MRI]) have been shown to be more sensitive to cystic fibrosis (CF) lung obstruction than traditional spirometry. However, functional techniques may be sensitive to different underlying structural abnormalities. The purpose of this study was to determine relationships between these functional markers, their pathophysiology, and 1-year clinical outcomes.
Spirometry, MBW, 129Xe MRI, and ultrashort echo-time (UTE) MRI were obtained in a same-day assessment of 27 pediatric CF patients (ages 11.5±5.0) who had not begun CFTR modulator therapies. UTE MRI was scored for structural abnormalities and functional metrics obtained via spirometry, MBW and 129Xe MRI. 1-year outcomes (ΔFEV1 and pulmonary exacerbations), during which ≈50% initiated modulator therapy, were obtained from the electronic medical record.
MBW, 129Xe MRI, and UTE MRI detected clinically significant disease in more subjects (>78%) compared to spirometry (<30%). UTE MRI suggests increased odds of bronchial changes when mucus plugging is present in the same lobe. MBW and 129Xe MRI correlated best with mucus plugging, while spirometry correlated best with consolidations. Bronchial abnormalities were associated with future pulmonary exacerbations.
MBW, 129Xe MRI, and UTE MRI are more sensitive for detection of pediatric CF lung disease when compared to spirometry. MBW and 129Xe MRI correlated with structural abnormalities which occur in early CF disease, suggesting MBW and 129Xe MRI are valuable tools in mild CF lung disease that can guide clinical decision making.</description><subject>Child</subject><subject>Cystic Fibrosis - diagnostic imaging</subject><subject>Cystic Fibrosis - physiopathology</subject><subject>Female</subject><subject>Function</subject><subject>Humans</subject><subject>Life Sciences & Biomedicine</subject><subject>Magnetic Resonance Imaging - methods</subject><subject>Male</subject><subject>MRI</subject><subject>Multiple breath washout</subject><subject>Prospective Studies</subject><subject>Respiratory System</subject><subject>Science & Technology</subject><subject>Sensitivity and Specificity</subject><subject>Spirometry</subject><subject>Structure</subject><subject>Xenon</subject><subject>Xenon Isotopes</subject><issn>1569-1993</issn><issn>1873-5010</issn><fulltext>true</fulltext><rsrctype>article</rsrctype><creationdate>2021</creationdate><recordtype>article</recordtype><sourceid>HGBXW</sourceid><sourceid>EIF</sourceid><recordid>eNqNkcuqFDEQhhtRPMejD-BGeilIj6lOupNGEGTwBgdcqOuQTiqacToZcxmZtzfjjINuxFVVUd9fF_6meQxkBQTG55vVRttVT_paw4rAdKe5BsFpNxAgd2s-jFMH00SvmgcpbQgBTri431xR2gvBOLtuvn1En1x2e2xTjkXnEtW2Vd60tnidXfC1XFClEnFBn9OvHnQHVLHdle1SgXhoQ8k6LJha59sdGqdydLrVh5RrsG6OIbn0sLln1Tbho3O8aT6_ef1p_a67_fD2_frVbafZALkzMA5oZ8M5F7NmyhqwfBCo2MAYn21vjCKDNZRSpZUWSluwRE04sWlADvSmeXmauyvzgkbXs-tTchfdUm-VQTn5d8e7r_JL2EsB_LiiDnh6HhDD94Ipy8Uljdut8hhKkj0bBWU9jKyicEJ1fTFFtJc1QOTRJLmR1SR5NEkCyGpS1Tz5876L4rcrFRAn4AfOwSbt0Gu8YISQcZigF1PNKFm7rI4-rUPxuUqf_b-00i9ONFY79g6jPCuMi6izNMH944-fWrXH5A</recordid><startdate>20210501</startdate><enddate>20210501</enddate><creator>Willmering, Matthew M.</creator><creator>Roach, David J.</creator><creator>Kramer, Elizabeth L.</creator><creator>Walkup, Laura L.</creator><creator>Cleveland, Zackary I.</creator><creator>Woods, Jason C.</creator><general>Elsevier B.V</general><general>Elsevier</general><scope>BLEPL</scope><scope>DTL</scope><scope>HGBXW</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><scope>5PM</scope><orcidid>https://orcid.org/0000-0001-6195-9061</orcidid><orcidid>https://orcid.org/0000-0002-3278-2161</orcidid></search><sort><creationdate>20210501</creationdate><title>Sensitive structural and functional measurements and 1-year pulmonary outcomes in pediatric cystic fibrosis</title><author>Willmering, Matthew M. ; Roach, David J. ; Kramer, Elizabeth L. ; Walkup, Laura L. ; Cleveland, Zackary I. ; Woods, Jason C.</author></sort><facets><frbrtype>5</frbrtype><frbrgroupid>cdi_FETCH-LOGICAL-c451t-d165efbd7778bc4afd1f758ea45447bf2dda05fd333acac8acf1f0a9e9495e713</frbrgroupid><rsrctype>articles</rsrctype><prefilter>articles</prefilter><language>eng</language><creationdate>2021</creationdate><topic>Child</topic><topic>Cystic Fibrosis - diagnostic imaging</topic><topic>Cystic Fibrosis - physiopathology</topic><topic>Female</topic><topic>Function</topic><topic>Humans</topic><topic>Life Sciences & Biomedicine</topic><topic>Magnetic Resonance Imaging - methods</topic><topic>Male</topic><topic>MRI</topic><topic>Multiple breath washout</topic><topic>Prospective Studies</topic><topic>Respiratory System</topic><topic>Science & Technology</topic><topic>Sensitivity and Specificity</topic><topic>Spirometry</topic><topic>Structure</topic><topic>Xenon</topic><topic>Xenon Isotopes</topic><toplevel>peer_reviewed</toplevel><toplevel>online_resources</toplevel><creatorcontrib>Willmering, Matthew M.