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Clinical spectrum of chronic interstitial lung disease in children

Identifieur interne : 000157 ( Istex/Corpus ); précédent : 000156; suivant : 000158

Clinical spectrum of chronic interstitial lung disease in children

Auteurs : Leland L. Fan ; Ann L. W. Mullen ; Susan M. Brugman ; Stephen C. Inscore ; David P. Parks ; Carl W. White

Source :

RBID : ISTEX:22214B84479BCC51693FADC09723EDA4919EB750

English descriptors

Abstract

To describe the clinical spectrum of interstitial lung disease in children, we reviewed our experience with 48 patients during a 12-year period. Most patients initially had typical findings of restrictive lung disease and hypoxemia. Growth failure or pulmonary hypertension or both were found in more than one third. Specific diagnosis, made in 35 patients (70%), most often required invasive studies, particularly open lung biopsy. Although the diagnostic yield from open lung biopsy was high, the diagnosis of many patients remained uncertain. Many different disorders were encountered. The response to corticosteroids, bronchodilators, and chloroquine was inconsistent. Six patients died, five within 1 year after the initial evaluation. The spectrum of pediatric interstitial lung disease includes a large, heterogeneous group of rare disorders associated with high morbidity and mortallty rates.

Url:
DOI: 10.1016/S0022-3476(05)80330-0

Links to Exploration step

ISTEX:22214B84479BCC51693FADC09723EDA4919EB750

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<div type="abstract" xml:lang="en">To describe the clinical spectrum of interstitial lung disease in children, we reviewed our experience with 48 patients during a 12-year period. Most patients initially had typical findings of restrictive lung disease and hypoxemia. Growth failure or pulmonary hypertension or both were found in more than one third. Specific diagnosis, made in 35 patients (70%), most often required invasive studies, particularly open lung biopsy. Although the diagnostic yield from open lung biopsy was high, the diagnosis of many patients remained uncertain. Many different disorders were encountered. The response to corticosteroids, bronchodilators, and chloroquine was inconsistent. Six patients died, five within 1 year after the initial evaluation. The spectrum of pediatric interstitial lung disease includes a large, heterogeneous group of rare disorders associated with high morbidity and mortallty rates.</div>
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<ce:textfn>Original article</ce:textfn>
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<ce:title>Clinical spectrum of chronic interstitial lung disease in children</ce:title>
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<ce:author>
<ce:degrees>MD</ce:degrees>
<ce:given-name>Leland L.</ce:given-name>
<ce:surname>Fan</ce:surname>
<ce:cross-ref refid="aff1">
<ce:sup>a</ce:sup>
</ce:cross-ref>
<ce:cross-ref refid="aff2">
<ce:sup>b</ce:sup>
</ce:cross-ref>
<ce:cross-ref refid="aff3">
<ce:sup>c</ce:sup>
</ce:cross-ref>
<ce:cross-ref refid="cor1">
<ce:sup>**</ce:sup>
</ce:cross-ref>
</ce:author>
<ce:author>
<ce:degrees>RN, MS</ce:degrees>
<ce:given-name>Ann L.W.</ce:given-name>
<ce:surname>Mullen</ce:surname>
<ce:cross-ref refid="aff1">
<ce:sup>a</ce:sup>
</ce:cross-ref>
<ce:cross-ref refid="aff2">
<ce:sup>b</ce:sup>
</ce:cross-ref>
<ce:cross-ref refid="aff3">
<ce:sup>c</ce:sup>
</ce:cross-ref>
</ce:author>
<ce:author>
<ce:degrees>MD</ce:degrees>
<ce:given-name>Susan M.</ce:given-name>
<ce:surname>Brugman</ce:surname>
<ce:cross-ref refid="aff1">
<ce:sup>a</ce:sup>
</ce:cross-ref>
<ce:cross-ref refid="aff2">
<ce:sup>b</ce:sup>
</ce:cross-ref>
<ce:cross-ref refid="aff3">
<ce:sup>c</ce:sup>
</ce:cross-ref>
</ce:author>
<ce:author>
<ce:degrees>MD</ce:degrees>
<ce:given-name>Stephen C.</ce:given-name>
<ce:surname>Inscore</ce:surname>
<ce:cross-ref refid="aff1">
<ce:sup>a</ce:sup>
</ce:cross-ref>
<ce:cross-ref refid="aff2">
<ce:sup>b</ce:sup>
</ce:cross-ref>
<ce:cross-ref refid="aff3">
