Pulmonary Fibrosis in Children
Identifieur interne : 000459 ( Pmc/Checkpoint ); précédent : 000458; suivant : 000460Pulmonary Fibrosis in Children
Auteurs : Nadia Nathan [France] ; Chiara Sileo [France] ; Guillaume Thouvenin [France] ; Laura Berdah [France] ; Céline Delestrain [France] ; Effrosyne Manali [Grèce] ; Spyros Papiris [Grèce] ; Pierre-Louis Léger [France] ; Hubert Ducou Le Pointe [France] ; Aurore Coulomb L Ermine [France] ; Annick Clement [France]Source :
- Journal of Clinical Medicine [ 2077-0383 ] ; 2019.
Abstract
Pulmonary fibrosis (PF) is a very rare condition in children, which may be observed in specific forms of interstitial lung disease. None of the clinical, radiological, or histological descriptions used for PF diagnosis in adult patients, especially in situations of idiopathic PF, can apply to pediatric situations. This observation supports the view that PF expression may differ with age and, most likely, may cover distinct entities. The present review aims at summarizing the current understanding of PF pathophysiology in children and identifying suitable diagnostic criteria.
Url:
DOI: 10.3390/jcm8091312
PubMed: 31455000
PubMed Central: 6780823
Affiliations:
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Department, Reference Center for Rare Lung Diseases (RespiRare), Armand Trousseau Hospital, Assistance Publique Hôpitaux de Paris (AP-HP), 75012 Paris</wicri:regionArea><placeName><region type="region" nuts="2">Île-de-France</region><settlement type="city">Paris</settlement></placeName></affiliation><affiliation wicri:level="3"><nlm:aff id="af2-jcm-08-01312">Inserm UMR_S933, Sorbonne Université, 75012 Paris, France</nlm:aff><country xml:lang="fr">France</country><wicri:regionArea>Inserm UMR_S933, Sorbonne Université, 75012 Paris</wicri:regionArea><placeName><region type="region" nuts="2">Île-de-France</region><settlement type="city">Paris</settlement></placeName></affiliation></author><author><name sortKey="Sileo, Chiara" sort="Sileo, Chiara" uniqKey="Sileo C" first="Chiara" last="Sileo">Chiara Sileo</name><affiliation wicri:level="3"><nlm:aff id="af3-jcm-08-01312">Pediatric Radiology Department, Armand Trousseau Hospital, AP-HP, 75012 Paris, France</nlm:aff><country 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last="Delestrain">Céline Delestrain</name><affiliation wicri:level="3"><nlm:aff id="af1-jcm-08-01312">Pediatric Pulmonology Department, Reference Center for Rare Lung Diseases (RespiRare), Armand Trousseau Hospital, Assistance Publique Hôpitaux de Paris (AP-HP), 75012 Paris, France</nlm:aff><country xml:lang="fr">France</country><wicri:regionArea>Pediatric Pulmonology Department, Reference Center for Rare Lung Diseases (RespiRare), Armand Trousseau Hospital, Assistance Publique Hôpitaux de Paris (AP-HP), 75012 Paris</wicri:regionArea><placeName><region type="region" nuts="2">Île-de-France</region><settlement type="city">Paris</settlement></placeName></affiliation></author><author><name sortKey="Manali, Effrosyne" sort="Manali, Effrosyne" uniqKey="Manali E" first="Effrosyne" last="Manali">Effrosyne Manali</name><affiliation wicri:level="1"><nlm:aff id="af4-jcm-08-01312">2nd Pulmonary Medicine Department, General University Hospital “Attikon”, Medical School, National and Kapodistrian University of Athens, 12462 Athens, Greece</nlm:aff><country xml:lang="fr">Grèce</country><wicri:regionArea>2nd Pulmonary Medicine Department, General University Hospital “Attikon”, Medical School, National and Kapodistrian University of Athens, 12462 Athens</wicri:regionArea><placeName><settlement type="city">Athènes</settlement><region nuts="2" type="region">Attique (région)</region></placeName></affiliation></author><author><name sortKey="Papiris, Spyros" sort="Papiris, Spyros" uniqKey="Papiris