</creatorcontrib><creatorcontrib>Roach, David J.</creatorcontrib><creatorcontrib>Kramer, Elizabeth L.</creatorcontrib><creatorcontrib>Walkup, Laura L.</creatorcontrib><creatorcontrib>Cleveland, Zackary I.</creatorcontrib><creatorcontrib>Woods, Jason C.</creatorcontrib><collection>Web of Science Core Collection</collection><collection>Science Citation Index Expanded</collection><collection>Web of Science - Science Citation Index Expanded - 2021</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><collection>PubMed Central (Full Participant titles)</collection><jtitle>Journal of cystic fibrosis</jtitle></facets><delivery><delcategory>Remote Search Resource</delcategory><fulltext>fulltext</fulltext></delivery><addata><au>Willmering, Matthew M.</au><au>Roach, David J.</au><au>Kramer, Elizabeth L.</au><au>Walkup, Laura L.</au><au>Cleveland, Zackary I.</au><au>Woods, Jason C.</au><format>journal</format><genre>article</genre><ristype>JOUR</ristype><atitle>Sensitive structural and functional measurements and 1-year pulmonary outcomes in pediatric cystic fibrosis</atitle><jtitle>Journal of cystic fibrosis</jtitle><stitle>J CYST FIBROS</stitle><addtitle>J Cyst Fibros</addtitle><date>2021-05-01</date><risdate>2021</risdate><volume>20</volume><issue>3</issue><spage>533</spage><epage>539</epage><pages>533-539</pages><issn>1569-1993</issn><eissn>1873-5010</eissn><abstract>•LCI2.5, 129Xe VDP, and UTE more sensitive to pediatric CF disease than spirometry.•1H MRI suggests increased odds for airway damage in lobes with mucus plugging.•Most spirometry measures correlated with consolidations and ground glass.•LCI2.5 and 129Xe VDP correlated with mucus plugging.•Increased exacerbations most strongly correlated with mucus plugs and airway damage.
Two functional measurements (multiple breath washout [MBW] and hyperpolarized 129Xe ventilation magnetic resonance imaging [129Xe MRI]) have been shown to be more sensitive to cystic fibrosis (CF) lung obstruction than traditional spirometry. However, functional techniques may be sensitive to different underlying structural abnormalities. The purpose of this study was to determine relationships between these functional markers, their pathophysiology, and 1-year clinical outcomes.
Spirometry, MBW, 129Xe MRI, and ultrashort echo-time (UTE) MRI were obtained in a same-day assessment of 27 pediatric CF patients (ages 11.5±5.0) who had not begun CFTR modulator therapies. UTE MRI was scored for structural abnormalities and functional metrics obtained via spirometry, MBW and 129Xe MRI. 1-year outcomes (ΔFEV1 and pulmonary exacerbations), during which ≈50% initiated modulator therapy, were obtained from the electronic medical record.
MBW, 129Xe MRI, and UTE MRI detected clinically significant disease in more subjects (>78%) compared to spirometry (<30%). UTE MRI suggests increased odds of bronchial changes when mucus plugging is present in the same lobe. MBW and 129Xe MRI correlated best with mucus plugging, while spirometry correlated best with consolidations. Bronchial abnormalities were associated with future pulmonary exacerbations.
MBW, 129Xe MRI, and UTE MRI are more sensitive for detection of pediatric CF lung disease when compared to spirometry. MBW and 129Xe MRI correlated with structural abnormalities which occur in early CF disease, suggesting MBW and 129Xe MRI are valuable tools in mild CF lung disease that can guide clinical decision making.</abstract><cop>AMSTERDAM</cop><pub>Elsevier B.V</pub><pmid>33288474</pmid><doi>10.1016/j.jcf.2020.11.019</doi><tpages>7</tpages><orcidid>https://orcid.org/0000-0001-6195-9061</orcidid><orcidid>https://orcid.org/0000-0002-3278-2161</orcidid><oa>free_for_read</oa></addata></record> |
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| subjects | Child Cystic Fibrosis - diagnostic imaging Cystic Fibrosis - physiopathology Female Function Humans Life Sciences & Biomedicine Magnetic Resonance Imaging - methods Male MRI Multiple breath washout Prospective Studies Respiratory System Science & Technology Sensitivity and Specificity Spirometry Structure Xenon Xenon Isotopes |
| title | Sensitive structural and functional measurements and 1-year pulmonary outcomes in pediatric cystic fibrosis |
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