Abnormal preschool Lung Clearance Index (LCI) reflects clinical status and predicts lower spirometry later in childhood in cystic fibrosis

AbstractBackgroundClinical and prognostic value of preschool Multiple Breath Washout (MBW) remains unclear. MethodsInitial MBW results (Exhalyzer® D, EcoMedics AG) in preschool Cystic Fibrosis (CF) subjects (age 2–6 years) at a time of clinical stability were compared to (1) concurrent clinical stat...

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Veröffentlicht in:Journal of cystic fibrosis 2019-09, Vol.18 (5), p.721-727
Hauptverfasser: Hardaker, K.M, Panda, H, Hulme, K, Wong, A, Coward, E, Cooper, P, Fitzgerald, D.A, Pandit, C, Towns, S, Selvadurai, H, Robinson, P.D
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container_end_page 727
container_issue 5
container_start_page 721
container_title Journal of cystic fibrosis
container_volume 18
creator Hardaker, K.M
Panda, H
Hulme, K
Wong, A
Coward, E
Cooper, P
Fitzgerald, D.A
Pandit, C
Towns, S
Selvadurai, H
Robinson, P.D
description AbstractBackgroundClinical and prognostic value of preschool Multiple Breath Washout (MBW) remains unclear. MethodsInitial MBW results (Exhalyzer® D, EcoMedics AG) in preschool Cystic Fibrosis (CF) subjects (age 2–6 years) at a time of clinical stability were compared to (1) concurrent clinical status measures and (2) later spirometry outcomes. Abnormal Lung Clearance Index (LCI) was defined using published reference data (ULN for LCI 8.0). ResultsLCI was abnormal in 56% (28/50), with mean (SD) LCI 8.61(1.85) at age 4.71(1.3) years. Abnormal LCI was associated with higher dornase alfa use, previous positive bacterial cultures and pF508.del homozygous genotype. Later spirometry ( n = 44; mean (SD) 2.3(0.5) years after MBW) demonstrated that abnormal initial preschool LCI was a strong predictor of lower later spirometry outcomes. ConclusionAbnormal preschool LCI was associated with concurrent measures of clinical status and later spirometry deficits, suggesting early prognostic utility of MBW testing in this age range.
doi_str_mv 10.1016/j.jcf.2019.02.007
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MethodsInitial MBW results (Exhalyzer® D, EcoMedics AG) in preschool Cystic Fibrosis (CF) subjects (age 2–6 years) at a time of clinical stability were compared to (1) concurrent clinical status measures and (2) later spirometry outcomes. Abnormal Lung Clearance Index (LCI) was defined using published reference data (ULN for LCI 8.0). ResultsLCI was abnormal in 56% (28/50), with mean (SD) LCI 8.61(1.85) at age 4.71(1.3) years. Abnormal LCI was associated with higher dornase alfa use, previous positive bacterial cultures and pF508.del homozygous genotype. Later spirometry ( n = 44; mean (SD) 2.3(0.5) years after MBW) demonstrated that abnormal initial preschool LCI was a strong predictor of lower later spirometry outcomes. ConclusionAbnormal preschool LCI was associated with concurrent measures of clinical status and later spirometry deficits, suggesting early prognostic utility of MBW testing in this age range.</description><identifier>ISSN: 1569-1993</identifier><identifier>EISSN: 1873-5010</identifier><identifier>DOI: 10.1016/j.jcf.2019.02.007</identifier><identifier>PMID: 30827846</identifier><language>eng</language><publisher>Netherlands: Elsevier B.V</publisher><subject>Cystic fibrosis ; Multiple breath washout ; Preschool ; Pulmonary/Respiratory ; Spirometry</subject><ispartof>Journal of cystic fibrosis, 2019-09, Vol.18 (5), p.721-727</ispartof><rights>2019</rights><rights>Copyright © 2019. 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MethodsInitial MBW results (Exhalyzer® D, EcoMedics AG) in preschool Cystic Fibrosis (CF) subjects (age 2–6 years) at a time of clinical stability were compared to (1) concurrent clinical status measures and (2) later spirometry outcomes. Abnormal Lung Clearance Index (LCI) was defined using published reference data (ULN for LCI 8.0). ResultsLCI was abnormal in 56% (28/50), with mean (SD) LCI 8.61(1.85) at age 4.71(1.3) years. Abnormal LCI was associated with higher dornase alfa use, previous positive bacterial cultures and pF508.del homozygous genotype. Later spirometry ( n = 44; mean (SD) 2.3(0.5) years after MBW) demonstrated that abnormal initial preschool LCI was a strong predictor of lower later spirometry outcomes. ConclusionAbnormal preschool LCI was associated with concurrent measures of clinical status and later spirometry deficits, suggesting early prognostic utility of MBW testing in this age range.