S" first="Spyros" last="Papiris">Spyros Papiris</name><affiliation wicri:level="1"><nlm:aff id="af4-jcm-08-01312">2nd Pulmonary Medicine Department, General University Hospital “Attikon”, Medical School, National and Kapodistrian University of Athens, 12462 Athens, Greece</nlm:aff><country xml:lang="fr">Grèce</country><wicri:regionArea>2nd Pulmonary Medicine Department, General University Hospital “Attikon”, Medical School, National and Kapodistrian University of Athens, 12462 Athens</wicri:regionArea><placeName><settlement type="city">Athènes</settlement><region nuts="2" type="region">Attique (région)</region></placeName></affiliation></author><author><name sortKey="Leger, Pierre Louis" sort="Leger, Pierre Louis" uniqKey="Leger P" first="Pierre-Louis" last="Léger">Pierre-Louis Léger</name><affiliation wicri:level="3"><nlm:aff id="af5-jcm-08-01312">Intensive Care Unit, Armand Trousseau Hospital, AP-HP, 75012 Paris, France</nlm:aff><country xml:lang="fr">France</country><wicri:regionArea>Intensive Care Unit, Armand Trousseau Hospital, AP-HP, 75012 Paris</wicri:regionArea><placeName><region type="region" nuts="2">Île-de-France</region><settlement type="city">Paris</settlement></placeName></affiliation></author><author><name sortKey="Ducou Le Pointe, Hubert" sort="Ducou Le Pointe, Hubert" uniqKey="Ducou Le Pointe H" first="Hubert" last="Ducou Le Pointe">Hubert Ducou Le Pointe</name><affiliation wicri:level="3"><nlm:aff id="af3-jcm-08-01312">Pediatric Radiology Department, Armand Trousseau Hospital, AP-HP, 75012 Paris, France</nlm:aff><country xml:lang="fr">France</country><wicri:regionArea>Pediatric Radiology Department, Armand Trousseau Hospital, AP-HP, 75012 Paris</wicri:regionArea><placeName><region type="region" nuts="2">Île-de-France</region><settlement type="city">Paris</settlement></placeName></affiliation></author><author><name sortKey="Coulomb L Ermine, Aurore" sort="Coulomb L Ermine, Aurore" uniqKey="Coulomb L Ermine A" first="Aurore" last="Coulomb L Ermine">Aurore Coulomb L Ermine</name><affiliation wicri:level="3"><nlm:aff id="af6-jcm-08-01312">Pathology Department, Armand Trousseau Hospital, AP-HP, 75012 Paris, France</nlm:aff><country xml:lang="fr">France</country><wicri:regionArea>Pathology Department, Armand Trousseau Hospital, AP-HP, 75012 Paris</wicri:regionArea><placeName><region type="region" nuts="2">Île-de-France</region><settlement type="city">Paris</settlement></placeName></affiliation></author><author><name sortKey="Clement, Annick" sort="Clement, Annick" uniqKey="Clement A" first="Annick" last="Clement">Annick Clement</name><affiliation wicri:level="3"><nlm:aff id="af1-jcm-08-01312">Pediatric Pulmonology Department, Reference Center for Rare Lung Diseases (RespiRare), Armand Trousseau Hospital, Assistance Publique Hôpitaux de Paris (AP-HP), 75012 Paris, France</nlm:aff><country xml:lang="fr">France</country><wicri:regionArea>Pediatric Pulmonology Department, Reference Center for Rare Lung Diseases (RespiRare), Armand Trousseau Hospital, Assistance Publique Hôpitaux de Paris (AP-HP), 75012 Paris</wicri:regionArea><placeName><region type="region" nuts="2">Île-de-France</region><settlement type="city">Paris</settlement></placeName></affiliation><affiliation wicri:level="3"><nlm:aff id="af2-jcm-08-01312">Inserm UMR_S933, Sorbonne Université, 75012 Paris, France</nlm:aff><country xml:lang="fr">France</country><wicri:regionArea>Inserm UMR_S933, Sorbonne Université, 75012 Paris</wicri:regionArea><placeName><region type="region" nuts="2">Île-de-France</region><settlement type="city">Paris</settlement></placeName></affiliation></author></analytic><series><title level="j">Journal of Clinical Medicine</title><idno type="eISSN">2077-0383</idno><imprint><date