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Idiopathic pulmonary fibrosis in infants : good prognosis with conservative management

Identifieur interne : 000062 ( PascalFrancis/Checkpoint ); précédent : 000061; suivant : 000063

Idiopathic 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 :

RBID : Pascal:00-0386276

Descripteurs français

English descriptors

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:00-0386276

Le document en format XML

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<fC07 i1="04" i2="X" l="ENG">
<s0>Interstitial pneumonitis</s0>
<s5>39</s5>
</fC07>
<fC07 i1="04" i2="X" l="SPA">
<s0>Neumopatía intersticial</s0>
<s5>39</s5>
</fC07>
<fN21>
<s1>262</s1>
</fN21>
</pA>
</standard>
</inist>
<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>

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