Relevance of 3D Cholangiography and Transient Elastography to Assess Cystic Fibrosis-Associated Liver Disease?
Identifieur interne : 000003 ( Pmc/Checkpoint ); précédent : 000002; suivant : 000004Relevance of 3D Cholangiography and Transient Elastography to Assess Cystic Fibrosis-Associated Liver Disease?
Auteurs : C. Lemaitre [France] ; S. Dominique [France] ; E. Billoud [France] ; M. Eliezer [France] ; H. Montialoux [France] ; M. Quillard [France] ; G. Riachi [France] ; E. Koning [France] ; H. Morisse-Pradier [France] ; G. Savoye [France] ; C. Savoye-Collet [France] ; O. Goria [France]Source :
- Canadian Respiratory Journal [ 1198-2241 ] ; 2016.
Abstract
Url:
DOI: 10.1155/2016/4592702
PubMed: 27445541
PubMed Central: 4939173
Affiliations:
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<record><TEI><teiHeader><fileDesc><titleStmt><title xml:lang="en">Relevance of 3D Cholangiography and Transient Elastography to Assess Cystic Fibrosis-Associated Liver Disease?</title><author><name sortKey="Lemaitre, C" sort="Lemaitre, C" uniqKey="Lemaitre C" first="C." last="Lemaitre">C. Lemaitre</name><affiliation wicri:level="1"><nlm:aff id="I1">Department of Gastroenterology and Hepatology, Rouen University Hospital, Rouen, France</nlm:aff><country xml:lang="fr">France</country><wicri:regionArea>Department of Gastroenterology and Hepatology, Rouen University Hospital, Rouen</wicri:regionArea><placeName><settlement type="city">Rouen</settlement></placeName></affiliation></author><author><name sortKey="Dominique, S" sort="Dominique, S" uniqKey="Dominique S" first="S." last="Dominique">S. Dominique</name><affiliation wicri:level="1"><nlm:aff id="I2">Department of Pneumology, Adult Cystic Fibrosis Centre, Rouen University Hospital, Rouen, France</nlm:aff><country xml:lang="fr">France</country><wicri:regionArea>Department of Pneumology, Adult Cystic Fibrosis Centre, Rouen University Hospital, Rouen</wicri:regionArea><placeName><settlement type="city">Rouen</settlement></placeName></affiliation></author><author><name sortKey="Billoud, E" sort="Billoud, E" uniqKey="Billoud E" first="E." last="Billoud">E. Billoud</name><affiliation wicri:level="1"><nlm:aff id="I3">Department of Gastroenterology, Le Havre General Hospital, Le Havre, France</nlm:aff><country xml:lang="fr">France</country><wicri:regionArea>Department of Gastroenterology, Le Havre General Hospital, Le Havre</wicri:regionArea><placeName><settlement type="city">Le Havre</settlement></placeName></affiliation></author><author><name sortKey="Eliezer, M" sort="Eliezer, M" uniqKey="Eliezer M" first="M." last="Eliezer">M. Eliezer</name><affiliation wicri:level="1"><nlm:aff id="I4">Department of Radiology, Rouen University Hospital, Rouen, France</nlm:aff><country xml:lang="fr">France</country><wicri:regionArea>Department of Radiology, Rouen University Hospital, Rouen</wicri:regionArea><placeName><settlement type="city">Rouen</settlement></placeName></affiliation></author><author><name sortKey="Montialoux, H" sort="Montialoux, H" uniqKey="Montialoux H" first="H." last="Montialoux">H. Montialoux</name><affiliation wicri:level="1"><nlm:aff id="I1">Department of Gastroenterology and Hepatology, Rouen University Hospital, Rouen, France</nlm:aff><country xml:lang="fr">France</country><wicri:regionArea>Department of Gastroenterology and Hepatology, Rouen University Hospital, Rouen</wicri:regionArea><placeName><settlement type="city">Rouen</settlement></placeName></affiliation></author><author><name sortKey="Quillard, M" sort="Quillard, M" uniqKey="Quillard M" first="M." last="Quillard">M. Quillard</name><affiliation wicri:level="1"><nlm:aff id="I5">Clinical Biology Institute, Rouen University Hospital, Rouen, France</nlm:aff><country xml:lang="fr">France</country><wicri:regionArea>Clinical Biology Institute, Rouen University Hospital, Rouen</wicri:regionArea><placeName><settlement type="city">Rouen</settlement></placeName></affiliation></author><author><name sortKey="Riachi, G" sort="Riachi, G" uniqKey="Riachi G" first="G." last="Riachi">G. Riachi</name><affiliation wicri:level="1"><nlm:aff id="I1">Department of Gastroenterology and Hepatology, Rouen University Hospital, Rouen, France</nlm:aff><country xml:lang="fr">France</country><wicri:regionArea>Department of Gastroenterology and Hepatology, Rouen University Hospital, Rouen</wicri:regionArea><placeName><settlement type="city">Rouen</settlement></placeName></affiliation></author><author><name sortKey="Koning, E" sort="Koning, E" uniqKey="Koning