when="2019">2019</date></imprint></series></biblStruct></sourceDesc></fileDesc><profileDesc><textClass></textClass></profileDesc></teiHeader><front><div type="abstract" xml:lang="en"><p>Pulmonary fibrosis (PF) is a very rare condition in children, which may be observed in specific forms of interstitial lung disease. None of the clinical, radiological, or histological descriptions used for PF diagnosis in adult patients, especially in situations of idiopathic PF, can apply to pediatric situations. This observation supports the view that PF expression may differ with age and, most likely, may cover distinct entities. The present review aims at summarizing the current understanding of PF pathophysiology in children and identifying suitable diagnostic criteria.</p></div></front><back><div1 type="bibliography"><listBibl><biblStruct><analytic><author><name sortKey="Deutsch, G H" uniqKey="Deutsch G">G.H. Deutsch</name></author><author><name sortKey="Young, L R" uniqKey="Young L">L.R. Young</name></author><author><name sortKey="Deterding, R R" uniqKey="Deterding R">R.R. Deterding</name></author><author><name sortKey="Fan, L L" uniqKey="Fan L">L.L. Fan</name></author><author><name sortKey="Dell, S D" uniqKey="Dell S">S.D. Dell</name></author><author><name sortKey="Bean, J A" uniqKey="Bean J">J.A. 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<front><journal-meta><journal-id journal-id-type="nlm-ta">J Clin Med</journal-id><journal-id journal-id-type="iso-abbrev">J Clin Med</journal-id><journal-id journal-id-type="publisher-id">jcm</journal-id><journal-title-group><journal-title>Journal of Clinical Medicine</journal-title></journal-title-group><issn pub-type="epub">2077-0383</issn><publisher><publisher-name>MDPI</publisher-name></publisher></journal-meta><article-meta><article-id pub-id-type="pmid">31455000</article-id><article-id pub-id-type="pmc">6780823</article-id><article-id pub-id-type="doi">10.3390/jcm8091312</article-id><article-id pub-id-type="publisher-id">jcm-08-01312</article-id><article-categories><subj-group subj-group-type="heading"><subject>Review</subject></subj-group></article-categories><title-group><article-title>Pulmonary Fibrosis in Children</article-title></title-group><contrib-group><contrib contrib-type="author"><contrib-id contrib-id-type="orcid" authenticated="true">https://orcid.org/0000-0001-5149-7975</contrib-id><name><surname>Nathan</surname><given-names>Nadia</given-names></name><xref ref-type="aff" rid="af1-jcm-08-01312">1</xref><xref ref-type="aff" rid="af2-jcm-08-01312">2</xref><xref rid="c1-jcm-08-01312" ref-type="corresp">*</xref></contrib><contrib contrib-type="author"><name><surname>Sileo</surname><given-names>Chiara</given-names></name><xref ref-type="aff" rid="af3-jcm-08-01312">3</xref></contrib><contrib contrib-type="author"><name><surname>Thouvenin</surname><given-names>Guillaume</given-names></name><xref ref-type="aff" rid="af1-jcm-08-01312">1</xref></contrib><contrib contrib-type="author"><name><surname>Berdah</surname><given-names>Laura</given-names></name><xref ref-type="aff" rid="af1-jcm-08-01312">1</xref></contrib><contrib contrib-type="author"><name><surname>Delestrain</surname><given-names>Céline</given-names></name><xref ref-type="aff" rid="af1-jcm-08-01312">1</xref></contrib><contrib contrib-type="author"><name><surname>Manali</surname><given-names>Effrosyne</given-names></name><xref ref-type="aff" rid="af4-jcm-08-01312">4</xref></contrib><contrib contrib-type="author"><name><surname>Papiris</surname><given-names>Spyros</given-names></name><xref ref-type="aff" rid="af4-jcm-08-01312">4</xref></contrib><contrib contrib-type="author"><name><surname>Léger</surname><given-names>Pierre-Louis</given-names></name><xref ref-type="aff" rid="af5-jcm-08-01312">5</xref></contrib><contrib