</description><subject>Cystic fibrosis</subject><subject>Multiple breath washout</subject><subject>Preschool</subject><subject>Pulmonary/Respiratory</subject><subject>Spirometry</subject><issn>1569-1993</issn><issn>1873-5010</issn><fulltext>true</fulltext><rsrctype>article</rsrctype><creationdate>2019</creationdate><recordtype>article</recordtype><recordid>eNp9Uk1v1DAUjBCIlsIP4IJ8LIekz07ixEJCqlYUVlqJQ8vZcmyHOjjxYieF_Qv86r6wLYceOPlrZp7fzMuytxQKCpRfDMWg-4IBFQWwAqB5lp3StinzGig8x33NRU6FKE-yVykNALSBpn2ZnZTQsqat-Gn257KbQhyVJ_tok74NwZPdMn0nG29VVJO2ZDsZ-5uc7zbb9yTa3ls9J6K9m5xGWprVvCSiJrMqGLc--vDLRpL2LobRzvFAvJrxwk1E3zpvsIj5ezik2WnSuy6G5NLr7EWvfLJvHtaz7NvVp5vNl3z39fN2c7nLdVXTOS9F1XXKCMa6SjS0bwVlBltTTde10FDK6s5w1hvDDeiGC1r2uuTAjOp7ju6cZedH3X0MPxebZjm6pK33arJhSZKhhaLmrGoRSo9QjT9M2LzcRzeqeJAU5BqBHCRGINcIJDCJESDn3YP80o3W_GM8eo6AD0eAxSbvnI0yaWfRaeMieitNcP-V__iE_RjFD3uwaQhLnNA9SWVCgrxeZ2AdASpKAFbz8h75gK1L</recordid><startdate>20190901</startdate><enddate>20190901</enddate><creator>Hardaker, K.M</creator><creator>Panda, H</creator><creator>Hulme, K</creator><creator>Wong, A</creator><creator>Coward, E</creator><creator>Cooper, P</creator><creator>Fitzgerald, D.A</creator><creator>Pandit, C</creator><creator>Towns, S</creator><creator>Selvadurai, H</creator><creator>Robinson, P.D</creator><general>Elsevier B.V</general><scope>NPM</scope><scope>AAYXX</scope><scope>CITATION</scope><scope>7X8</scope><orcidid>https://orcid.org/0000-0001-7397-105X</orcidid></search><sort><creationdate>20190901</creationdate><title>Abnormal preschool Lung Clearance Index (LCI) reflects clinical status and predicts lower spirometry later in childhood in cystic fibrosis</title><author>Hardaker, K.M ; Panda, H ; Hulme, K ; Wong, A ; Coward, E ; Cooper, P ; Fitzgerald, D.A ; Pandit, C ; Towns, S ; Selvadurai, H ; Robinson, P.D</author></sort><facets><frbrtype>5</frbrtype><frbrgroupid>cdi_FETCH-LOGICAL-c451t-394bbad922b4971f8912d001a7bb8071125bd62fdd6d0c76913fc3602daff6873</frbrgroupid><rsrctype>articles</rsrctype><prefilter>articles</prefilter><language>eng</language><creationdate>2019</creationdate><topic>Cystic fibrosis</topic><topic>Multiple breath washout</topic><topic>Preschool</topic><topic>Pulmonary/Respiratory</topic><topic>Spirometry</topic><toplevel>peer_reviewed</toplevel><toplevel>online_resources</toplevel><creatorcontrib>Hardaker, K.M</creatorcontrib><creatorcontrib>Panda, H</creatorcontrib><creatorcontrib>Hulme, K</creatorcontrib><creatorcontrib>Wong, A</creatorcontrib><creatorcontrib>Coward, E</creatorcontrib><creatorcontrib>Cooper, P</creatorcontrib><creatorcontrib>Fitzgerald, D.A</creatorcontrib><creatorcontrib>Pandit, C</creatorcontrib><creatorcontrib>Towns, S</creatorcontrib><creatorcontrib>Selvadurai, H</creatorcontrib><creatorcontrib>Robinson, P.D</creatorcontrib><collection>PubMed</collection><collection>CrossRef</collection><collection>MEDLINE - Academic</collection><jtitle>Journal of cystic fibrosis</jtitle></facets><delivery><delcategory>Remote Search Resource</delcategory><fulltext>fulltext</fulltext></delivery><addata><au>Hardaker, K.M</au><au>Panda, H</au><au>Hulme, K</au><au>Wong, A</au><au>Coward, E</au><au>Cooper, P</au><au>Fitzgerald, D.A</au><au>Pandit, C</au><au>Towns, S</au><au>Selvadurai, H</au><au>Robinson, P.D</au><format>journal</format><genre>article</genre><ristype>JOUR</ristype><atitle>Abnormal preschool Lung Clearance Index (LCI) reflects clinical status and predicts lower spirometry later in childhood in cystic fibrosis</atitle><jtitle>Journal of cystic fibrosis</jtitle><addtitle>J Cyst Fibros</addtitle><date>2019-09-01</date><risdate>2019</risdate><volume>18</volume><issue>5</issue><spage>721</spage><epage>727</epage><pages>721-727</pages><issn>1569-1993</issn><eissn>1873-5010</eissn><abstract>AbstractBackgroundClinical and prognostic value of preschool Multiple Breath Washout (MBW) remains unclear. 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source ScienceDirect Journals (5 years ago - present); EZB-FREE-00999 freely available EZB journals
subjects Cystic fibrosis
Multiple breath washout
Preschool
Pulmonary/Respiratory
Spirometry
title Abnormal preschool Lung Clearance Index (LCI) reflects clinical status and predicts lower spirometry later in childhood in cystic fibrosis
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