E" first="E." last="Koning">E. Koning</name><affiliation wicri:level="1"><nlm:aff id="I4">Department of Radiology, Rouen University Hospital, Rouen, France</nlm:aff><country xml:lang="fr">France</country><wicri:regionArea>Department of Radiology, Rouen University Hospital, Rouen</wicri:regionArea><placeName><settlement type="city">Rouen</settlement></placeName></affiliation></author><author><name sortKey="Morisse Pradier, H" sort="Morisse Pradier, H" uniqKey="Morisse Pradier H" first="H." last="Morisse-Pradier">H. Morisse-Pradier</name><affiliation wicri:level="1"><nlm:aff id="I2">Department of Pneumology, Adult Cystic Fibrosis Centre, Rouen University Hospital, Rouen, France</nlm:aff><country xml:lang="fr">France</country><wicri:regionArea>Department of Pneumology, Adult Cystic Fibrosis Centre, Rouen University Hospital, Rouen</wicri:regionArea><placeName><settlement type="city">Rouen</settlement></placeName></affiliation></author><author><name sortKey="Savoye, G" sort="Savoye, G" uniqKey="Savoye G" first="G." last="Savoye">G. Savoye</name><affiliation wicri:level="1"><nlm:aff id="I1">Department of Gastroenterology and Hepatology, Rouen University Hospital, Rouen, France</nlm:aff><country xml:lang="fr">France</country><wicri:regionArea>Department of Gastroenterology and Hepatology, Rouen University Hospital, Rouen</wicri:regionArea><placeName><settlement type="city">Rouen</settlement></placeName></affiliation><affiliation wicri:level="1"><nlm:aff id="I6">INSERM U1073, Institute for Research and Innovation in Biomedicine, Faculty of Medicine, University of Rouen, Rouen, France</nlm:aff><country xml:lang="fr">France</country><wicri:regionArea>INSERM U1073, Institute for Research and Innovation in Biomedicine, Faculty of Medicine, University of Rouen, Rouen</wicri:regionArea><placeName><settlement type="city">Rouen</settlement></placeName></affiliation></author><author><name sortKey="Savoye Collet, C" sort="Savoye Collet, C" uniqKey="Savoye Collet C" first="C." last="Savoye-Collet">C. Savoye-Collet</name><affiliation wicri:level="1"><nlm:aff id="I4">Department of Radiology, Rouen University Hospital, Rouen, France</nlm:aff><country xml:lang="fr">France</country><wicri:regionArea>Department of Radiology, Rouen University Hospital, Rouen</wicri:regionArea><placeName><settlement type="city">Rouen</settlement></placeName></affiliation></author><author><name sortKey="Goria, O" sort="Goria, O" uniqKey="Goria O" first="O." last="Goria">O. Goria</name><affiliation wicri:level="1"><nlm:aff id="I1">Department of Gastroenterology and Hepatology, Rouen University Hospital, Rouen, France</nlm:aff><country xml:lang="fr">France</country><wicri:regionArea>Department of Gastroenterology and Hepatology, Rouen University Hospital, Rouen</wicri:regionArea><placeName><settlement type="city">Rouen</settlement></placeName></affiliation></author></titleStmt><publicationStmt><idno type="wicri:source">PMC</idno><idno type="pmid">27445541</idno><idno type="pmc">4939173</idno><idno type="url">http://www.ncbi.nlm.nih.gov/pmc/articles/PMC4939173</idno><idno type="RBID">PMC:4939173</idno><idno type="doi">10.1155/2016/4592702</idno><date when="2016">2016</date><idno type="wicri:Area/Pmc/Corpus">000018</idno><idno type="wicri:Area/Pmc/Curation">000018</idno><idno type="wicri:Area/Pmc/Checkpoint">000003</idno></publicationStmt><sourceDesc><biblStruct><analytic><title xml:lang="en" level="a" type="main">Relevance of 3D Cholangiography and Transient Elastography to Assess Cystic Fibrosis-Associated Liver Disease?</title><author><name sortKey="Lemaitre, C" sort="Lemaitre, C" uniqKey="Lemaitre C" first="C." last="Lemaitre">C. Lemaitre</name><affiliation wicri:level="1"><nlm:aff id="I1">Department of Gastroenterology and Hepatology, Rouen University Hospital, Rouen, France</nlm:aff><country xml:lang="fr">France</country><wicri:regionArea>Department of Gastroenterology and Hepatology, Rouen University Hospital, Rouen</wicri:regionArea><placeName><settlement type="city">Rouen</settlement></placeName></affiliation></author><author><name sortKey="Dominique, S" sort="Dominique, S" uniqKey="Dominique S" first="S." last="Dominique">S. Dominique</name><affiliation wicri:level="1"><nlm:aff id="I2">Department of Pneumology, Adult Cystic Fibrosis Centre, Rouen University Hospital, Rouen, France</nlm:aff><country xml:lang="fr">France</country><wicri:regionArea>Department of Pneumology, Adult Cystic Fibrosis Centre, Rouen University