contrib-type="author"><name><surname>Ducou le Pointe</surname><given-names>Hubert</given-names></name><xref ref-type="aff" rid="af3-jcm-08-01312">3</xref></contrib><contrib contrib-type="author"><name><surname>Coulomb l’Hermine</surname><given-names>Aurore</given-names></name><xref ref-type="aff" rid="af6-jcm-08-01312">6</xref></contrib><contrib contrib-type="author"><name><surname>Clement</surname><given-names>Annick</given-names></name><xref ref-type="aff" rid="af1-jcm-08-01312">1</xref><xref ref-type="aff" rid="af2-jcm-08-01312">2</xref></contrib></contrib-group><aff id="af1-jcm-08-01312"><label>1</label>Pediatric Pulmonology Department, Reference Center for Rare Lung Diseases (RespiRare), Armand Trousseau Hospital, Assistance Publique Hôpitaux de Paris (AP-HP), 75012 Paris, France</aff><aff id="af2-jcm-08-01312"><label>2</label>Inserm UMR_S933, Sorbonne Université, 75012 Paris, France</aff><aff id="af3-jcm-08-01312"><label>3</label>Pediatric Radiology Department, Armand Trousseau Hospital, AP-HP, 75012 Paris, France</aff><aff id="af4-jcm-08-01312"><label>4</label>2nd Pulmonary Medicine Department, General University Hospital “Attikon”, Medical School, National and Kapodistrian University of Athens, 12462 Athens, Greece</aff><aff id="af5-jcm-08-01312"><label>5</label>Intensive Care Unit, Armand Trousseau Hospital, AP-HP, 75012 Paris, France</aff><aff id="af6-jcm-08-01312"><label>6</label>Pathology Department, Armand Trousseau Hospital, AP-HP, 75012 Paris, France</aff><author-notes><corresp id="c1-jcm-08-01312"><label>*</label>Correspondence: <email>nadia.nathan@aphp.fr</email>; Tel./Fax: +33-(1)-44-73-66-18</corresp></author-notes><pub-date pub-type="epub"><day>26</day><month>8</month><year>2019</year></pub-date><pub-date pub-type="collection"><month>9</month><year>2019</year></pub-date><volume>8</volume><issue>9</issue><elocation-id>1312</elocation-id><history><date date-type="received"><day>16</day><month>7</month><year>2019</year></date><date date-type="accepted"><day>21</day><month>8</month><year>2019</year></date></history><permissions><copyright-statement>© 2019 by the authors.</copyright-statement><copyright-year>2019</copyright-year><license license-type="open-access"><license-p>Licensee MDPI, Basel, Switzerland. This article is an open access article distributed under the terms and conditions of the Creative Commons Attribution (CC BY) license (<ext-link ext-link-type="uri" xlink:href="http://creativecommons.org/licenses/by/4.0/">http://creativecommons.org/licenses/by/4.0/</ext-link>).</license-p></license></permissions><abstract><p>Pulmonary fibrosis (PF) is a very rare condition in children, which may be observed in specific forms of interstitial lung disease. None of the clinical, radiological, or histological descriptions used for PF diagnosis in adult patients, especially in situations of idiopathic PF, can apply to pediatric situations. This observation supports the view that PF expression may differ with age and, most likely, may cover distinct entities. The present review aims at summarizing the current understanding of PF pathophysiology in children and identifying suitable diagnostic criteria.</p></abstract><kwd-group><kwd>pulmonary fibrosis</kwd><kwd>interstitial lung disease</kwd><kwd>children</kwd><kwd>usual interstitial pneumonia</kwd><kwd>nonspecific interstitial pneumonia</kwd></kwd-group></article-meta></front><floats-group><fig id="jcm-08-01312-f001" orientation="portrait" position="float"><label>Figure 1</label><caption><p>Pulmonary fibrosis examples in children. Panels A and B: Patient 1. (<bold>A</bold>) Chest X-ray: diffuse repartition of ground-glass opacities (GGOs). (<bold>B</bold>) Lung biopsy at age 6: no or mild parenchymal