Idiopathic pulmonary fibrosis in infants : good prognosis with conservative management
Identifieur interne : 002616 ( Main/Merge ); précédent : 002615; suivant : 002617Idiopathic pulmonary fibrosis in infants : good prognosis with conservative management
Auteurs : D. Hacking [Australie] ; R. Smyth [Royaume-Uni] ; N. Shaw [Royaume-Uni] ; G. Kokia [Royaume-Uni] ; H. Carty [Royaume-Uni] ; D. Heaf [Royaume-Uni]Source :
- Archives of disease in childhood [ 0003-9888 ] ; 2000.
Descripteurs français
- Pascal (Inist)
- Wicri :
- topic : Enfant.
English descriptors
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Abstract
Background-Pulmonary interstitial fibrosis in children is a disease of unknown aetiology, usually associated with a poor prognosis. Methods-In this case series we describe 11 children presenting over a 10 year period, managed conservatively and associated with a good prognosis. Results-In six, symptoms were present from birth and 10 had symptoms at or before 3 months. Diagnosis was made using chest computed tomography and percutaneous lung biopsy. All patients were treated with oral prednisolone. In five no steroid response was noted. One patient responded to hydroxychloroquine. Home oxygen was required in five patients. At follow up all patients are alive at a median age of 6 years (range 1 to 12 years). The two recently diagnosed children have significant symptoms, seven have dyspnoea on exercise, and two are symptom free. Conclusion-The good prognosis seen in these patients is different to previous case reports, indicating a greater than 50% mortality.
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Pascal:00-0386276Le document en format XML
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Smyth</name><affiliation wicri:level="1"><inist:fA14 i1="02"><s1>Respiratory Unit, Royal Liverpool Children's NHS Trust, Alder Hey Children's Hospital, Eaton Road</s1><s2>Liverpool</s2><s3>GBR</s3><sZ>2 aut.</sZ><sZ>3 aut.</sZ><sZ>6 aut.</sZ></inist:fA14><country>Royaume-Uni</country><wicri:noRegion>Liverpool</wicri:noRegion></affiliation></author><author><name sortKey="Shaw, N" sort="Shaw, N" uniqKey="Shaw N" first="N." last="Shaw">N. Shaw</name><affiliation wicri:level="1"><inist:fA14 i1="02"><s1>Respiratory Unit, Royal Liverpool Children's NHS Trust, Alder Hey Children's Hospital, Eaton Road</s1><s2>Liverpool</s2><s3>GBR</s3><sZ>2 aut.</sZ><sZ>3 aut.</sZ><sZ>6 aut.</sZ></inist:fA14><country>Royaume-Uni</country><wicri:noRegion>Liverpool</wicri:noRegion></affiliation></author><author><name sortKey="Kokia, G" sort="Kokia, G" uniqKey="Kokia G" first="G." last="Kokia">G. Kokia</name><affiliation wicri:level="1"><inist:fA14 i1="03"><s1>Pathology Department, Royal Liverpool Children's NHS Trust</s1><s3>GBR</s3><sZ>4 aut.</sZ></inist:fA14><country>Royaume-Uni</country><wicri:noRegion>Pathology Department, Royal Liverpool Children's NHS Trust</wicri:noRegion></affiliation></author><author><name sortKey="Carty, H" sort="Carty, H" uniqKey="Carty H" first="H." last="Carty">H. Carty</name><affiliation wicri:level="1"><inist:fA14 i1="04"><s1>Department of Radiology, Royal Liverpool Children's NHS Trust</s1><s3>GBR</s3><sZ>5 aut.</sZ></inist:fA14><country>Royaume-Uni</country><wicri:noRegion>Department of Radiology, Royal Liverpool Children's NHS Trust</wicri:noRegion></affiliation></author><author><name sortKey="Heaf, D" sort="Heaf, D" uniqKey="Heaf D" first="D." last="Heaf">D. 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Hacking</name><affiliation wicri:level="3"><inist:fA14 i1="01"><s1>Neonatal Unit, Royal Children's Hospital, Flemington Road</s1><s2>Melbourne</s2><s3>AUS</s3><sZ>1 aut.</sZ></inist:fA14><country>Australie</country><placeName><settlement type="city">Melbourne</settlement><region type="état">Victoria (État)</region></placeName></affiliation></author><author><name sortKey="Smyth, R" sort="Smyth, R" uniqKey="Smyth R" first="R." last="Smyth">R. Smyth</name><affiliation wicri:level="1"><inist:fA14 i1="02"><s1>Respiratory Unit, Royal Liverpool Children's NHS Trust, Alder Hey Children's Hospital, Eaton Road</s1><s2>Liverpool</s2><s3>GBR</s3><sZ>2 aut.</sZ><sZ>3 aut.</sZ><sZ>6 aut.</sZ></inist:fA14><country>Royaume-Uni</country><wicri:noRegion>Liverpool</wicri:noRegion></affiliation></author><author><name sortKey="Shaw, N" sort="Shaw, N" uniqKey="Shaw N" first="N." last="Shaw">N. Shaw</name><affiliation wicri:level="1"><inist:fA14 i1="02"><s1>Respiratory Unit, Royal Liverpool Children's NHS Trust, Alder Hey Children's Hospital, Eaton Road</s1><s2>Liverpool</s2><s3>GBR</s3><sZ>2 aut.</sZ><sZ>3 aut.</sZ><sZ>6 aut.