<ce:sup>c</ce:sup>
</ce:cross-ref>
</ce:author>
<ce:author>
<ce:degrees>MD</ce:degrees>
<ce:given-name>David P.</ce:given-name>
<ce:surname>Parks</ce:surname>
<ce:cross-ref refid="aff1">
<ce:sup>a</ce:sup>
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<ce:given-name>Carl W.</ce:given-name>
<ce:surname>White</ce:surname>
<ce:cross-ref refid="aff1">
<ce:sup>a</ce:sup>
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<ce:label>a</ce:label>
<ce:textfn>Division of Pediatric Pulmonology Natiohal Jewish Center for Immunology and Respiratory Medicine, Denver, Colorado, USA</ce:textfn>
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<ce:affiliation id="aff2">
<ce:label>b</ce:label>
<ce:textfn>Department of Pediatrics, University of Colorado Health Sciences Center, Denver, USA</ce:textfn>
</ce:affiliation>
<ce:affiliation id="aff3">
<ce:label>c</ce:label>
<ce:textfn>Department of Pediatrics, Brooke Army Medical Center, Fort Sam Houston, Texas USA</ce:textfn>
</ce:affiliation>
<ce:correspondence id="cor1">
<ce:label>**</ce:label>
<ce:text>Reprint requests: Leland L. Fan, MD, National Jewish Center for Immunology and Respiratory Medicine, 1400 Jackson, Denver, CO 80206.</ce:text>
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<ce:label>*</ce:label>
<ce:note-para>Supported in part (Dr. White) by a Grant-in-Aid and an Established Investigator Award from the American Heart Association National Center, and National Institutes of Health SCOR I P50 L 46481-01.</ce:note-para>
</ce:footnote>
</ce:author-group>
<ce:date-received day="30" month="12" year="1991"></ce:date-received>
<ce:date-accepted day="28" month="7" year="1992"></ce:date-accepted>
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<ce:simple-para>To describe the clinical spectrum of interstitial lung disease in children, we reviewed our experience with 48 patients during a 12-year period. Most patients initially had typical findings of restrictive lung disease and hypoxemia. Growth failure or pulmonary hypertension or both were found in more than one third. Specific diagnosis, made in 35 patients (70%), most often required invasive studies, particularly open lung biopsy. Although the diagnostic yield from open lung biopsy was high, the diagnosis of many patients remained uncertain. Many different disorders were encountered. The response to corticosteroids, bronchodilators, and chloroquine was inconsistent. Six patients died, five within 1 year after the initial evaluation. The spectrum of pediatric interstitial lung disease includes a large, heterogeneous group of rare disorders associated with high morbidity and mortallty rates.</ce:simple-para>
</ce:abstract-sec>
</ce:abstract>
</head>
<tail>
<ce:bibliography>
<ce:section-title>References</ce:section-title>
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<title>Clinical spectrum of chronic interstitial lung disease in children</title>
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<title>Clinical spectrum of chronic interstitial lung disease in children</title>
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<name type="personal">
<namePart type="given">Leland L.</namePart>
<namePart type="family">Fan</namePart>
<namePart type="termsOfAddress">MD</namePart>
<affiliation>Division of Pediatric Pulmonology Natiohal Jewish Center for Immunology and Respiratory Medicine, Denver, Colorado, USA</affiliation>
<affiliation>Department of Pediatrics, University of Colorado Health Sciences Center, Denver, USA</affiliation>
<affiliation>Department of Pediatrics, Brooke Army Medical Center, Fort Sam Houston, Texas USA</affiliation>
<affiliation>Reprint requests: Leland L. Fan, MD, National Jewish Center for Immunology and Respiratory Medicine, 1400 Jackson, Denver, CO 80206.</affiliation>
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<name type="personal">
<namePart type="given">Ann L.W.</namePart>
<namePart type="family">Mullen</namePart>
<namePart type="termsOfAddress">RN, MS</namePart>
<affiliation>Division of Pediatric Pulmonology Natiohal Jewish Center for Immunology and Respiratory Medicine, Denver, Colorado, USA</affiliation>
