Serveur d'exploration Chloroquine

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Clinical spectrum of chronic interstitial lung disease in children

Identifieur interne : 002E78 ( Main/Exploration ); précédent : 002E77; suivant : 002E79

Clinical spectrum of chronic interstitial lung disease in children

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

Source :

RBID : ISTEX:22214B84479BCC51693FADC09723EDA4919EB750

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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


Affiliations:


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<term>Colorado (epidemiology)</term>
<term>Follow-Up Studies</term>
<term>Humans</term>
<term>Infant</term>
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<term>Fibrose pulmonaire ()</term>
<term>Fibrose pulmonaire (diagnostic)</term>
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<term>Biopsy</term>
<term>Bronchoalveolar lavage</term>
<term>Cardiac catheterization</term>
<term>Child</term>
<term>Child, Preschool</term>
<term>Chloroquine</term>
<term>Chronic Disease</term>
<term>Chronic lung disease</term>
<term>Clinical findings</term>
<term>Clinical spectrum</term>
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<term>Pediatric</term>
<term>Pediatrics</term>
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<term>Pulmonary hemangiomatosis</term>
<term>Pulmonary hemosiderosis</term>
<term>Pulmonary hypertension</term>
<term>Pulmonary lymphangiomatosis</term>
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<term>Rare disorders</term>
<term>Recombinant interferon</term>
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<term>Teaching hospital</term>
<term>Total lung capacity</term>
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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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