Hospital, Rouen</wicri:regionArea><placeName><settlement type="city">Rouen</settlement></placeName></affiliation></author><author><name sortKey="Billoud, E" sort="Billoud, E" uniqKey="Billoud E" first="E." last="Billoud">E. Billoud</name><affiliation wicri:level="1"><nlm:aff id="I3">Department of Gastroenterology, Le Havre General Hospital, Le Havre, France</nlm:aff><country xml:lang="fr">France</country><wicri:regionArea>Department of Gastroenterology, Le Havre General Hospital, Le Havre</wicri:regionArea><placeName><settlement type="city">Le Havre</settlement></placeName></affiliation></author><author><name sortKey="Eliezer, M" sort="Eliezer, M" uniqKey="Eliezer M" first="M." last="Eliezer">M. Eliezer</name><affiliation wicri:level="1"><nlm:aff id="I4">Department of Radiology, Rouen University Hospital, Rouen, France</nlm:aff><country xml:lang="fr">France</country><wicri:regionArea>Department of Radiology, Rouen University Hospital, Rouen</wicri:regionArea><placeName><settlement type="city">Rouen</settlement></placeName></affiliation></author><author><name sortKey="Montialoux, H" sort="Montialoux, H" uniqKey="Montialoux H" first="H." last="Montialoux">H. Montialoux</name><affiliation wicri:level="1"><nlm:aff id="I1">Department of Gastroenterology and Hepatology, Rouen University Hospital, Rouen, France</nlm:aff><country xml:lang="fr">France</country><wicri:regionArea>Department of Gastroenterology and Hepatology, Rouen University Hospital, Rouen</wicri:regionArea><placeName><settlement type="city">Rouen</settlement></placeName></affiliation></author><author><name sortKey="Quillard, M" sort="Quillard, M" uniqKey="Quillard M" first="M." last="Quillard">M. Quillard</name><affiliation wicri:level="1"><nlm:aff id="I5">Clinical Biology Institute, Rouen University Hospital, Rouen, France</nlm:aff><country xml:lang="fr">France</country><wicri:regionArea>Clinical Biology Institute, Rouen University Hospital, Rouen</wicri:regionArea><placeName><settlement type="city">Rouen</settlement></placeName></affiliation></author><author><name sortKey="Riachi, G" sort="Riachi, G" uniqKey="Riachi G" first="G." last="Riachi">G. Riachi</name><affiliation wicri:level="1"><nlm:aff id="I1">Department of Gastroenterology and Hepatology, Rouen University Hospital, Rouen, France</nlm:aff><country xml:lang="fr">France</country><wicri:regionArea>Department of Gastroenterology and Hepatology, Rouen University Hospital, Rouen</wicri:regionArea><placeName><settlement type="city">Rouen</settlement></placeName></affiliation></author><author><name sortKey="Koning, E" sort="Koning, E" uniqKey="Koning E" first="E." last="Koning">E. Koning</name><affiliation wicri:level="1"><nlm:aff id="I4">Department of Radiology, Rouen University Hospital, Rouen, France</nlm:aff><country xml:lang="fr">France</country><wicri:regionArea>Department of Radiology, Rouen University Hospital, Rouen</wicri:regionArea><placeName><settlement type="city">Rouen</settlement></placeName></affiliation></author><author><name sortKey="Morisse Pradier, H" sort="Morisse Pradier, H" uniqKey="Morisse Pradier H" first="H." last="Morisse-Pradier">H. Morisse-Pradier</name><affiliation wicri:level="1"><nlm:aff id="I2">Department of Pneumology, Adult Cystic Fibrosis Centre, Rouen University Hospital, Rouen, France</nlm:aff><country xml:lang="fr">France</country><wicri:regionArea>Department of Pneumology, Adult Cystic Fibrosis Centre, Rouen University Hospital, Rouen</wicri:regionArea><placeName><settlement type="city">Rouen</settlement></placeName></affiliation></author><author><name sortKey="Savoye, G" sort="Savoye, G" uniqKey="Savoye G" first="G." last="Savoye">G. Savoye</name><affiliation wicri:level="1"><nlm:aff id="I1">Department of Gastroenterology and Hepatology, Rouen University Hospital, Rouen, France</nlm:aff><country xml:lang="fr">France</country><wicri:regionArea>Department of Gastroenterology and Hepatology, Rouen University Hospital, Rouen</wicri:regionArea><placeName><settlement type="city">Rouen</settlement></placeName></affiliation><affiliation wicri:level="1"><nlm:aff id="I6">INSERM U1073, Institute for Research and Innovation in Biomedicine, Faculty of Medicine, University of Rouen, Rouen, France</nlm:aff><country xml:lang="fr">France</country><wicri:regionArea>INSERM U1073, Institute for Research and Innovation in Biomedicine, Faculty of Medicine, University of Rouen, Rouen</wicri:regionArea><placeName><settlement type="city">Rouen</settlement></placeName></affiliation></author><author><name