distortion, diffuse thickening of the alveolar walls, hyperplasic alveolar epithelial cells (AECs), inflammatory cell recruitment, mild fibroblasts activation, and mild collagen deposition. Panels C and D: Patient 2. (<bold>C</bold>) Transverse HRCT scan obtained at the level of the upper lobes: diffuse repartition of mild GGOs (white stars), reticulations (with interlobular septal thickening (white arrows) and intralobular lines (black arrows)), and numerous cystic lesions (white arrowheads) with focal left subpleural honeycombing (black arrowhead). Reticulations and cystic lesions are wider on the right. (<bold>D</bold>) Lung biopsy: parenchymal distortion, inflammatory cell recruitment, lymphoid nodules, and mild collagen and severe elastic fiber deposition. Panels E and F: Patient 3. (<bold>E</bold>) Transverse HRCT scan obtained under the level of the carina: diffuse repartition of GGOs (white stars), moderate reticulations (with intralobular lines (black arrows)), and few subpleural cystic lesions (black arrowhead). (<bold>F</bold>) Lung autopsy: no parenchymal distortion, diffuse thickening of the alveolar walls, hyperplasic AECs, mild inflammatory cell recruitment, and elastic fiber deposition. Panels G and H: Patient 4. (<bold>G</bold>) Transverse HRCT scan obtained at the level of the upper lobes: diffuse repartition of severe GGOs (white stars), consolidations (black star), and few cystic lesions (white arrowhead). (<bold>H</bold>) Postmortem biopsy: no parenchymal distortion, diffuse thickening of the alveolar walls, hyperplasic AECs, mild inflammatory cell recruitment, elastic fiber deposition, and moderate alveolar proteinosis: intra-alveolar deposit with giant cells and liproproteic material. Panels I and J: Patient 5. (<bold>I</bold>) HRCT scan obtained at the level of the lung bases: diffuse and homogeneous repartition of GGOs (white stars) with reticulations (with intralobular lines (black arrows)). (<bold>J</bold>) Lung biopsy: no parenchymal distortion, diffuse thickening of the alveolar walls, hyperplasic AECs, and moderate inflammatory cell recruitment.</p></caption><graphic xlink:href="jcm-08-01312-g001"></graphic></fig><fig id="jcm-08-01312-f002" orientation="portrait" position="float"><label>Figure 2</label><caption><p>Potential pathophysiology pathway of pulmonary fibrosis in children. Repeated alveolar lesions’ effects on AECs and alveolar macrophages are likely to be observed in children and adult pulmonary fibrosis, leading to a cellular NSIP pattern. The chance of an evolution toward an adult PF pattern with more fibroblastic activation and extracellular matrix deposition remains unknown.</p></caption><graphic xlink:href="jcm-08-01312-g002"></graphic></fig><table-wrap id="jcm-08-01312-t001" orientation="portrait" position="float"><object-id pub-id-type="pii">jcm-08-01312-t001_Table 1</object-id><label>Table 1</label><caption><p>Suspected cases of pulmonary fibrosis (PF) in the children’s interstitial lung disease (chILD) cohort of Armand Trousseau Hospital.</p></caption><table frame="hsides" rules="groups"><thead><tr><th align="center" valign="middle" style="border-top:solid thin;border-bottom:solid thin" rowspan="1" colspan="1">chILD Condition</th><th align="center" valign="middle" style="border-top:solid thin;border-bottom:solid thin" rowspan="1" colspan="1">Number of Patients</th><th align="center" valign="middle" style="border-top:solid thin;border-bottom:solid thin" rowspan="1" colspan="1">Number of Patients with Available Lung Samples</th><th align="center" valign="middle" style="border-top:solid thin;border-bottom:solid thin" rowspan="1" colspan="1">Number of Cases with Suspected PF</th></tr></thead><tbody><tr><td align="center" valign="middle" style="border-bottom:solid