<affiliation>Department of Pediatrics, University of Colorado Health Sciences Center, Denver, USA</affiliation>
<affiliation>Department of Pediatrics, Brooke Army Medical Center, Fort Sam Houston, Texas USA</affiliation>
<role>
<roleTerm type="text">author</roleTerm>
</role>
</name>
<name type="personal">
<namePart type="given">Susan M.</namePart>
<namePart type="family">Brugman</namePart>
<namePart type="termsOfAddress">MD</namePart>
<affiliation>Division of Pediatric Pulmonology Natiohal Jewish Center for Immunology and Respiratory Medicine, Denver, Colorado, USA</affiliation>
<affiliation>Department of Pediatrics, University of Colorado Health Sciences Center, Denver, USA</affiliation>
<affiliation>Department of Pediatrics, Brooke Army Medical Center, Fort Sam Houston, Texas USA</affiliation>
<role>
<roleTerm type="text">author</roleTerm>
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</name>
<name type="personal">
<namePart type="given">Stephen C.</namePart>
<namePart type="family">Inscore</namePart>
<namePart type="termsOfAddress">MD</namePart>
<affiliation>Division of Pediatric Pulmonology Natiohal Jewish Center for Immunology and Respiratory Medicine, Denver, Colorado, USA</affiliation>
<affiliation>Department of Pediatrics, University of Colorado Health Sciences Center, Denver, USA</affiliation>
<affiliation>Department of Pediatrics, Brooke Army Medical Center, Fort Sam Houston, Texas USA</affiliation>
<role>
<roleTerm type="text">author</roleTerm>
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</name>
<name type="personal">
<namePart type="given">David P.</namePart>
<namePart type="family">Parks</namePart>
<namePart type="termsOfAddress">MD</namePart>
<affiliation>Division of Pediatric Pulmonology Natiohal Jewish Center for Immunology and Respiratory Medicine, Denver, Colorado, USA</affiliation>
<affiliation>Department of Pediatrics, University of Colorado Health Sciences Center, Denver, USA</affiliation>
<affiliation>Department of Pediatrics, Brooke Army Medical Center, Fort Sam Houston, Texas USA</affiliation>
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<name type="personal">
<namePart type="given">Carl W.</namePart>
<namePart type="family">White</namePart>
<namePart type="termsOfAddress">MD</namePart>
<affiliation>Division of Pediatric Pulmonology Natiohal Jewish Center for Immunology and Respiratory Medicine, Denver, Colorado, USA</affiliation>
<affiliation>Department of Pediatrics, University of Colorado Health Sciences Center, Denver, USA</affiliation>
<affiliation>Department of Pediatrics, Brooke Army Medical Center, Fort Sam Houston, Texas USA</affiliation>
<affiliation>*Supported in part (Dr. White) by a Grant-in-Aid and an Established Investigator Award from the American Heart Association National Center, and National Institutes of Health SCOR I P50 L 46481-01.</affiliation>
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<abstract lang="en">To describe the clinical spectrum of interstitial lung disease in children, we reviewed our experience with 48 patients during a 12-year period. Most patients initially had typical findings of restrictive lung disease and hypoxemia. Growth failure or pulmonary hypertension or both were found in more than one third. Specific diagnosis, made in 35 patients (70%), most often required invasive studies, particularly open lung biopsy. Although the diagnostic yield from open lung biopsy was high, the diagnosis of many patients remained uncertain. Many different disorders were encountered. The response to corticosteroids, bronchodilators, and chloroquine was inconsistent. Six patients died, five within 1 year after the initial evaluation. The spectrum of pediatric interstitial lung disease includes a large, heterogeneous group of rare disorders associated with high morbidity and mortallty rates.</abstract>
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<identifier type="ISSN">0022-3476</identifier>
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