sortKey="Savoye Collet, C" sort="Savoye Collet, C" uniqKey="Savoye Collet C" first="C." last="Savoye-Collet">C. Savoye-Collet</name><affiliation wicri:level="1"><nlm:aff id="I4">Department of Radiology, Rouen University Hospital, Rouen, France</nlm:aff><country xml:lang="fr">France</country><wicri:regionArea>Department of Radiology, Rouen University Hospital, Rouen</wicri:regionArea><placeName><settlement type="city">Rouen</settlement></placeName></affiliation></author><author><name sortKey="Goria, O" sort="Goria, O" uniqKey="Goria O" first="O." last="Goria">O. Goria</name><affiliation wicri:level="1"><nlm:aff id="I1">Department of Gastroenterology and Hepatology, Rouen University Hospital, Rouen, France</nlm:aff><country xml:lang="fr">France</country><wicri:regionArea>Department of Gastroenterology and Hepatology, Rouen University Hospital, Rouen</wicri:regionArea><placeName><settlement type="city">Rouen</settlement></placeName></affiliation></author></analytic><series><title level="j">Canadian Respiratory Journal</title><idno type="ISSN">1198-2241</idno><idno type="eISSN">1916-7245</idno><imprint><date when="2016">2016</date></imprint></series></biblStruct></sourceDesc></fileDesc><profileDesc><textClass></textClass></profileDesc></teiHeader><front><div type="abstract" xml:lang="en"><p><italic>Background.</italic> Cystic fibrosis-associated liver disease (CFLD) is a major cause of death. The objective of our retrospective study was to describe the relevance of magnetic resonance imaging (MRI) and liver stiffness measurement (LSM) for CFLD evaluation.<italic> Methods.</italic> All cystic fibrosis adult patients evaluated by MRI and LSM were included. MR signs of portal hypertension (PHT), dysmorphia, or cholangitis were collected and LSM expressed in kPa and Metavir.<italic> Results.</italic> Of 25 patients, 52% had abnormal MRI. Median LSM was 5.7 kPa (3.4–9.9). Three patients had F2 score and one had F3 score. In patients with PHT, LSM was 7.85 kPa (3.7–9.9) compared to 5 (3.4–7.5) in others, <italic>p</italic> = 0.02. In patients with abnormal liver function tests, 50% had increased LSM (≥F2), whereas 94% with normal tests had normal LSM (<italic>p</italic> = 0.04). Seven patients had abnormal MRI despite normal ultrasonography.<italic> Conclusions.</italic> MRI and LSM provide useful information on CFLD and may help to screen patients with PHT.</p></div></front><back><div1 type="bibliography"><listBibl><biblStruct><analytic><author><name sortKey="Sokol, R J" uniqKey="Sokol R">R. J. Sokol</name></author><author><name sortKey="Durie, P R" uniqKey="Durie P">P. R. Durie</name></author></analytic></biblStruct><biblStruct><analytic><author><name sortKey="Colombo, C" uniqKey="Colombo C">C. Colombo</name></author><author><name sortKey="Battezzati, P M" uniqKey="Battezzati P">P. M. Battezzati</name></author><author><name sortKey="Crosignani, A" uniqKey="Crosignani A">A. Crosignani</name></author></analytic></biblStruct><biblStruct><analytic><author><name sortKey="Lindblad, A" uniqKey="Lindblad A">A. Lindblad</name></author><author><name sortKey="Glaumann, H" uniqKey="Glaumann H">H. 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<front><journal-meta><journal-id journal-id-type="nlm-ta">Can Respir J</journal-id><journal-id journal-id-type="iso-abbrev">Can. Respir. J</journal-id><journal-id journal-id-type="publisher-id">CRJ</journal-id><journal-title-group><journal-title>Canadian Respiratory Journal</journal-title></journal-title-group><issn pub-type="ppub">1198-2241</issn><issn pub-type="epub">1916-7245</issn><publisher><publisher-name>Hindawi Publishing Corporation</publisher-name></publisher></journal-meta><article-meta><article-id pub-id-type="pmid">27445541</article-id><article-id pub-id-type="pmc">4939173</article-id><article-id pub-id-type="doi">10.1155/2016/4592702</article-id><article-categories><subj-group subj-group-type="heading"><subject>Research Article</subject></subj-group></article-categories><title-group><article-title>Relevance of 3D Cholangiography and Transient Elastography to Assess Cystic Fibrosis-Associated Liver Disease?</article-title></title-group><contrib-group><contrib contrib-type="author"><name><surname>Lemaitre</surname><given-names>C.</given-names></name><xref ref-type="aff" rid="I1"><sup>1</sup></xref><xref ref-type="corresp" rid="cor1"><sup>*</sup></xref></contrib><contrib contrib-type="author"><name><surname>Dominique</surname><given-names>S.