thin" rowspan="1" colspan="1">Surfactant disorders</td><td align="center" valign="middle" style="border-bottom:solid thin" rowspan="1" colspan="1">17</td><td align="center" valign="middle" style="border-bottom:solid thin" rowspan="1" colspan="1">5</td><td align="center" valign="middle" style="border-bottom:solid thin" rowspan="1" colspan="1">2</td></tr><tr><td align="center" valign="middle" style="border-bottom:solid thin" rowspan="1" colspan="1">Autoinflammatory and systemic disorders</td><td align="center" valign="middle" style="border-bottom:solid thin" rowspan="1" colspan="1">6</td><td align="center" valign="middle" style="border-bottom:solid thin" rowspan="1" colspan="1">6</td><td align="center" valign="middle" style="border-bottom:solid thin" rowspan="1" colspan="1">1</td></tr><tr><td align="center" valign="middle" style="border-bottom:solid thin" rowspan="1" colspan="1">Developmental disorders</td><td align="center" valign="middle" style="border-bottom:solid thin" rowspan="1" colspan="1">8</td><td align="center" valign="middle" style="border-bottom:solid thin" rowspan="1" colspan="1">8</td><td align="center" valign="middle" style="border-bottom:solid thin" rowspan="1" colspan="1">0</td></tr><tr><td align="center" valign="middle" style="border-bottom:solid thin" rowspan="1" colspan="1">Others</td><td align="center" valign="middle" style="border-bottom:solid thin" rowspan="1" colspan="1">88</td><td align="center" valign="middle" style="border-bottom:solid thin" rowspan="1" colspan="1">25</td><td align="center" valign="middle" style="border-bottom:solid thin" rowspan="1" colspan="1">7</td></tr><tr><td align="center" valign="middle" style="border-bottom:solid thin" rowspan="1" colspan="1"><bold>Total</bold></td><td align="center" valign="middle" style="border-bottom:solid thin" rowspan="1" colspan="1"><bold>119</bold></td><td align="center" valign="middle" style="border-bottom:solid thin" rowspan="1" colspan="1"><bold>44</bold></td><td align="center" valign="middle" style="border-bottom:solid thin" rowspan="1" colspan="1"><bold>10</bold></td></tr></tbody></table></table-wrap><table-wrap id="jcm-08-01312-t002" orientation="portrait" position="float"><object-id pub-id-type="pii">jcm-08-01312-t002_Table 2</object-id><label>Table 2</label><caption><p>Clinical data and outcomes of five patients of the Trousseau Hospital chILD cohort with lung fibrosis.</p></caption><table frame="hsides" rules="groups"><thead><tr><th align="center" valign="middle" style="border-top:solid thin;border-bottom:solid thin" rowspan="1" colspan="1">Patient Number</th><th align="center" valign="middle" style="border-top:solid thin;border-bottom:solid thin" rowspan="1" colspan="1">Clinical Presentation</th><th align="center" valign="middle" style="border-top:solid thin;border-bottom:solid thin" rowspan="1" colspan="1">Treatment</th><th align="center" valign="middle" style="border-top:solid thin;border-bottom:solid thin" rowspan="1" colspan="1">Outcome</th></tr></thead><tbody><tr><td align="center" valign="middle" style="border-bottom:solid thin" rowspan="1" colspan="1">1 [<xref rid="B14-jcm-08-01312" ref-type="bibr">14</xref>,<xref rid="B15-jcm-08-01312" ref-type="bibr">15</xref>]</td><td align="center" valign="middle" style="border-bottom:solid thin" rowspan="1" colspan="1">6-year-old girl, <italic>ABCA3</italic>-related disease</td><td align="center" valign="middle" style="border-bottom:solid thin" rowspan="1" colspan="1">HCQ, azithromycin</td><td align="center" valign="middle" style="border-bottom:solid thin" rowspan="1" colspan="1">Diffuse fibrosing ILD at age 26</td></tr><tr><td align="center" valign="middle" style="border-bottom:solid thin" rowspan="1" colspan="1">2 [<xref rid="B16-jcm-08-01312" ref-type="bibr">16</xref>]</td><td align="center" valign="middle" style="border-bottom:solid thin" rowspan="1" colspan="1">8-year-old boy, TMEM173-related disease</td><td align="center" valign="middle" style="border-bottom:solid thin" rowspan="1" colspan="1">Corticosteroid pulses, oral corticosteroids, ruxolitinib at age 13.