</sZ></inist:fA14><country>Royaume-Uni</country><wicri:noRegion>Liverpool</wicri:noRegion></affiliation></author><author><name sortKey="Kokia, G" sort="Kokia, G" uniqKey="Kokia G" first="G." last="Kokia">G. Kokia</name><affiliation wicri:level="1"><inist:fA14 i1="03"><s1>Pathology Department, Royal Liverpool Children's NHS Trust</s1><s3>GBR</s3><sZ>4 aut.</sZ></inist:fA14><country>Royaume-Uni</country><wicri:noRegion>Pathology Department, Royal Liverpool Children's NHS Trust</wicri:noRegion></affiliation></author><author><name sortKey="Carty, H" sort="Carty, H" uniqKey="Carty H" first="H." last="Carty">H. Carty</name><affiliation wicri:level="1"><inist:fA14 i1="04"><s1>Department of Radiology, Royal Liverpool Children's NHS Trust</s1><s3>GBR</s3><sZ>5 aut.</sZ></inist:fA14><country>Royaume-Uni</country><wicri:noRegion>Department of Radiology, Royal Liverpool Children's NHS Trust</wicri:noRegion></affiliation></author><author><name sortKey="Heaf, D" sort="Heaf, D" uniqKey="Heaf D" first="D." last="Heaf">D. Heaf</name><affiliation wicri:level="1"><inist:fA14 i1="02"><s1>Respiratory Unit, Royal Liverpool Children's NHS Trust, Alder Hey Children's Hospital, Eaton Road</s1><s2>Liverpool</s2><s3>GBR</s3><sZ>2 aut.</sZ><sZ>3 aut.</sZ><sZ>6 aut.</sZ></inist:fA14><country>Royaume-Uni</country><wicri:noRegion>Liverpool</wicri:noRegion></affiliation></author></analytic><series><title level="j" type="main">Archives of disease in childhood</title><title level="j" type="abbreviated">Arch. dis. child.</title><idno type="ISSN">0003-9888</idno><imprint><date when="2000">2000</date></imprint></series></biblStruct></sourceDesc><seriesStmt><title level="j" type="main">Archives of disease in childhood</title><title level="j" type="abbreviated">Arch. dis. child.</title><idno type="ISSN">0003-9888</idno></seriesStmt></fileDesc><profileDesc><textClass><keywords scheme="KwdEn" xml:lang="en"><term>Antiinflammatory agent</term><term>Case study</term><term>Chemotherapy</term><term>Child</term><term>Corticosteroid</term><term>Hamman Rich interstitial pulmonary fibrosis</term><term>Idiopathic</term><term>Immunosuppressive agent</term><term>Infant</term><term>Long term</term><term>Prednisolone</term><term>Prognosis</term><term>Treatment</term></keywords><keywords scheme="Pascal" xml:lang="fr"><term>Fibrose diffuse interstitielle poumon Hamman Rich</term><term>Idiopathique</term><term>Pronostic</term><term>Long terme</term><term>Prednisolone</term><term>Antiinflammatoire</term><term>Immunodépresseur</term><term>Chimiothérapie</term><term>Traitement</term><term>Etude cas</term><term>Nourrisson</term><term>Enfant</term><term>Corticostéroïde</term></keywords><keywords scheme="Wicri" type="topic" xml:lang="fr"><term>Enfant</term></keywords></textClass></profileDesc></teiHeader><front><div type="abstract" xml:lang="en">Background-Pulmonary interstitial fibrosis in children is a disease of unknown aetiology, usually associated with a poor prognosis. Methods-In this case series we describe 11 children presenting over a 10 year period, managed conservatively and associated with a good prognosis. Results-In six, symptoms were present from birth and 10 had symptoms at or before 3 months. Diagnosis was made using chest computed tomography and percutaneous lung biopsy. All patients were treated with oral prednisolone. In five no steroid response was noted. One patient responded to hydroxychloroquine. Home oxygen was required in five patients. At follow up all patients are alive at a median age of 6 years (range 1 to 12 years). The two recently diagnosed children have significant symptoms, seven have dyspnoea on exercise, and two are symptom free. Conclusion-The good prognosis seen in these patients is different to previous case reports, indicating a greater than 50% mortality.</div></front></TEI><affiliations><list><country><li>Australie</li><li>Royaume-Uni</li></country><region><li>Victoria (État)</li></region><settlement><li>Melbourne</li></settlement></list><tree><country name="Australie"><region name="Victoria (État)"><name sortKey="Hacking, D" sort="Hacking, D" uniqKey="Hacking D" first="D." last="Hacking">D. Hacking</name></region></country><country name="Royaume-Uni"><noRegion><name sortKey="Smyth, R" sort="Smyth, R" uniqKey="Smyth R" first="R." last="Smyth">R. Smyth</name></noRegion><name sortKey="Carty, H" sort="Carty, H" uniqKey="Carty H" first="H." last="Carty">H. Carty</name><name sortKey="Heaf, D" sort="Heaf, D" uniqKey="Heaf D" first="D." last="Heaf">D. Heaf</name><name sortKey="Kokia, G" sort="Kokia, G" uniqKey="Kokia G" first="G." last="Kokia">G. Kokia</name><name sortKey="Shaw, N" sort="Shaw, N" uniqKey="Shaw N" first="N." last="Shaw">N. Shaw</name></country></tree></affiliations></record>Pour manipuler ce document sous Unix (Dilib)
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