</given-names></name><xref ref-type="aff" rid="I2"><sup>2</sup></xref></contrib><contrib contrib-type="author"><name><surname>Billoud</surname><given-names>E.</given-names></name><xref ref-type="aff" rid="I3"><sup>3</sup></xref></contrib><contrib contrib-type="author"><name><surname>Eliezer</surname><given-names>M.</given-names></name><xref ref-type="aff" rid="I4"><sup>4</sup></xref></contrib><contrib contrib-type="author"><name><surname>Montialoux</surname><given-names>H.</given-names></name><xref ref-type="aff" rid="I1"><sup>1</sup></xref></contrib><contrib contrib-type="author"><name><surname>Quillard</surname><given-names>M.</given-names></name><xref ref-type="aff" rid="I5"><sup>5</sup></xref></contrib><contrib contrib-type="author"><name><surname>Riachi</surname><given-names>G.</given-names></name><xref ref-type="aff" rid="I1"><sup>1</sup></xref></contrib><contrib contrib-type="author"><name><surname>Koning</surname><given-names>E.</given-names></name><xref ref-type="aff" rid="I4"><sup>4</sup></xref></contrib><contrib contrib-type="author"><name><surname>Morisse-Pradier</surname><given-names>H.</given-names></name><xref ref-type="aff" rid="I2"><sup>2</sup></xref></contrib><contrib contrib-type="author"><name><surname>Savoye</surname><given-names>G.</given-names></name><xref ref-type="aff" rid="I1"><sup>1</sup></xref><xref ref-type="aff" rid="I6"><sup>6</sup></xref></contrib><contrib contrib-type="author"><name><surname>Savoye-Collet</surname><given-names>C.</given-names></name><xref ref-type="aff" rid="I4"><sup>4</sup></xref></contrib><contrib contrib-type="author"><name><surname>Goria</surname><given-names>O.</given-names></name><xref ref-type="aff" rid="I1"><sup>1</sup></xref></contrib></contrib-group><aff id="I1"><sup>1</sup>Department of Gastroenterology and Hepatology, Rouen University Hospital, Rouen, France</aff><aff id="I2"><sup>2</sup>Department of Pneumology, Adult Cystic Fibrosis Centre, Rouen University Hospital, Rouen, France</aff><aff id="I3"><sup>3</sup>Department of Gastroenterology, Le Havre General Hospital, Le Havre, France</aff><aff id="I4"><sup>4</sup>Department of Radiology, Rouen University Hospital, Rouen, France</aff><aff id="I5"><sup>5</sup>Clinical Biology Institute, Rouen University Hospital, Rouen, France</aff><aff id="I6"><sup>6</sup>INSERM U1073, Institute for Research and Innovation in Biomedicine, Faculty of Medicine, University of Rouen, Rouen, France</aff><author-notes><corresp id="cor1">*C. Lemaitre: <email>caroline.lemaitre@chu-rouen.fr</email></corresp><fn fn-type="other"><p>Academic Editor: Christophe Leroyer</p></fn></author-notes><pub-date pub-type="ppub"><year>2016</year></pub-date><pub-date pub-type="epub"><day>26</day><month>6</month><year>2016</year></pub-date><volume>2016</volume><elocation-id>4592702</elocation-id><history><date date-type="received"><day>16</day><month>9</month><year>2015</year></date><date date-type="rev-recd"><day>25</day><month>5</month><year>2016</year></date><date date-type="accepted"><day>2</day><month>6</month><year>2016</year></date></history><permissions><copyright-statement>Copyright © 2016 C. Lemaitre et al.</copyright-statement><copyright-year>2016</copyright-year><license license-type="open-access"><license-p>This is an open access article distributed under the Creative Commons Attribution License, which permits unrestricted use, distribution, and reproduction in any medium, provided the original work is properly cited.</license-p></license></permissions><abstract><p><italic>Background.</italic> Cystic fibrosis-associated liver disease (CFLD) is a major cause of death. The objective of our retrospective study was to describe the relevance of magnetic resonance imaging (MRI) and liver stiffness measurement (LSM) for CFLD evaluation.<italic> Methods.</italic> All cystic fibrosis adult patients evaluated by MRI and LSM were included. MR signs of portal hypertension (PHT), dysmorphia, or cholangitis were collected and LSM expressed in kPa and Metavir.<italic> Results.</italic> Of 25 patients, 52% had abnormal MRI. Median LSM was 5.7 kPa (3.4–9.9). Three patients had F2 score and one had F3 score. In patients with PHT, LSM was 7.85 kPa (3.7–9.9) compared to 5 (3.4–7.5) in others, <italic>p</italic> = 0.02. In patients with abnormal liver function tests, 50% had increased LSM (≥F2), whereas 94% with normal tests had normal LSM (<italic>p</italic> = 0.04). Seven patients had abnormal MRI despite normal ultrasonography.<italic> Conclusions.