</td><td align="center" valign="middle" style="border-bottom:solid thin" rowspan="1" colspan="1">Lung transplantation at age 14, died at age 16 after second lung transplantation</td></tr><tr><td align="center" valign="middle" style="border-bottom:solid thin" rowspan="1" colspan="1">3</td><td align="center" valign="middle" style="border-bottom:solid thin" rowspan="1" colspan="1">3-year-old boy, undefined chILD</td><td align="center" valign="middle" style="border-bottom:solid thin" rowspan="1" colspan="1">Corticosteroids</td><td align="center" valign="middle" style="border-bottom:solid thin" rowspan="1" colspan="1">Died at age 3 from respiratory failure</td></tr><tr><td align="center" valign="middle" style="border-bottom:solid thin" rowspan="1" colspan="1">4</td><td align="center" valign="middle" style="border-bottom:solid thin" rowspan="1" colspan="1">2-year-old girl, undefined chILD</td><td align="center" valign="middle" style="border-bottom:solid thin" rowspan="1" colspan="1">Corticosteroid pulses, oral corticosteroids, azithromycin, immunosuppressive drugs</td><td align="center" valign="middle" style="border-bottom:solid thin" rowspan="1" colspan="1">Died at age 2 from respiratory failure</td></tr><tr><td align="center" valign="middle" style="border-bottom:solid thin" rowspan="1" colspan="1">5</td><td align="center" valign="middle" style="border-bottom:solid thin" rowspan="1" colspan="1">2-year-old girl, undefined chILD</td><td align="center" valign="middle" style="border-bottom:solid thin" rowspan="1" colspan="1">Corticosteroid pulses, oral corticosteroids, azithromycin</td><td align="center" valign="middle" style="border-bottom:solid thin" rowspan="1" colspan="1">Asymptomatic at age 8</td></tr></tbody></table><table-wrap-foot><fn><p>Abbreviations: chILD, children interstitial lung disease; HCQ, hydroxychloroquine.</p></fn></table-wrap-foot></table-wrap><table-wrap id="jcm-08-01312-t003" orientation="portrait" position="float"><object-id pub-id-type="pii">jcm-08-01312-t003_Table 3</object-id><label>Table 3</label><caption><p>Childhood pathological findings of pulmonary fibrosis compared to adults.</p></caption><table frame="hsides" rules="groups"><thead><tr><th align="center" valign="middle" style="border-top:solid thin;border-bottom:solid thin" rowspan="1" colspan="1"></th><th align="center" valign="middle" style="border-top:solid thin;border-bottom:solid thin" rowspan="1" colspan="1">Pediatric PF</th><th align="center" valign="middle" style="border-top:solid thin;border-bottom:solid thin" rowspan="1" colspan="1">Adult IPF/Probable IPF</th></tr></thead><tbody><tr><td align="center" valign="middle" style="border-bottom:solid thin" rowspan="1" colspan="1">Parenchymal distortion</td><td align="center" valign="middle" style="border-bottom:solid thin" rowspan="1" colspan="1">+</td><td align="center" valign="middle" style="border-bottom:solid thin" rowspan="1" colspan="1">+++</td></tr><tr><td align="center" valign="middle" style="border-bottom:solid thin" rowspan="1" colspan="1">Cellular recruitment</td><td align="center" valign="middle" style="border-bottom:solid thin" rowspan="1" colspan="1">+++</td><td align="center" valign="middle" style="border-bottom:solid thin" rowspan="1" colspan="1">+</td></tr><tr><td align="center" valign="middle" style="border-bottom:solid thin" rowspan="1" colspan="1">Extracellular matrix deposition</td><td align="center" valign="middle" style="border-bottom:solid