</italic> MRI and LSM provide useful information on CFLD and may help to screen patients with PHT.</p></abstract></article-meta></front><floats-group><fig id="fig1" orientation="portrait" position="float"><label>Figure 1</label><caption><p>Flow chart for inclusion in our study.</p></caption><graphic xlink:href="CRJ2016-4592702.001"></graphic></fig><fig id="fig2" orientation="portrait" position="float"><label>Figure 2</label><caption><p>3D MRI cholangiogram shows intrahepatic and extrahepatic biliary duct irregularity, with choledochus stenosis.</p></caption><graphic xlink:href="CRJ2016-4592702.002"></graphic></fig><fig id="fig3" orientation="portrait" position="float"><label>Figure 3</label><caption><p>ROC curve for liver stiffness measurement predicting the presence of portal hypertension.</p></caption><graphic xlink:href="CRJ2016-4592702.003"></graphic></fig><table-wrap id="tab1" orientation="portrait" position="float"><label>Table 1</label><caption><p>Patients' characteristics at study enrolment. </p></caption><table frame="hsides" rules="groups"><thead><tr><th align="left" rowspan="1" colspan="1">Patients' characteristics</th><th align="center" rowspan="1" colspan="1">Studied patients<break></break><italic>n</italic> = 25 (%)</th><th align="center" rowspan="1" colspan="1">Whole CF cohort<break></break><italic>n</italic> = 57 (%)</th><th align="center" rowspan="1" colspan="1"> <italic>p</italic></th></tr></thead><tbody><tr><td align="left" rowspan="1" colspan="1">Gender (male/female)</td><td align="center" rowspan="1" colspan="1">0.46</td><td align="center" rowspan="1" colspan="1">0.50</td><td align="center" rowspan="1" colspan="1">1</td></tr><tr><td align="left" rowspan="1" colspan="1">Median age (yrs [min.–max.])</td><td align="center" rowspan="1" colspan="1">25 [18–43]</td><td align="center" rowspan="1" colspan="1">30.1 [18–62]</td><td align="center" rowspan="1" colspan="1">0.12</td></tr><tr><td align="left" rowspan="1" colspan="1">Meconium ileus</td><td align="center" rowspan="1" colspan="1">4 (16%)</td><td align="center" rowspan="1" colspan="1">12 (21%)</td><td align="center" rowspan="1" colspan="1">0.76</td></tr><tr><td align="left" rowspan="1" colspan="1">Pancreatic insufficiency </td><td align="center" rowspan="1" colspan="1">22 (88%)</td><td align="center" rowspan="1" colspan="1">49 (85.9%)</td><td align="center" rowspan="1" colspan="1">1</td></tr><tr><td align="left" rowspan="1" colspan="1">Genotype</td><td align="center" rowspan="1" colspan="1"> </td><td align="center" rowspan="1" colspan="1"> </td><td align="center" rowspan="1" colspan="1"> </td></tr><tr><td align="left" rowspan="1" colspan="1"> ΔF508-ΔF508</td><td align="center" rowspan="1" colspan="1">11 (44%)</td><td align="center" rowspan="1" colspan="1">20 (35.1%)</td><td align="center" rowspan="1" colspan="1">0.46</td></tr><tr><td align="left" rowspan="1" colspan="1"> ΔF508, other mutations</td><td align="center" rowspan="1" colspan="1">12 (48%)</td><td align="center" rowspan="1" colspan="1">31 (54%)</td><td align="center" rowspan="1" colspan="1">0.63</td></tr><tr><td align="left" rowspan="1" colspan="1"> 2 other mutations</td><td align="center" rowspan="1" colspan="1">1 (4%)</td><td align="center" rowspan="1" colspan="1">6 (10%)</td><td align="center" rowspan="1" colspan="1">1</td></tr><tr><td align="left" rowspan="1" colspan="1">Medium BMI (kg/m<sup>2</sup> [min.–max.])</td><td align="center" rowspan="1" colspan="1">19.3 [17.0–29.4]</td><td align="center" rowspan="1" colspan="1">21.4 [17.0–32.9]</td><td align="center" rowspan="1" colspan="1">0.11</td></tr><tr><td align="left" rowspan="1" colspan="1">Alcohol </td><td align="center" rowspan="1" colspan="1">0 (0%)</td><td align="center" rowspan="1" colspan="1">0 (0%)</td><td align="center" rowspan="1" colspan="1">1</td></tr><tr><td align="left" rowspan="1" colspan="1">Diabetes mellitus </td><td align="center" rowspan="1" colspan="1">4 (16%)</td><td align="center" rowspan="1" colspan="1">13 (22.8%)</td><td align="center" rowspan="1" colspan="1">0.57</td></tr><tr><td align="left" rowspan="1" colspan="1">Dyslipidemia</td><td align="center" rowspan="1" colspan="1">1 (4%)</td><td align="center" rowspan="1" colspan="1">1 (1.7%)</td><td align="center" rowspan="1" colspan="1">0.52</td></tr><tr><td align="left" rowspan="1" colspan="1">Antibiotics</td><td align="center" rowspan="1" colspan="1">17 (68%)</td><td align="center" rowspan="1" colspan="1">41 (72%)</td><td align="center" rowspan="1" colspan="1">0.79</td></tr><tr><td align="left" rowspan="1" colspan="1">Antifungal drugs</td><td align="center" rowspan="1" colspan="1">19 (76%)</td><td align="center" rowspan="1" colspan="1">29 (51%)</td><td align="center" rowspan="1" colspan="1">0.051</td></tr><tr><td align="left" rowspan="1" colspan="1">UDCA treatment </td><td align="center" rowspan="1" colspan="1">10 (40%)</td><td align="center" rowspan="1" colspan="1">14 (24%)</td><td align="center" rowspan="1" colspan="1">0.19</td></tr><tr><td align="left" rowspan="1" colspan="1">Creatinine clearance ≥ 70 mL/min</td><td align="center" rowspan="1" colspan="1">25 (100%)</td><td align="center" rowspan="1" colspan="1">57 (100%)</td><td align="center" rowspan="1" colspan="1">1</td></tr><tr><td align="center" colspan="4" rowspan="1"><hr></hr></td></tr><tr><td align="left" rowspan="1" colspan="1">FEV1 (%)</td><td align="center" rowspan="1" colspan="1">67.6% [50.4–84.8]</td><td align="center" rowspan="1" colspan="1">71.2% [47.5–94.9]</td><td align="center" rowspan="1" colspan="1">0.33</td></tr></tbody></table><table-wrap-foot><fn><p>BMI: body mass index; UDCA: ursodeoxycholic acid.</p></fn></table-wrap-foot></table-wrap><table-wrap id="tab2" orientation="portrait" position="float"><label>Table 2</label><caption><p>MRI results. </p></caption><table frame="hsides" rules="groups"><thead><tr><th align="left" rowspan="1" colspan="1">Diagnosis in MRI</th><th align="left" rowspan="1" colspan="1">Specific abnormalities in MRI</th><th align="center" rowspan="1" colspan="1"><italic>N</italic></th><th align="center" rowspan="1" colspan="1">Total number (%)</th></tr></thead><tbody><tr><td align="left" rowspan="1" colspan="1">Abnormal MRI</td><td align="left" rowspan="1" colspan="1"> </td><td align="center" rowspan="1" colspan="1"> </td><td align="center" rowspan="1" colspan="1">13 (52%)</td></tr><tr><td align="center" colspan="4" rowspan="1"><hr></hr></td></tr><tr><td rowspan="2" align="left" colspan="1">Hepatic dysmorphia</td><td align="left" rowspan="1" colspan="1">Included lobulation of liver surface</td><td align="center" rowspan="1" colspan="1">3</td><td rowspan="2" align="center" colspan="1">3 (12%)</td></tr><tr><td align="left" rowspan="1" colspan="1">Included first-segment hypertrophy</td><td align="center" rowspan="1" colspan="1">0</td></tr><tr><td align="center" colspan="4" rowspan="1"><hr></hr></td></tr><tr><td rowspan="3" align="left" colspan="1">Portal hypertension</td><td align="left" rowspan="1" colspan="1">Included splenomegaly</td><td align="center" rowspan="1" colspan="1">5</td><td rowspan="3" align="center" colspan="1">5 (20%)</td></tr><tr><td align="left" rowspan="1" colspan="1">Included portal vein dilatation</td><td align="center" rowspan="1" colspan="1">2</td></tr><tr><td align="left" rowspan="1" colspan="1">Included splenic vein dilatation</td><td align="center" rowspan="1" colspan="1">1</td></tr><tr><td align="center" colspan="4" rowspan="1"><hr></hr></td></tr><tr><td rowspan="2" align="left" colspan="1">Isolated biliary abnormalities</td><td align="left" rowspan="1" colspan="1">Intrahepatic</td><td align="center" rowspan="1" colspan="1">1</td><td rowspan="2" align="center" colspan="1">1 (4%)</td></tr><tr><td align="left" rowspan="1" colspan="1">Extrahepatic</td><td align="center" rowspan="1" colspan="1">0</td></tr><tr><td align="center" colspan="4" rowspan="1"><hr></hr></td></tr><tr><td align="left" rowspan="1" colspan="1">Hepatic dysmorphia and portal hypertension</td><td align="left" rowspan="1" colspan="1"> </td><td align="center" rowspan="1" colspan="1">0</td><td align="center" rowspan="1" colspan="1">0 (0%)</td></tr><tr><td align="center" colspan="4" rowspan="1"><hr></hr></td></tr><tr><td align="left" rowspan="1" colspan="1">Hepatic dysmorphia and biliary abnormalities</td><td align="left" rowspan="1" colspan="1">Included lobulation of liver surface and intrahepatic abnormalities</td><td align="center" rowspan="1" colspan="1">1</td><td align="center" rowspan="1" colspan="1">1 (4%)</td></tr><tr><td align="center" colspan="4" rowspan="1"><hr></hr></td></tr><tr><td align="left" rowspan="1" colspan="1">Portal hypertension and biliary abnormalities</td><td align="left" rowspan="1" colspan="1">Included portal vein hypertrophy and intrahepatic abnormalities</td><td align="center" rowspan="1" colspan="1">1</td><td align="center" rowspan="1" colspan="1">1 (4%)</td></tr><tr><td align="center" colspan="4" rowspan="1"><hr></hr></td></tr><tr><td align="left" rowspan="1" colspan="1">Hepatic