thin" rowspan="1" colspan="1">+</td><td align="center" valign="middle" style="border-bottom:solid thin" rowspan="1" colspan="1">+++</td></tr><tr><td align="center" valign="middle" style="border-bottom:solid thin" rowspan="1" colspan="1">Fibroblast foci</td><td align="center" valign="middle" style="border-bottom:solid thin" rowspan="1" colspan="1">+/−</td><td align="center" valign="middle" style="border-bottom:solid thin" rowspan="1" colspan="1">+++</td></tr><tr><td align="center" valign="middle" style="border-bottom:solid thin" rowspan="1" colspan="1">Honeycombing</td><td align="center" valign="middle" style="border-bottom:solid thin" rowspan="1" colspan="1">+/−</td><td align="center" valign="middle" style="border-bottom:solid thin" rowspan="1" colspan="1">+++</td></tr><tr><td align="center" valign="middle" style="border-bottom:solid thin" rowspan="1" colspan="1">Global pattern</td><td align="center" valign="middle" style="border-bottom:solid thin" rowspan="1" colspan="1">Predominant NSIP mixed with alveolar proteinosis, DIP, and follicular bronchiolitis</td><td align="center" valign="middle" style="border-bottom:solid thin" rowspan="1" colspan="1">Predominant UIP pattern</td></tr></tbody></table><table-wrap-foot><fn><p>+/−: absent or moderate, +: moderate, +++: important; Abbreviations: PF, pulmonary fibrosis; IPF, idiopathic pulmonary fibrosis; NSIP, nonspecific interstitial pneumonia; DIP, desquamative interstitial pneumonia; UIP, usual interstitial pneumonia.</p></fn></table-wrap-foot></table-wrap></floats-group></pmc><affiliations><list><country><li>France</li><li>Grèce</li></country><region><li>Attique (région)</li><li>Île-de-France</li></region><settlement><li>Athènes</li><li>Paris</li></settlement></list><tree><country name="France"><region name="Île-de-France"><name sortKey="Nathan, Nadia" sort="Nathan, Nadia" uniqKey="Nathan N" first="Nadia" last="Nathan">Nadia Nathan</name></region><name sortKey="Berdah, Laura" sort="Berdah, Laura" uniqKey="Berdah L" first="Laura" last="Berdah">Laura Berdah</name><name sortKey="Clement, Annick" sort="Clement, Annick" uniqKey="Clement A" first="Annick" last="Clement">Annick Clement</name><name sortKey="Clement, Annick" sort="Clement, Annick" uniqKey="Clement A" first="Annick" last="Clement">Annick Clement</name><name sortKey="Coulomb L Ermine, Aurore" sort="Coulomb L Ermine, Aurore" uniqKey="Coulomb L Ermine A" first="Aurore" last="Coulomb L Ermine">Aurore Coulomb L Ermine</name><name sortKey="Delestrain, Celine" sort="Delestrain, Celine" uniqKey="Delestrain C" first="Céline" last="Delestrain">Céline Delestrain</name><name sortKey="Ducou Le Pointe, Hubert" sort="Ducou Le Pointe, Hubert" uniqKey="Ducou Le Pointe H" first="Hubert" last="Ducou Le Pointe">Hubert Ducou Le Pointe</name><name sortKey="Leger, Pierre Louis" sort="Leger, Pierre Louis" uniqKey="Leger P" first="Pierre-Louis" last="Léger">Pierre-Louis Léger</name><name sortKey="Nathan, Nadia" sort="Nathan, Nadia" uniqKey="Nathan N" first="Nadia" last="Nathan">Nadia Nathan</name><name sortKey="Sileo, Chiara" sort="Sileo, Chiara" uniqKey="Sileo C" first="Chiara" last="Sileo">Chiara Sileo</name><name sortKey="Thouvenin, Guillaume" sort="Thouvenin, Guillaume" uniqKey="Thouvenin G" first="Guillaume" last="Thouvenin">Guillaume Thouvenin</name></country><country name="Grèce"><region name="Attique (région)"><name sortKey="Manali, Effrosyne" sort="Manali, Effrosyne" uniqKey="Manali E" first="Effrosyne" last="Manali">Effrosyne Manali</name></region><name sortKey="Papiris, Spyros" sort="Papiris, Spyros" uniqKey="Papiris S" first="Spyros" last="Papiris">Spyros Papiris</name></country></tree></affiliations></record>Pour manipuler ce document sous Unix (Dilib)
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