dysmorphia, portal hypertension, and biliary abnormalities</td><td align="left" rowspan="1" colspan="1">Included splenomegaly, lobulation of liver surface, first-segment hypertrophy, and intrahepatic and extrahepatic abnormalities</td><td align="center" rowspan="1" colspan="1">2</td><td align="center" rowspan="1" colspan="1">2 (8%)</td></tr><tr><td align="center" colspan="4" rowspan="1"><hr></hr></td></tr><tr><td align="left" rowspan="1" colspan="1">Normal MRI</td><td align="left" rowspan="1" colspan="1"> </td><td align="center" rowspan="1" colspan="1"> </td><td align="center" rowspan="1" colspan="1">12 (48%)</td></tr><tr><td align="center" colspan="4" rowspan="1"><hr></hr></td></tr><tr><td align="left" rowspan="1" colspan="1">Total </td><td align="left" rowspan="1" colspan="1"> </td><td align="center" rowspan="1" colspan="1"> </td><td align="center" rowspan="1" colspan="1">25 (100%)</td></tr></tbody></table><table-wrap-foot><fn><p><italic>N</italic>: number of patients; MRI: magnetic resonance imaging.</p></fn></table-wrap-foot></table-wrap><table-wrap id="tab3" orientation="portrait" position="float"><label>Table 3</label><caption><p>Results of LSM and MRI according to the results of liver function tests and abdominal ultrasonography. </p></caption><table frame="hsides" rules="groups"><thead><tr><th colspan="2" align="center" rowspan="1">LFT or US abnormalities</th><th rowspan="2" align="center" colspan="1"><italic>p</italic></th></tr><tr><th align="left" rowspan="1" colspan="1">Yes (<italic>n</italic> = 6)</th><th align="center" rowspan="1" colspan="1">No (<italic>n</italic> = 17)</th></tr></thead><tbody><tr><td align="left" rowspan="1" colspan="1">50% F0-F1 (<italic>n</italic> = 3)</td><td align="center" rowspan="1" colspan="1">94% F0-F1 (<italic>n</italic> = 16)</td><td rowspan="2" align="center" colspan="1">0.04</td></tr><tr><td align="left" rowspan="1" colspan="1">50% ≥ F2 (<italic>n</italic> = 3)</td><td align="center" rowspan="1" colspan="1">6% ≥ F2 (<italic>n</italic> = 1)</td></tr><tr><td align="center" colspan="3" rowspan="1"><hr></hr></td></tr><tr><td align="left" rowspan="1" colspan="1">33.3% normal MRI (<italic>n</italic> = 2)</td><td align="center" rowspan="1" colspan="1">58.8% normal MRI (<italic>n</italic> = 10)</td><td rowspan="2" align="center" colspan="1">0.37</td></tr><tr><td align="left" rowspan="1" colspan="1">66.7% abnormal MRI (<italic>n</italic> = 4)</td><td align="center" rowspan="1" colspan="1">41.2% abnormal MRI (<italic>n</italic> = 7)</td></tr></tbody></table><table-wrap-foot><fn><p>MRI: magnetic resonance imaging; US: ultrasonography; LFT: liver function test.</p></fn></table-wrap-foot></table-wrap></floats-group></pmc><affiliations><list><country><li>France</li></country><settlement><li>Le Havre</li><li>Rouen</li></settlement></list><tree><country name="France"><noRegion><name sortKey="Lemaitre, C" sort="Lemaitre, C" uniqKey="Lemaitre C" first="C." last="Lemaitre">C. Lemaitre</name></noRegion><name sortKey="Billoud, E" sort="Billoud, E" uniqKey="Billoud E" first="E." last="Billoud">E. Billoud</name><name sortKey="Dominique, S" sort="Dominique, S" uniqKey="Dominique S" first="S." last="Dominique">S. Dominique</name><name sortKey="Eliezer, M" sort="Eliezer, M" uniqKey="Eliezer M" first="M." last="Eliezer">M. Eliezer</name><name sortKey="Goria, O" sort="Goria, O" uniqKey="Goria O" first="O." last="Goria">O. Goria</name><name sortKey="Koning, E" sort="Koning, E" uniqKey="Koning E" first="E." last="Koning">E. Koning</name><name sortKey="Montialoux, H" sort="Montialoux, H" uniqKey="Montialoux H" first="H." last="Montialoux">H. Montialoux</name><name sortKey="Morisse Pradier, H" sort="Morisse Pradier, H" uniqKey="Morisse Pradier H" first="H." last="Morisse-Pradier">H. Morisse-Pradier</name><name sortKey="Quillard, M" sort="Quillard, M" uniqKey="Quillard M" first="M." last="Quillard">M. Quillard</name><name sortKey="Riachi, G" sort="Riachi, G" uniqKey="Riachi G" first="G." last="Riachi">G. Riachi</name><name sortKey="Savoye Collet, C" sort="Savoye Collet, C" uniqKey="Savoye Collet C" first="C." last="Savoye-Collet">C. Savoye-Collet</name><name sortKey="Savoye, G" sort="Savoye, G" uniqKey="Savoye G" first="G." last="Savoye">G. Savoye</name><name sortKey="Savoye, G" sort="Savoye, G" uniqKey="Savoye G" first="G." last="Savoye">G. Savoye</name></country></tree></affiliations></record>Pour manipuler ce document sous Unix (Dilib)
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